Pediatric Lower Limb Deformities 2016

Sanjeev Sabharwal
Editor
Pediatric Lower
Limb Deformities
Principles and Techniques

of Management
123
Pediatric Lower Limb Deformities
Sanjeev Sabharwal
Editor
Pediatric Lower Limb

Deformities
Principles and Techniques of Management
Editor
Sanjeev Sabharwal, MD, MPH
Department of Orthopedics
Division of Pediatric Orthopedics
Rutgers-New Jersey Medical School
Newark, NJ, USA
ISBN 978-3-319-17096-1 ISBN 978-3-319-17097-8 (eBook)

DOI 10.1007/978-3-319-17097-8
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Springer Cham Heidelberg New York Dordrecht London

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To all children with limb deformities and their caregivers.
Foreword
“There is only one child in the world and the Child’s name is All Children.” Thus did the
poet Carl Sandburg succinctly describe the universal appeal of children and humankind’s
concern for child welfare, health, and happiness. The most successful charitable organiza-
tions, such as Easter Seals (originally, the National Society for Crippled Children) and the
March of Dimes (originally, the National Foundation for Infantile Paralysis), continue to
focus fundraising and service projects on children with birth defects and developmental
and acquired deformities and disorders.
Likewise, major service organizations and clubs, including the Shriners and the Scottish
Rite Freemasons, point to their respective children’s hospitals with pride and a sense of accom-
plishment. All of these efforts, which occupy so much time and energy, are directed towards
a single goal: helping clinicians and researchers cure or relieve those conditions that prevent
a child from reaching his or her full potential. In this regard, there is no higher calling that a human
being could possibly undertake than being a frontline soldier in the battle against childhood
diseases, disorders, and deformities.
Up until the mid-twentieth century, surgery focused on correcting pediatric congenital,
developmental, and acquired musculoskeletal defects and deformities had a limited capacity to
obtain full restoration of function. Often times, amputation proved the most practical means of
achieving maximal functional capacity for a child with certain limb deficiencies. Correction of
angular long bone deformities, for example, involved wedge resection of osseous tissue that
would certainly improve the angulation but left the youngster with the need for clunky shoes
or braces (or both) to get around.
In 1951, Prof. G. A. Ilizarov discovered how to create new osseous tissue in a widening distrac-
tion gap. He worked in far-off Siberia, alone at first, away from the prying eyes of Soviet col-
leagues, giving him a chance to learn by trial and error the parameters of his evolving methodology.
More than 30 years elapsed before surgeons in Western countries learned of Ilizarov’s discoveries.
By then, techniques and instrumentation had been perfected, basic science research was com-
pleted, and a massive Medical Center had been constructed in Kurgan, USSR, all to provide care
to children and adults with musculoskeletal conditions never before thought treatable.
As a result, the Methods of Ilizarov first appeared in Western medicine as a mature system
of treatment, capable of achieving stunning results. In a sense, the situation resembled the
appearance of Nike Athena in Greek mythology, who sprung as a fully grown adult from the
head of Zeus, armor-clad and battle-ready.
In the 25 years since the introduction of Ilizarov’s therapeutic strategies into Western medi-
cine, ingenious surgeons have found ways to combine Ilizarov’s distraction osteogenesis with
well-established principles of deformity correction, growth rate prediction, and soft tissue
releases to yield new and ever-evolving paradigms to deal most effectively with conditions that
interfere with a child’s full participation in life’s activities.
Although these combined surgical techniques are reported piecemeal in journal articles and
at open meetings, there has not been, until now, a textbook updating the current principles of
pediatric deformity correction in a comprehensive way. The volume you are holding in your
hands was specifically designed to overcome such a deficiency. Dr. Sabharwal has prevailed
upon leading practitioners of these modern strategies to write chapters in their fields of interest
vii
viii Foreword
and clinical research. As a result, this book is a remarkable compendium of primary source
material that will aid clinicians around the world in treating lower limb deformities of
childhood.
My only regret is that visionary founders of the charitable organizations and service clubs
who dedicated so much effort to establish children’s hospitals and child-centered research and
support enterprises are not alive today to see what has become of their dreams and hard work.
They would be amazed, that’s for sure!
Orange, CA, USA Stuart A. Green, MD

Preface
In titling his 1741 book, Nicolas Andry coined the phrase Orthopédie from two Greek words:
orthos, “straight, correct,” and paiedeia, “rearing of children.” While the field of orthopedics
has branched further into a number of subspecialties affecting various body parts in the young
and old, Andry’s original illustration of a straight stake tied to a crooked sapling has withstood
the test of time. However, despite the ubiquitous presence of lower limb deformities in children
globally (albeit with varied etiologies), a textbook devoted to the treatment of the “crooked
child” is sorely missing from the current literature.
This unique text is primarily intended for orthopedic surgeons and trainees worldwide who
have an interest in pediatric lower limb deformities. This book is not meant to be a “how to
apply an external fixator to the tibia” type of manual, but rather a broad-based text highlighting
both general principles and specific strategies for managing the entire spectrum of pediatric
lower limb deformities, applying to circumstances of various etiology and resource availability.
The authors of the 32 chapters are well-known leaders in the field of pediatric lower limb
deformities, and I am truly indebted to every one of them and their coauthors for their excellent
contributions. I invited these contributors not only on the basis of their expertise in the field but
also in light of the diversity of their working environments and unique challenges that they face
when treating children with limb deformities.
This book is divided into five parts, although each chapter can serve as a standalone guide
for the clinician dealing with a specific patient. Part I highlights the general principles and
techniques, including patient evaluation, decision making, and various surgical methods for
deformity correction. Part II deals with related concepts, including management of soft tissue
contractures, amputation, and working in resource-challenged environments. Part III includes
lower limb deformities associated with specific metabolic, neuromuscular, and tumor-related
conditions, as well as skeletal dysplasias. Part IV covers specific congenital and developmental
disorders of the lower extremity. Finally, Part V explores various sequelae and complications
associated with lower extremity deformities in the growing child. All authors were encouraged
to incorporate relevant figures, tables, and highlight boxes to clearly deliver their message to
you, the reader. While I did spend many hours editing and making suggestions to each of the
corresponding authors, my role here was simply that of a facilitator.
When asked why I chose the field of pediatric orthopedics, I often respond by saying, “It
just feels right to make a child’s crooked leg straight,” perhaps not too far off from what
Nicholas Andry had in mind more than 250 years ago. While that may be too simplistic of an
answer, I do hope that this book will resonate with you and help guide your encounters with
these children and their caretakers.
Newark, NJ, USA Sanjeev Sabharwal, MD, MPH
ix
Acknowledgements
This work would not have been possible without the professional relationships that were
developed over the years with members of the Limb Lengthening and Reconstruction Society
(LLRS), Pediatric Orthopedic Society of North America (POSNA), and Pediatric Orthopedic
Society of India (POSI). I am thankful to the residents, staff, and faculty—in particular, the late
Fred Behrens, M.D.—of the Department of Orthopedics at the Rutgers, New Jersey Medical
School, who have taught me the value of patience and persistence. The constant support of the
Springer publishing team, especially Diane Lamsback and Kristopher Spring, was vital in
making this book a reality.
I am grateful to my parents, grandparents, mentors, and students for helping me recognize
the importance of integrity and hard work. Thanks to my dear wife, Ranjit, who for the past 30
years took care of essentially everything so that I could pursue an academic career in pediatric
orthopedics. I deeply appreciate our three children, Samir, Simran, and Sabhyta, for keeping
me honest and grounded.
xi
Contents
Part I General Principles and Techniques
1 Etiology of Lower Limb Deformity ........................................................................ 3

Viral V. Jain, Sarah Zawodny, and James McCarthy
2 Clinical Evaluation Including Imaging .................................................................. 15
Joseph J. Gugenheim
3 Decision Making in Lower Extremity Deformity Correction .............................. 37
Mark L. Miller and J. Eric Gordon
4 Growth Modulation for Angular and Length Correction .................................... 51
Peter M. Stevens
5 Physeal Bar Excision................................................................................................ 67
Karl E. Rathjen and Anthony I. Riccio
6 Acute Deformity Correction Using an Osteotomy ................................................ 79
Vrisha Madhuri and Sangeet Gangadharan
7 Gradual Deformity Correction ............................................................................... 105
David S. Feldman, Adam M. Kurland, and Abdel Majid Sheikh Taha
8 Hybrid Techniques for Limb Length and Deformity Correction ........................ 121
Mark T. Dahl and Chang-Wug Oh
9 Motorized Intramedullary Lengthening, an Emerging
Technology for Limb Length and Deformity Correction ..................................... 131
Mark T. Dahl
Part II Related Concepts and Management Options
10 Biomechanically Based Clinical Decision Making in Pediatric Foot

and Ankle Surgery ................................................................................................... 153
Jon R. Davids
11 Pediatric Joint Contractures ................................................................................... 163
Christopher Iobst
12 Physical Therapy During Limb Lengthening
and Deformity Correction: Principles and Techniques ........................................ 181
Anil Bhave, Erin Baker, and Mary Campbell
13 Amputation and Prosthetic Management:
Amputation as a Reconstructive Option ................................................................ 199
John A. Herring
xiii
xiv Contents
14 Working in Resource-Challenged Environments .................................................. 215

Scott C. Nelson and Hugh G. Watts
Part III Underlying Conditions
15 Metabolic Disorders ................................................................................................. 231

Mehmet Kocaoglu, I. Levent Eralp, and F. Erkal Bilen
16 Osteogenesis Imperfecta .......................................................................................... 255
François R. Fassier
17 Lower Limb Deformity in Neuromuscular Disorders:
Pathophysiology, Assessment, Goals, and Principles of Management ................ 267
Unni G. Narayanan
18 Arthrogryposis ......................................................................................................... 297
Reggie Hamdy and Noémi Dahan-Oliel
19 Limb Lengthening and Deformity Correction in Patients
with Skeletal Dysplasias .......................................................................................... 313
Mihir M. Thacker, Ellen Dean Davis, Colleen P. Ditro,
and William Mackenzie
20 Lower Extremity Benign Bone Lesions and Related Conditions ........................ 333
Lori Karol
21 Management of Juxtaphyseal Malignant Bone Tumors
Around the Knee Joint: New Concepts in Limb-Sparing Surgery ..................... 349
Hidenori Matsubara and Hiroyuki Tsuchiya
Part IV Congenital and Developmental Disorders
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery ....... 361

Dror Paley, David Y. Chong, and Daniel E. Prince
23 Fibular Hemimelia: Principles and Techniques of Management ........................ 427
John E. Herzenberg, Lior Shabtai, and Shawn C. Standard
24 Tibial Hemimelia ...................................................................................................... 455
Dror Paley and David Y. Chong
25 Congenital Pseudarthrosis of the Tibia .................................................................. 483
Corinna C. Franklin and Richard S. Davidson
26 Congenital Posteromedial Bowing of the Tibia ..................................................... 495
Benjamin Joseph, Hitesh Shah, and N.D. Siddesh
27 Controversies in Blount’s Disease........................................................................... 503
John G. Birch
Part V Sequelae and Complications
28 Methods to Enhance Bone Formation in Distraction Osteogenesis .................... 519

Hae-Ryong Song, Dong Hoon Lee, Seung-Ju Kim,
and Ashok Kumar Ramanathan
29 Residual Deformities of the Hip.............................................................................. 535
Shawn C. Standard and Daniel K. Ruggles
Contents xv
30 Posttraumatic Lower Limb Deformities in Children............................................ 569

Ashok N. Johari, Sandeep A. Patwardhan, and Taral Vishanji Nagda
31 Postinfectious Deformities of the Lower Limb ...................................................... 587
In Ho Choi
32 Iatrogenic Deformities ............................................................................................. 603
Austin T. Fragomen and S. Robert Rozbruch
Index .................................................................................................................................. 621

Contributors
Erin Baker, MPT Rubin Institute for Advanced Orthopedics, Sinai Hospital, Baltimore,
MD, USA
Anil Bhave, MS (PT) Rubin Institute for Advanced Orthopedics, Sinai Hospital, Baltimore,
MD, USA
F. Erkal Bilen, MD, FEBOT Department of Orthopedics and Traumatology, Istanbul
Memorial Hospital, Piyalepasa Bulvari Okmeydani, Istanbul, Marmara, Turkey
John G. Birch, MD, FRCS(C) Department of Orthopedics, Texas Scottish Rite Hospital for
Children, Dallas, TX, USA
Mary Campbell, DPT Rubin Institute for Advanced Orthopedics, Sinai Hospital, Baltimore,
MD, USA
In Ho Choi, MD, PhD Division of Pediatric Orthopedics, Seoul National University
Children’s Hospital, Seoul, Republic of Korea
David Y. Chong, MD Department of Orthopedic Surgery, University of Oklahoma Health
Sciences Center, Oklahoma City, OK, USA
Noémi Dahan-Oliel, PhD, OT Clinical Research/Rehabilitation, Shriners Hospital for
Children, Montreal, QC, Canada
Mark T. Dahl, MD Gillette Children’s Specialty Healthcare, University of Minnesota,
St. Paul, MN, USA
Jon R. Davids, MD Shriners Hospital for Children, Sacramento, CA, USA
Richard S. Davidson, MD Department of Orthopedic Surgery, The Children’s Hospital of
Philadelphia, Philadelphia, PA, USA
Ellen Dean Davis, MD Department of Orthopedics, St. Joseph’s Regional Medical Center,
Wayne, NJ, USA
Colleen P. Ditro, DNP Department of Orthopedic Surgery, Nemours Alfred I. duPont Hospital
for Children, Wilmington, DE, USA
I. Levent Eralp, MD Department Orthopedics and Traumatology, Istanbul Medical School,
University of Istanbul, Sisli, Istanbul, Turkey
François R. Fassier, MD Department of Orthopedics, Shriners Hospital for Children –
Canada, Montreal, QC, Canada H3G1A6
David S. Feldman, MD Department of Orthopedic Surgery, Division of Pediatric Orthopedics,
Hospital for Joint Diseases, New York University, New York, NY, USA
Austin T. Fragomen, MD Department of Orthopedics, Hospital for Special Surgery,
New York, NY, USA
xvii
xviii Contributors
Corinna C. Franklin, MD Shriners Hospital for Children, Philadelphia, PA, USA

Sangeet Gangadharan, DNB Orth Paediatric Orthopedics Unit, CMC Hospital, Christian
Medical College, Vellore, Tamil Nadu, India
J. Eric Gordon, MD Department of Pediatric Orthopedic Surgery, St. Louis Children’s
Hospital, St. Louis, MO, USA
Stuart A. Green, MD Department of Orthopedic Surgery, School of Medicine, University of
California, Irvine, Orange, CA, USA
Joseph J. Gugenheim, MD Texas Orthopedic Hospital, Houston, TX, USA
Reggie Hamdy, MD Division of Pediatric Orthopedics, Department of Orthopedics, Shriners
Hospital for Children, McGill University, Montreal, QC, Canada
John A. Herring, MD, FRCS (Ire, Hon) Department of Orthopedic Surgery, The Texas
Scottish Rite Hospital for Children, University of Texas Southwestern Medical School,
Dallas, TX, USA
John E. Herzenberg, MD, FRCSC International Center for Limb Lengthening, Rubin
Institute for Advanced Orthopedics, Sinai Hospital of Baltimore, Baltimore, MD, USA
Christopher Iobst, MD Department of Orthopedic Surgery, Nemours Children’ s Hospital,
Orlando, FL, USA
Viral V. Jain, MD Department of Orthopedic Surgery, Cincinnati Children’s Hospital Medical
Center, Cincinnati, OH, USA
Ashok N. Johari, MS(Orth), DOrth Department of Paediatric Orthopedics, Children’s
Orthopedic Centre, Mumbai, Maharashtra, India
Benjamin Joseph, MS Orth, MCh Orth Department of Paediatric Orthopedics, Aster
Medcity, Kochi, Kerala, India
Lori Karol, MD Department of Orthopedic Surgery, Texas Scottish Rite Hospital, Dallas,
TX, USA
Seung-Ju Kim, MD, PhD Department of Orthopedic Surgery, KEPCO Medical Center,
Seoul, Republic of Korea
Mehmet Kocaoglu, MD Department of Orthopedics and Traumatology, Istanbul Memorial
Hospital, Piyalepasa Bulvari Okmeydani, Istanbul, Marmara, Turkey
Adam M. Kurland, BA Department of Orthopedic Surgery, Division of Pediatric Orthopedics,
Hospital for Joint Diseases, New York University, New York, NY, USA
Dong Hoon Lee, MD, PhD Department of Orthopedic Surgery, Severance Children’s
Hospital, Seoul, Republic of Korea
William Mackenzie, MD, FRCSCC, FACS Department of Orthopedic Surgery, Nemours
Alfred I. duPont Hospital for Children, Wilmington, DE, USA
Vrisha Madhuri, D Orth, MS Orth, MCh Orth (L pool) Paediatric Orthopedics Unit,
CMC Hospital, Christian Medical College, Vellore, Tamil Nadu, India
Hidenori Matsubara, MD, PD Department of Orthopedic Surgery, Kanazawa University
Hospital, Kanazawa, Ishikawa, Japan
James McCarthy, MD Department of Orthopedic Surgery, Cincinnati Children’s Hospital
Medical Center, Cincinnati, OH, USA
Mark L. Miller, MD Department of Pediatric Orthopedic Surgery, St. Louis Children’s
Hospital, St. Louis, MO, USA
Contributors xix
Taral Vishanji Nagda, MS, DNB, D Ortho Department of Pediatric Orthopedics, Hinduja
Hospital Mumbai, Mumbai, Maharashtra, India
Unni G. Narayanan, MBBS, MSc, FRCS(C) Department of Surgery, The Hospital for Sick
Children, University of Toronto, Toronto, ON, Canada
Scott C. Nelson, MD Department of Orthopedic Surgery, Loma Linda University School of
Medicine, Loma Linda, CA, USA
Chang-Wug Oh, MD Department of Orthopedic Surgery, Kyungpook National University
Hospital, Daegu, South Korea
Dror Paley, MD, FRCSC St. Mary’s Medical Center, Paley Advanced Limb Lengthening
Institute, West Palm Beach, FL, USA
Sandeep A. Patwardhan, MS (Orth), D Orth Department of Pediatric Orthopedics, Sancheti
Institute for Orthopedics and Rehabilitation, Pune, Maharashtra, India
Daniel E. Prince, MD, MPH Paley Advanced Limb Lengthening Institute, West Palm Beach,
FL, USA
Ashok Kumar Ramanathan, MBBS, MS (Ortho) Department of Orthopedic Surgery,
Madurai Medical College, Madurai, Tamil Nadu, India
Karl E. Rathjen, MD Department of Orthopedic Surgery, Texas Scottish Rite Hospital for
Children, University of Texas Southwestern Medical Center, Dallas, TX, USA
Anthony I. Riccio, MD Department of Orthopedic Surgery, Texas Scottish Rite Hospital for
Children, University of Texas Southwestern Medical Center, Dallas, TX, USA
S. Robert Rozbruch, MD Department of Orthopedics, Hospital for Special Surgery,
New York, NY, USA
Weill Cornell Medical College, Cornell University, New York, NY, USA
Daniel K. Ruggles, DO Department of Orthopedic Surgery, Nationwide Children’s Hospital,
Columbus, OH, USA
Sanjeev Sabharwal, MD, MPH Department of Orthopedics, Rutgers-New Jersey Medical
School, Newark, NJ, USA
Lior Shabtai, MD Department of Pediatric Orthopedics, Tel Aviv Sourasky Medical Center,
Dana Children’s Hospital, Tel Aviv, Israel
Hitesh Shah, MS (Orthopedics), DNB (Orthopedics) Department of Orthopedics, Kasturba
Medical College, Kasturba Hospital, Manipal University, Manipal, Karnataka, India
N.D. Siddesh, MS (Orthopedics), FRCS (Glasg) Department of Orthopedics, Kasturba
Medical College, Guro Hospital, Manipal University, Manipal, Karnataka, India
Hae-Ryong Song, MD, PhD Department of Orthopedic Surgery, Korea University Medical
Center, Seoul, Republic of Korea
Shawn C. Standard, MD International Center for Limb Lengthening, Rubin Institute for
Advanced Orthopedics, Sinai Hospital of Baltimore, Baltimore, MD, USA
Peter M. Stevens, MD Department of Orthopedics, University of Utah, Salt Lake City, UT, USA
Abdel Majid Sheikh Taha, MD Department of Orthopedic Surgery, Division of Pediatric
Orthopedics, Hospital for Joint Diseases, New York University, New York, NY, USA
Mihir M. Thacker, MD Department of Orthopedic Surgery, Nemours Alfred I. duPont
Hospital for Children, Wilmington, DE, USA
xx Contributors
Hiroyuki Tsuchiya, MD, PD Department of Orthopedic Surgery, Kanazawa University

Hospital, Kanazawa, Ishikawa, Japan
Hugh G. Watts, MD Department of Orthopedic Surgery, Shriners Hospital for Children,
Los Angeles, CA, USA
Sarah Zawodny, MD Department of Orthopedic Surgery, Cincinnati Children’s Hospital
Medical Center, Cincinnati, OH, USA
Part I
General Principles and Techniques
Etiology of Lower Limb Deformity
1
Viral V. Jain, Sarah Zawodny, and James McCarthy
slipped capital femoral epiphysis (SCFE). These same disor-

Introduction ders can render the chondrocytes more susceptible to com-
pressive forces, leading to angular deformities.
Lower limb deformities can consist of any combination of
length inequality, angular deformity, and/or asymmetric Rickets
girth. While the list of possible etiologies is quite extensive, Rickets is a clinical manifestation of defective mineralization
categorizing them can help simplify diagnosis and treat- of the long bone physes due to inadequate levels of calcium or
ment. Many investigators have broadly classified lower phosphate in children. Osteomalacia, the defective mineral-
limb deformity into congenital and acquired, with some ization of osteoid at cortical and trabecular surfaces, is seen in
including a developmental group. This chapter focuses on both children and adults. There are many different forms of
the etiologies that are most clinically relevant to the ortho- rickets that result in very similar radiographic and clinical
pedic surgeons. manifestations (Table 1.2). Patients may have decreased lon-
We have divided the causes of pediatric lower limb defor- gitudinal growth and angular deformities [3] (Fig. 1.1).
mities into four broad categories: namely underlying condi-
tions, congenital, developmental, and acquired deformities Renal Osteodystrophy
from sequelae of disease or complications (Table 1.1). Most Renal osteodystrophy is the combination of rickets and
of these disorders are discussed in greater details in the sub- hyperparathyroidism that occurs secondary to end-stage
sequent chapters. renal disease. The findings of rickets are explained by the
failing kidney being unable to produce a sufficient amount of
1, 25-dihydroxyvitamin D3. Additionally, the dysfunction of
Underlying Generalized Conditions the tubular system decreases clearance of phosphate and
causes secondary hyperparathyroidism, which manifests as
Metabolic Disorders brown tumors and subperiosteal erosions [3].
The deformity that results from these metabolic disorders
Certain metabolic disorders and endocrinopathies can affect depends on the stage along the physiologic varus/valgus spec-
the maturation of growth plate chondrocytes by the alteration trum that the child is at the age of onset of the disorder. The
of normal regulatory signals or by disturbance of the func- onset of nutritional rickets and inherited disorders is generally
tional matrix components necessary for new bone formation. seen during infancy and therefore they produce genu varum.
Some endocrine disorders such as hypothyroidism and On the other hand, renal osteodystrophy usually causes genu
growth hormone deficiency [1] and metabolic disorders valgum because acquired renal failure occurs later when chil-
including renal osteodystrophy [2] can mechanically weaken dren are more likely in the physiologic valgus age group [4].
the physis, predisposing to pathologic conditions such as
Genetic Disorders
V.V. Jain, MD (*) • S. Zawodny, MD • J. McCarthy, MD
Department of orthopedic Surgery, Cincinnati Children’s Hospital Osteogenesis Imperfecta
Medical Center, 3333 Burnet Avenue MLC# 2017, Cincinnati, OH
Osteogenesis imperfecta is caused by genetic mutations that
45229, USA
e-mail: [email protected]; [email protected]; james. affect the quantity or quality of collagen produced. Many
[email protected] distinct mutations have been identified and can thus create a
S. Sabharwal (ed.), Pediatric Lower Limb Deformities, 3

DOI 10.1007/978-3-319-17097-8_1, © Springer International Publishing Switzerland 2016
4 V.V. Jain et al.
Table 1.1 General categorization of the types of lower limb deformi-

ties in children based on etiology
Underlying Metabolic: rickets, renal osteodystrophy
conditions Genetic disorders: osteogenesis imperfecta,
neurofibromatosis
Neuromuscular: cerebral palsy,
Charcot-Marie-Tooth arthrogryposis
Skeletal dysplasias
Benign tumors: fibrous dysplasia, Ollier’s,
multiple hereditary exostoses
Malignant tumors
Inflammation: rheumatoid arthridities,
hemophilic arthropathy
Vascular anomalies
Congenital Congenital femoral deficiency (proximal
focal femoral deficiency)
Congenital fibular deficiency (fibular
hemimelia)
Congenital tibial deficiency (tibial hemimelia)
Tibial dysplasia: congenital pseudarthrosis,
posteromedial bowing
Hemihypertrophy
Amniotic band syndrome
Fig. 1.1 Genu valgum in a 7-year old-female. The etiology was nutri-
Congenital knee dislocation tional rickets. Image courtesy Dr. Hitesh Shah
Developmental Genu varum
Blount’s disease
Genu valgum
Neurofibromatosis
Acquired: sequelae Residual hip deformity: developmental
and complications dysplasia of the hip, slipped capital femoral Neurofibromatoses (NF) are a group of genetic multisystem
epiphysis, Legg-Calve-Perthes, avascular disorders involving products of all three germ lines: neu-
necrosis roectoderm, mesoderm, and endoderm. The most com-
Posttraumatic: Cozen fracture mon and orthopedically relevant form is neurofibromatosis
Post-infectious type 1 (NF1) previously known as von Recklinghausen
Iatrogenic
disease, which involves predominantly peripheral ner-
vous system although it involves cells of mesodermal ori-
Table 1.2 Rickets and related disorders
gin as well.
NF1 is one of the most common autosomal dominant
Type Cause
disorders with a prevalence rate of 1:4,000 [6]. It occurs
Nutritional Vitamin D deficiency
almost equally in all ethnic groups. Approximately 50 %
Dietary calcium deficiency
of all cases of NF1 are due to new mutations. The NF1
Combination of both
Gastrointestinal Poor absorption due to disease
gene mutations have variable expression—the clinical
(gluten sensitivity, Crohn’s/ manifestations of NF1 range from subclinical to severe.
ulcerative colitis, short gut) However, it has nearly 100 % penetrance in adult individu-
End-organ insensitivity Insensitivity to vitamin D3 als. Thus, an adult carrying the mutation will eventually
Vitamin D dependent 1 alpha hydroxylase deficiency exhibit some clinical feature of the disease. The NF1 gene
X-linked hypophosphatemia PHEX gene defect, wasting of is located on the long arm of chromosome 17 (17q) and of
phosphate in kidney
relatively large size (350,000 base pairs). This may explain
Renal tubule abnormality Kidneys waste many molecules,
particularly phosphate
the large rate of new mutation associated with this disor-
Hypophosphatasia Alkaline phosphatase deficiency der. The gene product neurofibromin acts as a tumor-sup-
(gene mutation on chromosome 1) pressor gene [7].
Renal osteodystrophy Renal failure Several distinguishing clinical features of NF1 help to
make the diagnosis of the condition (Fig. 1.2; Table 1.3).
There are a number of clinical issues associated with NF1
wide spectrum of phenotypic manifestations. Those who are that are important from an orthopedic standpoint. These
more severely affected have extensive bony fragility that include tibial dysplasia and hemihypertrophy leading to leg
leads to numerous fractures, short stature, and angular defor- length discrepancies and limb deformities, which are dis-
mities of the long bones and the spine [5]. cussed later in the chapter.
1 Etiology of Lower Limb Deformity 5
Fig. 1.3 A 17-year-old male patient with Charcot-Marie-Tooth dis-

ease. Note the cavus deformity of the foot bilaterally
Fig. 1.2 A 3-year-old female patient with neurofibromatosis type 1

(NF1). Note the café au lait spots on the abdomen. Café au lait spots are
the most easily noticeable feature of NF1 Neuromuscular Disorders
Cerebral Palsy
Depending on the distribution and severity of their underlying
Table 1.3 Diagnostic criteria of neurofibromatosis type 1 spasticity, children with cerebral palsy can have a wide
More than six café au lait spots measuring at least 15 mm in adults array of rotational, angular deformities and joint contractures.
and 5 mm in children Excessive femoral anteversion is quite common in spastic
Two or more neurofibromas of any type or one plexiform diplegia and is associated with increased tone in the medial
neurofibroma hamstrings and adductors. Femoral anteversion can also be
Freckling in the axillary or inguinal regions
accompanied by compensatory tibial external rotation in
Optic glioma
diplegic or by internal tibial rotation in a hemiplegic patient.
Two or more Lisch nodules (i.e., iris hamartomas)
Muscle tone imbalances also cause deformities of the hips,
A distinctive bony lesion, such as sphenoid wing dysplasia, or
thinning of the cortex of a long bone with or without pseudarthrosis knees, ankles, and feet [10].
A first-degree relative with NF-1, as suggested by these criteria
Charcot-Marie-Tooth Disease
Charcot-Marie-Tooth disease (CMT) is the most common of
the seven types of hereditary motor and sensory neuropathies
Another important manifestation of orthopedic interest is (HMSN). The two general types are demyelinating and axo-
development of spinal deformities. Spinal deformities are nal, but they are not distinguishable by clinical exam. As
the most common orthopedic manifestation of NF1. True research has identified specific locations of different genetic
incidence of spinal deformities in NF1 is not known. It is abnormalities, the number of described subtypes has
quoted as from 2 to 36 % in the literature [8, 9]. In the NF increased. Type IA CMT disease is the most common sub-
clinic at our institution, it is 23 % [6]. Traditionally, two type and occurs via autosomal dominant transmission of a
forms of spinal deformities are described in NF1: dystrophic peripheral myelin protein 22 gene duplication on chromo-
and non-dystrophic. Non-dystrophic curves are similar to some 17. The X-linked dominant form of disease is second
idiopathic curves with some exceptions. The dystrophic most common and is attributed to a mutation of the connexin-
changes in NF1 include rib penciling, vertebral scalloping, 32 gene. In general, demyelination of peripheral nerves and
vertebral wedging, spindling of transverse processes, and degeneration of the dorsal root ganglia and dorsal column of
enlarged intervertebral foramina. the spinal cord in the central nervous system occur, leading
The spinal deformities tend to develop early in the life; to progressive muscle atrophy, loss of proprioception, and
therefore all preadolescent children with NF1 should be loss of deep tendon reflexes. The most common deformity is
evaluated by scoliosis screening or the Adam’s forward-bend worsening cavovarus foot posture with claw toes (Fig. 1.3)
test to rule out the presence of a spinal deformity. due to selective intrinsic and extrinsic (tibialis anterior
6 V.V. Jain et al.
and peroneus brevis) muscle weakness and imbalance. The

gastrocnemius-soleus complex is also weak and contracted,
contributing to the cavovarus deformity and to the thin “stork
leg.” The hands can be affected and, less commonly, patients
can develop scoliosis or hip dysplasia [11].
Arthrogryposis Multiplex Congenita
Arthrogryposis multiplex congenita (multiple congenital

contractures) is actually a symptom complex of deformed,
rigid, and sometimes dislocated joints that can occur as part
of at least 300 different disorders. Amyoplasia is the classic
and most common form of arthrogryposis that involves all Fig. 1.4 A 14-year-old male patient with juvenile rheumatoid arthritis
four extremities. It is a nonprogressive syndrome character- (JRA). Note enlarged small joints of the hands. Image courtesy
Dr. Hitesh Shah
ized by extremities that appear cylindrical, fusiform, or cone
shaped due to atrophied or absent muscles, decreased subcu-
taneous tissue, and diminished skin creases. In the lower often including the nearby physis that leaves the child with
limb, the foot deformities commonly seen include a rigid an increasing length discrepancy with growth.
clubfoot or vertical talus. The knees may have either flexion Polyostotic diseases such as fibrous dysplasia and Ollier’s
or extension contractures. The hips are also very often disease are known to cause severe limb deformities. In fibrous
involved, usually with flexion, abduction contractures, and dysplasia, expanding fibro-osseous tissue in the intramedul-
hip dislocation noted in 15–30 % of patients with amyopla- lary canal causes repetitive microfractures, pain, and progres-
sia. These clinical findings result from decreased fetal move- sively bowed long bone deformities such as the classic
ment. Fetal akinesia is due to failure of skeletal muscle shepherd’s crook appearance of the femur [14]. Ollier’s dis-
development, often related to abnormal development of ante- ease, a form of multiple enchondromatosis, produces islands
rior horn cells in the spinal cord [12]. of cartilage in the diaphyseal and metaphyseal regions that
encroach upon the physis to cause inhibition of growth.
Ollier’s is often unilateral, so it is not only associated with
Skeletal Dysplasias angular deformities such as distal femoral varus, but also
tends to cause severe lower limb length discrepancies [15].
Children with various skeletal dysplasias present clinically
with short stature and variable growth disturbances in the
trunk and extremities. Specific deformities are typical for Inflammatory Disorders
each type of dysplasia and are caused by specific structural
abnormalities within the bones and cartilage. The underlying Children with inflammatory arthritis such as rheumatoid
genetic mutation and mode of transmission, autosomal domi- conditions have chronic joint inflammation that causes pain
nant for most but not all, are known for the majority of bone and muscle spasms, which eventually results in hip and knee
dysplasias. In the lower extremities, specific angular deformi- flexion contractures, growth disturbances, and angular
ties and involved joints are characteristic for each diagnosis. deformities. However, these patients will have typical fea-
tures such as small joint involvements in upper extremity
during earlier stage of the disease, which provides a clue
Benign and Malignant Tumors to the diagnosis (Fig. 1.4). In children with hemophilic
arthropathy, the repeated intra-articular bleeding causes
Tumors, both benign and malignant, have the potential to synovitis that ultimately results in joint contractures second-
cause limb deformities. Solitary lesions, even benign simple ary to cartilage degradation from both direct compression
bone cysts, can be complicated by growth arrest due to from the hypertrophic synovial pannus and proteolytic
pathologic fracture, juxtaposition to the physis, or injury dur- enzyme release [16]. Along with the intra-articular hemor-
ing therapeutic curettage or injection [13]. The risk of growth rhage, there is hyperemia that often stimulates growth,
disturbance is inherently higher with malignant tumors as resulting in angular deformity if there is asymmetric
they are much more aggressive. Treatment of malignancies involvement of the adjacent physis or limb length discrep-
may require a wide resection, with or without an amputation, ancy if symmetric involvement occurs [17].
Table 1.4 Common overgrowth and undergrowth syndromes

Common overgrowth syndromes
Beckwith–Wiedemann Macroglossia, macrosomia, midline Mutation of 11p15, leading to overactivity
syndrome abdominal wall defects, ear creases or ear of the IGF-2 gene
pits, and neonatal hypoglycemia
Proteus syndrome Progressively worsening macrodactyly, Somatic activating mutation in AKT1
hemihypertrophy, thickening of the skin, kinase in a mosaic manner
lipomas, subcutaneous tumors, verrucae,
epidermal nevus and macrocephaly
Neurofibromatosis type 1 Café-au-lait spots, cutaneous neurofibroma, Mutation of NF1 gene located on
axillary freckling, optic glioma, Lisch chromosome 17 leading to disruption of
nodules, vertebral scalloping, tibial dysplasia Ras signaling controlled by neurofibromin
Klippel-Trenaunay Nevus flammeus (port-wine stain), venous In some patients only: angiogenic factor
syndrome and lymphatic malformations, and soft-tissue VG5Q mutation. Translocation (5:11, 8:14)
and bony hypertrophy of the affected limb
Common undergrowth syndromes
Russell-Silver syndrome Short stature; small, triangular faces; In 10 % patients: maternal uniparental
disproportionately normal head disomy on chromosome 7
circumference; clinodactyly
Cutis marmorata Generalized or localized reticulated Unknown
telangiectatica congenita cutaneous vascular mottling, macrocephaly,
glaucoma, hemiatrophy, vascular anomalies
Vascular Malformations
Various generalized and local vascular malformations are

another group of disorders that can cause significant limb
length inequality of up to 10 cm in addition to hypertrophy in
girth and skin alterations [18].
Congenital
Hemihypertrophy and Hemiatrophy
There are no clear guidelines in literature as to what consti-

tutes hemihypertrophy or hemiatrophy. It is also difficult to
diagnose these conditions as there exists a normal variation
between two sides of the body. However, when clinically sig- Fig. 1.5 A 9-year-old female with idiopathic hemihypertrophy of right
nificant differences are found, it is important to investigate lower extremity. The patient did not develop any neoplasm
further, as most of them would follow under one of the con-
ditions discussed below (Table 1.4).
malities are very similar to genetic abnormalities found with
Idiopathic Hemihyperplasia Beckwith–Wiedemann syndrome. There is some evidence
Also known as idiopathic hemihypertrophy (IH) or non- that the patients who have above-mentioned genetic abnor-
syndromic hemihypertrophy, this is a condition of unknown malities have a higher likelihood of developing embryonal
origin (Fig. 1.5). Its importance lies in the fact that IH can be tumors [21].
associated with embryonal tumors [19] such as Wilm’s tumor,
adrenal cell carcinoma, and hepatoblastoma in some patients. Beckwith–Wiedemann Syndrome
In up to 80 % of patients with IH, no genetic abnormalities Beckwith–Wiedemann syndrome [22, 23] is characterized
are found. When genetic abnormalities are found, these most by neonatal hypoglycemia, macroglossia, visceromegaly,
commonly are uniparental paternal disomy of 11p15, loss of and hemihypertrophy [24]. Most patients have a birth weight
maternal methylation of KCNQ1OT1, or hypermethylation of above the 90th percentile. Deletions and translocations in
H19. All these are mosaic abnormalities [20]. These abnor- chromosome 11, near the locus of insulin-like growth factor
8 V.V. Jain et al.
Fig. 1.6 (a, b) An 18-month-old

male patient with focal femoral
deficiency. Note short femur and
apparent pseudarthrosis/absence
of proximal femur on the left side
(IGF) II, can be identified in 70 % of the patients. As with IH, anomalies, with abnormalities of the hip, hand, or spine
patients with Beckwith–Wiedemann syndrome are more occurring alone or in various combinations [26].
prone to having embryonal tumors. The overall rate is 7–9 %
in patients with Beckwith–Wiedemann syndrome, but those Congenital Fibular Deficiency
with the presence of hemihypertrophy are at substantially Also known as fibular hemimelia, this condition includes
greater risk (24–27 %) [20, 24, 25]. partial or complete absence of fibula along with variable
As molecular techniques cannot always identify all the involvement of postaxial (lateral half) bones of the lower
patients with tumor risk (especially in IH), serial abdominal extremity. The exact etiology is not known and the cases are
ultrasound every 3 months up to age 7 and evaluation by a sporadic. Up to 15 % of patients have associated femoral
clinical geneticist is recommended [20] for both conditions. anomalies [27].
When the fibula is partially absent/short, the spectrum of
abnormalities include ipsilateral shortening of femur, hypo-
Congenital Deficiency of the Long Bones plastic lateral femoral condyle, cruciate ligament deficiency,
ball and socket ankle, tarsal coalitions, and absent lateral
Longitudinal Tibial Deficiency rays. Children with hypoplastic or absent fibula often have
Commonly known as tibial hemimelia, this is a syndrome of associated tibial shortening with anteromedial bowing and
partial to complete absence of the tibia at birth. In most cases marked equinovalgus deformity.
the etiology is unknown. In a minority of cases, there is an
autosomal dominant pattern. These specific entities include: Congenital Femoral Deficiency
1. Warner syndrome: Tibial hemimelia–foot polydactyly– This is a condition of variable deficiency of the femur. In its
triphalangeal thumb syndrome mildest form the femur is short, while in severe case the
2. Tibial hemimelia-micromelia-trigonobrachycephaly proximal femur is absent and the distal femoral shaft creates
syndrome a pseudoarticulation at the hip (Fig. 1.6). In some cases there
3. Tibial hemimelia diplopodia syndrome is radiographic discontinuity noted between femoral neck
4. Tibial hemimelia-split hand and foot syndrome and shaft. Similar to other long bone deficiencies, the etiol-
The heritable tibial hemimelia is usually bilateral and ogy is unknown. However, there is an autosomal dominant
associated with anomalies of toes and hand. In a review by inheritance noted in femoral hypoplasia–unusual facies syn-
Schoenecker et al., 79 % of patients had associated congenital drome [28].
Congenital Bowing of the Tibia
Anterolateral Bowing of the Tibia

“Congenital” Tibial Dysplasia
“Congenital” tibial dysplasia is associated with the develop-
ment of pseudarthrosis of the tibia, which often develops
subsequently and is usually not present at birth. As a result,
the term dysplasia will be used instead of “congenital pseud-
arthrosis of the tibia.” It is a very rare disorder affecting 1 in
140,000–190,000 [30–32].
Anterolateral tibial bowing and pseudarthrosis are associ-
ated with NF 1 in up to 55 % of the patients. Some authors
have found an even higher association [32]. Fibrous dyspla-
Fig. 1.7 A newborn male with congenital dislocation of the right knee. sia is present in up to 15 % of patients. The amniotic band
The patient was initially treated by serial splinting and later with a syndrome (causing local constriction, eventually leading to
quadriceps release pseudarthrosis) is also rarely associated with this type of
tibial dysplasia [11].
Congenital Dislocation of the Knee
Congenital Posteromedial Bowing of the Tibia
The proper terminology for this condition is congenital knee The deformity is present at birth and the tibial bowing is
hyperextension, with knee dislocation representing the most accompanied by a calcaneus foot and dorsiflexion contracture
severe variety (Fig. 1.7). The incidence of congenital hyper- of the ankle. It is secondary to intrauterine malposition [33]
extension is less than 1 per 1,000 [29]. Bilateral congenital (Fig. 1.8).
dislocation of the knee (CDK) is usually associated with neu-
romuscular syndromes such as Larsen’s or Beals’ syndrome. Anteromedial Bowing of the Tibia
Persistent hyperflexion of the hip is indicative of ipsilateral This deformity of tibia is commonly associated with con-
hip dislocation, which is commonly presented in neurologic genital fibular deficiency, as noted in the previous section.
conditions such as arthrogryposis and spinal dysraphism.
The most commonly accepted theory for etiology of CDK
is abnormal fetal positioning. Subsequent akinesia (second- Developmental Conditions
ary to the neuromuscular conditions mentioned above) causes
hyperextension leading to quadriceps atrophy and fibrosis. It has long been recognized that there are periods of physio-
logic genu varum and valgum in early childhood develop-
ment. Newborns have genu varum which transitions to
Congenital Coxa Vara neutral alignment just before age 2, and then to maximum
valgus around age 3 or 4. The valgus gradually decreases
Congenital coxa vara is a developmental abnormality char- until the age of 7 or 8, when it becomes stable at the 5–7° of
acterized by a primary cartilaginous defect in the femoral tibiofemoral valgus seen in adults [34].
neck with an abnormal decrease in the femoral neck–shaft
angle, shortening of the femoral neck, relative overgrowth of
the greater trochanter, and shortening of the affected lower Genu Varum
limb. It is rare with an incidence estimated to be 1 in 25,000
live births. When present bilaterally, the condition is often Physiologic Genu Varum
associated with other autosomal dominant disorders such as Physiologic genu varum is most common and has been
cleidocranial dysostosis, spondylometaphyseal dysplasia, defined radiographically as more than 10° of bilateral
and metaphyseal dysostosis, Jansen type. Isolated congenital femoral-tibial varus after 18 months of age [35]. The
coxa vara is also believed to have a genetic etiology as it can entire lower limb appears bowed with both the distal
occur in families and in twins. The coxa vara associated with femur and proximal tibia physes and epiphyses appearing
congenital femoral deficiency is considered a separate entity normal and symmetric. These children often walk early,
as it does not share similar radiographic and clinical findings before 12 months of age, and a family history of bowlegs
with congenital coxa vara. is common.
10 V.V. Jain et al.
Fig. 1.8 (a, b) A 9-year-old male

patient with congenital
posteromedial tibial bowing. This
patient does not have significant
calcaneus deformity. Image
courtesy Dr. Hitesh Shah
Tibia Vara/Blount’s Disease

Tibia vara occurs when the apex of deformity occurs at the
site of growth retardation at the posteromedial aspect of the
proximal tibial physis and results in persistent or progressive
bowing. There are two or three forms of the disease described
by different authors based on age of onset, but all agree that
the most common cause of pathologic genu varum is infan-
tile tibia vara or Blount’s disease (Fig. 1.9). In 1937, Blount
characterized the deformity as an abrupt angulation just
below the proximal tibial physis, an irregular physeal line,
and a wedge-shaped epiphysis with a “beak” at the medial
metaphysis. Apparent lateral subluxation of the proximal
end of the tibia is often present [36].
It can sometimes be difficult to distinguish physiologic
genu varum from early stages of infantile Blount’s disease
as they seem to occur along the same continuum of bilateral
bowing. There may be a positive family history, but Blount’s
disease is considered a developmental disorder rather than
congenital since the radiographic features are not seen in
patients younger than 1 year [37]. Children with infantile
Blount’s are typically early walkers and often obese, plac-
ing enough force on the medial proximal tibial physis to
impair normal longitudinal growth of the medial proximal Fig. 1.9 A 4-year-old male patient with Blount’s disease, which is
tibial physis. Finite-element analysis of the knee has shown more common in patients of African American origin
that a compressive force sufficient to retard physeal growth
by the Heuter-Volkmann principle is produced on the A distinct form of tibia vara occurring later in childhood
medial tibial plateau of a 2-year-old in the 90th percentile has been called late-onset or adolescent Blount’s disease.
for body weight and with a 20° varus deformity during single- Frequently, these patients are morbidly obese males in their
limb stance [38]. early adolescence without any known insult to the proximal
Table 1.5 Common causes of genu varum

Physiologic
Blount’s disease
Focal fibrocartilaginous dysplasia
Metabolic bone disease: rickets
Renal osteodystrophy
Posttraumatic
Post-infectious
Congenital deficiencies
Skeletal dysplasias: metaphyseal chondrodysplasia,
spondyloepiphyseal dysplasia, multiple epiphyseal
dysplasia, achondroplasia, pseudoachondroplasia
Neoplastic disease: fibrous dysplasia
Osteogenesis imperfecta
Table 1.6 Common causes of genu valgum

Unilateral Posttraumatic physeal injury
Proximal tibia metaphyseal fracture (Cozen)
Neoplastic:
Osteochondroma
Focal fibrocartilaginous dysplasia
Fibrous dysplasia
Enchondromatosis (Ollier’s) Fig. 1.10 A 6-year-old male status 1 year postproximal tibial metaphy-
Bilateral Physiologic seal fracture on the right who developed a genu valgum deformity. The
Metabolic: rickets patient was observed serially, and eventually the proximal tibia was
Skeletal dysplasias: remodeled completely
Chondroectodermal dysplasia (Ellis-van Creveld)
Pseudoachondroplasia
Morquio (MPS IV)
Spondyloepiphyseal dysplasia
Acquired Sequelae and Complications
Neoplastic: multiple hereditary exostoses
Residual Hip Deformities
tibia to explain the medial physeal growth retardation around Lower limb deformity or length discrepancy can occur as a
the knee. Similar to the infantile form, the etiology is not result of several acquired conditions that are often evident in
fully resolved, but the most popular theory is mechanical the child’s history. A favorable outcome of pediatric hip con-
injury from excess weight, with or without preexisting mild ditions such as developmental dysplasia of the hip (DDH),
varus deformity [39]. SCFE, and Perthes disease is largely dependent on the con-
Proximal focal fibrocartilaginous dysplasia is a rare, idio- gruency of the hip joint and avoidance of avascular necrosis
pathic disorder with presentation similar to infantile Blount’s. (AVN). Residual hip deformity due to growth disturbances
Clinically, the abrupt varus deformity is more distal than the and AVN presents as coxa vara, coxa valgum, or short femo-
knee joint line and corresponds to cortical sclerosis at the ral neck depending on the location and extent of premature
medial metaphyseal-diaphyseal junction of the proximal capital femoral epiphyseal closure as well as overgrowth of
tibia at the insertion of the pes anserinus [40]. the greater trochanter that causes an abductor lurch.
There are numerous other non-physiologic causes of genu
varum (Table 1.5) that can be distinguished on radiographs.
Physeal Fractures
Genu Valgum Fractures involving the physis are at risk for growth arrest
due to direct injury to the chondrocytes and premature phy-
Excessive physiologic genu valgum is frequently idiopathic. seal closure. The Salter-Harris classification in combination
It is pathologic when the tibiofemoral angle increases after with the mechanism of injury and degree of initial displace-
7 years of age and walking becomes difficult as the knees rub ment can predict physeal disruption. In Salter-Harris type I
together. Although it is less common than genu varum, there and II injuries, the separation occurs primarily through the
is still a wide range of causes [41] (Table 1.6, Fig. 1.10). hypertrophic zone in the horizontal plane. Growth slows at
12 V.V. Jain et al.
least temporarily, but the physis can appear histologically and securing grafts across growth plates for anterior cruciate
normal by 25 days [42]. In types III and IV, the fracture ligament reconstructions in skeletally immature patients can
passes through all layers of the physis, creating a potential also cause genu valgum, recurvatum, or overgrowth [44].
conduit between the bone of the metaphysis and epiphysis. The purpose of this chapter was to provide an introduc-
Blood vessels invade this gap, allowing migration of osteo- tion to the vast number of etiologies of lower limb deformi-
blasts to form a bony bar which tethers further longitudinal ties and to organize them into a logical and clinically relevant
growth [42]. This can manifest as a leg length discrepancy or framework. The remaining chapters in this book will delve
an angular deformity depending on the location and size of further into the diagnosis, classification, and management of
the physeal arrest or bony bar. specific disorders.
Acknowledgements The authors would like to thank Dr. Hitesh Shah

Non-physeal Fractures from Manipal, India, for providing clinical photographs depicted in this
chapter.
Fractures do not necessarily have to directly involve the

growth plate to result in a deformity. Long-bone fractures,
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1961;43-A:625–42. therapeutic alternatives. J Am Acad Orthop Surg. 1995;3:275–83.
28. Burn J, Winter RM, Baraitser M, Hall CM, Fixsen J. The femoral 42. Levine AM, Drennan JC. Physiological bowing and tibia vara. The
hypoplasia-unusual facies syndrome. J Med Genet. 1984;21: metaphyseal-diaphyseal angle in the measurement of bowleg defor-
331–40. mities. J Bone Joint Surg Am. 1982;64:1158–63.
29. Charif P, Reichelderfer TE. Genu recurvatum congenitum in the 43. Blount WP. Osteochondrosis deformans tibiae. J Bone Joint Surg
newborn: its incidence, course, treatment, prognosis. Clin Pediatr Am. 1937;19:1.
(Phila). 1965;4:587–94. 44. Langenskioeld A, Riska EB. Tibia vara (osteochondrosis defor-
30. Andersen KS. Congenital pseudarthrosis of the leg. Late results. mans tibiae): a survey of seventy-one cases. J Bone Joint Surg Am.
J Bone Joint Surg Am. 1976;58:657–62. 1964;46:1405–20.
Clinical Evaluation Including Imaging
2
Joseph J. Gugenheim
Do the parents or grandparents want to “do something

Introduction before it is too late,” or do they want treatment, even if it is
not indicated, because physicians prescribed treatment in the
The majority of pediatric lower extremity deformities pre- past to family members of previous generations [3]? These
senting for evaluation are normal physiologic angular and/or situations require a clear explanation with great patience
rotational conditions that resolve spontaneously without why treatment is not necessary. The author, who has been
expensive and unnecessary treatment, diagnostic imaging, or practicing for almost 40 years, likes to humorously explain
follow-up. However, limb length discrepancy and asymmet- that when he began his practice, we physicians treated these
ric and/or severe angular deformities often do require imag- conditions with special shoes, braces, exercises, shoe modi-
ing and follow-up. Thus, the clinician must distinguish fications, and inserts and had 100 % excellent results. We
between the physiologic angular and rotational conditions, congratulated ourselves for being brilliant physicians for
which require only reassurance to the family, and deformity producing such excellent results until we learned that the
secondary to pathologic conditions, which requires follow-up children straightened up, not because of us, but in spite of us!
and possible intervention. The Pediatric Orthopedic Society By explaining that the evaluation of any treatment in the
has defined normal as that which occurs within two standard practice of medicine requires comparing treated to untreated
deviations of the mean [1]. Angular or rotational conditions patients and that such evaluations show that treatment in
within this range are considered variations; those outside this many conditions does not affect the outcome, accelerate the
range are considered deformities [2]. The common use of the natural resolution, or prevent later disability, the physician
term deformity to include both variations and deformities is can explain to the parents that treatment and further testing
used in this chapter. Accurate assessment of a child’s lower are not indicated [4].
extremities requires quantifying the deformity and knowing Are the parents worried about cosmesis? Are they worried
the mean value and standard deviations for the child’s age. about future athletic performance? Are they worried about
Fortunately, the history and physical examination and some- damage to the feet, knees, hips, or spine? Are they worried
times imaging are usually all that are needed to determine the that their bowlegged toddler may grow up to resemble a
diagnosis, prognosis, and recommendations for the family. bowlegged elderly relative with painful unicompartmental
knee arthritis?
Is the child coming to the physician because of pain?
History If so, an etiology besides physiologic angulation/rotational
variations should be sought.
First, the clinician must determine why the child is brought When evaluating a lower extremity deformity or physio-
in for evaluation. Is it because the referring physician wants logic variant, the clinician should include questions regarding
to determine the diagnosis and need for treatment? If so, the the relevant factors as described in Table 2.1.
family may not require much counseling and reassurance. The clinician should determine at what age the deformity
was first noticed. Intoeing which does not appear until
approximately age 2 years is usually due to resolution of the
J.J. Gugenheim, MD (*)
physiologic external rotation contracture of the infant hip,
Texas Orthopedic Hospital, 7401 Main Street, Houston,
TX 77030, USA unmasking the persistent tibial torsion and/or proximal
e-mail: [email protected] femoral antetorsion.

16 J.J. Gugenheim
Does it seem to be getting better, worse, or remaining the Charcot–Marie–Tooth disease, or kidney failure with renal
same? For example, bowing of the lower extremities is a nor- rickets should alert the clinician to conditions that may be
mal consequence of the in utero position and will resolve inherited.
spontaneously (Fig. 2.1); the gradual improvement in the
bowing helps establish the diagnosis and rules out Blount
disease or metabolic bone disease, two conditions that Physical Examination
require a more extensive work-up and treatment.
Is there any history of fractures, lower extremity injuries, Although many children who present for evaluation have an
bone or soft-tissue infections, or previous surgery? The obvious diagnosis, one must always perform an examination
development of an angular deformity or leg length discrep- that includes at least the vital signs (height, weight, body
ancy following a fracture or infection may indicate a partial mass index (BMI)), upper extremities, spine and trunk, neu-
or complete injury to the growth plate. rological, gait, and lower extremities. Although the gait and
Is there any family history of orthopedic, neuromuscular, lower extremity exam will be the most detailed, the other
renal, or endocrine disorders? Is there any family history of components are necessary and can be performed quite
short stature? A family history of hypophosphatemic rickets, rapidly.
Vital Signs
Table 2.1 Relevant aspects of history (besides the onset, duration,
progression, and prior treatment of the lower limb deformity)
Height and weight should be measured at the office visit
Age
instead of asking the family to furnish this information
Gender
because parents often are quite inaccurate with their child’s
Number of siblings
stated height and weight [5]. The BMI is a proxy for adiposity
Birth order
and can be calculated from the formula
Length of pregnancy
Obstetric presentation Weight ( kilograms ) Weight ( pounds )
Presence or absence of difficulty with delivery BMI = = ´ 4536
Height ² ( meters ) Height ² ( cm )
Birth weight
As active or less active than siblings in utero
Developmental motor milestones The percentile for age-related Z score should be determined
Family history of orthopedic, neuromuscular, renal, or endocrine from charts available from the Centers for Disease Control
disorders ( http://www.cdc.gov/growthcharts/clinical_charts.htm ).
Fig. 2.1 The development of the

tibiofemoral angle in children
during growth; the standard 25⬚
deviation is ±8°. Adapted with
permission from Zaleske
20⬚
DJ. Metabolic and endocrine
disorders. In: Morrissy RT,
Weinstein SL, editors. Lovell and 15⬚
Varus
Winter’s Pediatric orthopedics,

4th edition. Philadelphia: 10⬚
Lippincott-Raven; 1996 [58]
5⬚
0⬚ Years
1 2 3 4 5 6 7 8 9 10 11 12 13
-5⬚
Valgus
-10⬚
-15⬚
-20⬚
2 Clinical Evaluation Including Imaging 17
Very large age-related Z scores may indicate a condition of a neurological abnormality, a more detailed exam must be
associated with obesity such as Blount disease or Prader– done, including searching for pathologic primitive reflexes.
Willi syndrome. Very low Z scores may indicate malnutri-
tion, malabsorption, chronic metabolic disease, or skeletal
dysplasia. Gait
The examiner should watch the child walk. Sometimes children

Upper Extremity walk in a certain pattern as instructed by their parents when
aware that the physician is watching. Thus, it is helpful to
An abnormal carrying angle of the elbow, especially if sym- watch the child walk from the waiting area to the exam room
metric, may indicate a skeletal dysplasia. Joint laxity with when he or she is unaware of being examined. One should
excessive motion, such as hyperextension of the elbows or watch the child walk both with shoes and barefoot because
interphalangeal joints, may indicate a connective tissue dis- shoes may hide dynamic conditions such as searching toes or
order. Palpable metaphyseal enlargements may indicate a dynamic forefoot adduction, two conditions attributed to
rachitic disorder or multiple hereditary exostoses. contraction of the abductor hallucis creating a concave
medial border of the foot during gait.
Spine and Trunk Foot Progression Angle

The foot progression angle is the angle between the axis of
The rib cage should be palpated for a rachitic rosary: the foot and the line of progression, with intoeing expressed
swellings at the costochondral junction are indicative of a as negative values and out-toeing expressed as positive values
rachitic disorder. Screening for scoliosis is performed by [2, 6]. A study of gait in 160 children (up to 14 years of age)
checking for thoracic and/or lumbar asymmetry with for- concluded that the normal angle of gait was approximately
ward bending. 10° external at all ages with a slight decrease after age 7 [7]
(Fig. 2.2). The mean foot progression angle is a positive
value (external rotation) throughout childhood and adult-
Neurologic hood. However, negative values (internal rotation) are con-
sidered normal (within two standard deviations) throughout
A quick but quite sensitive screening battery for the detection childhood and early teens [2]. Etiologies contributing to an
of neurological disease affecting the lower extremity can be internal rotation gait include excessive acetabular antever-
done in children and adolescents of walking age by having sion, excessive femoral anteversion, internal tibial torsion,
them walk on their heels in dorsiflexion, on their toes (meta- metatarsus adductus (MTA), and searching toe. Etiologies
tarsal heads) in equinus, and hop on one leg at a time. contributing to an external rotation gait include acetabular
To perform these maneuvers, the child utilizes quadriceps retroversion, physiologic external rotation contracture of
(L2, L3, L4), hip extensors (L5, S1, S2), hip abductors (L5, the hips, femoral retroversion, external tibial torsion, and
S1), ankle plantar flexors (L5, S1, S2), and ankle dorsiflexors positional calcaneal valgus. These conditions may be additive
(L4, L5). If there is a high level of suspicion for the presence or may compensate for each other [8].
Fig. 2.2 The foot progression

angle changes with age. The
normal range is very broad.
Out-toeing
Adapted from [4] 20⬚
10⬚
0⬚
In-toeing
-10⬚
1 3 5 7 9 11 13 15 - 19 30’s 50’s 70+
Age (years)
18 J.J. Gugenheim
Short Leg Gait Table 2.2 Etiologies of lower extremity deformity

A child with a limb length inequality may walk with the foot Varus
and ankle in equinus or with an abductor lurch on the short Traumatic
side during stance phase of gait. Other compensatory mecha- Proximal tibial physeal injury (may also be valgus)
nisms for a short lower extremity include flexing the contra- Congenital
lateral knee and/or dropping the ipsilateral hemipelvis. Tibial hemimelia
Developmental
Equinus Achondroplasia
Unilateral equinus is often seen in patients with hemiparetic Campomelic dysplasia
cerebral palsy, some unilateral foot deformities, and limb Congenital pseudarthrosis of tibia
Hypochondroplasia
length inequality. An equinus position in stance phase of gait
Metaphyseal chondrodysplasia (Schmid, Jansen, Spahr types)
is the most common compensating mechanism for a limb
Neurofibromatosis
length inequality. Bilateral equinus may indicate idiopathic
Ollier disease (multiple enchondromatosis) (may also be valgus)
toe walking or neuromuscular disease, such as cerebral palsy
Osteogenesis imperfecta (may also be valgus)
or muscular dystrophy. Multiple epiphyseal dysplasia (may also be valgus)
Multiple hereditary exostosis (may also be valgus)
Trendelenburg Sign Spondyloepiphyseal dysplasia
The child should stand on one foot while the clinician Thanatophoric dysplasia
observes for the pelvis dropping toward the opposite side. Tibia vara (Blount disease)
Normally, the child should not allow the pelvis to drop for at Metabolic
least 15 s. A positive sign with the pelvis dropping indicates Vitamin D-deficient rickets
abnormal ipsilateral femoral pelvis anatomy (such as acetab- Hypophosphatemic rickets
ular dysplasia, dislocated or subluxated femoral head, proximal Valgus
femoral deformity) or abductor weakness. Traumatic
One must compare the gait appearance to the findings on Proximal tibial physeal injury (may also be varus)
the exam table. For example, forefoot adduction during gait Proximal tibial metaphyseal fracture sequela (Cozen
phenomenon)
but not in the nonweight-bearing position is frequently due
Congenital
to the normal phasic activity of the toe flexors and tibialis
Congenital deficiency of femur
posterior muscles in stance phase; these muscles insert Posteromedial bowing of tibia
medial to the midline of the foot and may act as forefoot Fibular hemimelia
adductors during gait. The great toe may abduct during Developmental
stance phase of gait. This condition, called wandering or Ellis-van Creveld dysplasia
atavistic toe, is attributed to the phasic activity of the abductor Metatropic dysplasia
hallucis muscle [4, 8]. Morquio syndrome
Multiple hereditary exostosis
Multiple epiphyseal dysplasia (may also be varus)
Lower Extremities Metabolic
Chronic renal failure
Supine Exam
A general assessment of the lower extremities in the supine
position on the exam table includes evaluation of the limb aspect of the extremity with the patellas forward. Measuring
lengths, as seen by the levels of the plantar aspects of both the distance between the knees or medial malleoli to quantify
feet and the measurement of the distance from the anterior varus or valgus is less reliable; there may be variability due
superior iliac spine to the medial malleoli. Any asymmetry to adiposity, size of the patient’s legs or thighs, and limb
of thigh circumference, leg circumference, or foot length torsion. Bowing of the lower extremities is normal until age
should also be noted. If a discrepancy in limb lengths is 2 years. Persistent varus after age 2 years or excessive valgus
noted, the patient should be examined with blocks under- may be a manifestation of a traumatic, congenital, develop-
neath the short leg to determine when the pelvis is level. This mental, or metabolic disease [11–16] (Table 2.2).
method has been shown to be the most reliable clinical mea- Although radiographs can distinguish between normal
surement for leg length discrepancy, although the studies physiologic bowing of infancy and rare conditions requiring
were done in adults [9, 10]. intervention, routine radiographic screening and referral are
Varus (bow legs) and valgus (knock knees) angulations at not cost effective and expose children to unnecessary radia-
the knee are measured with a goniometer on the anterior tion [11, 17]. The cover-up test is a useful screening to assess
and adolescents should be asked to report what amount of

elevation for the short extremity makes them feel level and
well corrected. Patients with long-standing limb length
discrepancies usually report that they feel well corrected
when the blocks under the foot undercompensate for the dis-
crepancy because they have become partially accustomed to
the discrepancy. Standing on blocks is more accurate (greater
reliability) and also incorporates the foot and pelvis, unlike
the supine tape measure technique [18].
The lower extremity may appear short if an ipsilateral hip
or knee flexion contracture or an adduction contracture of the
hip is present while standing; likewise the extremity may
appear long if an ipsilateral hip abduction contracture is
present. Any difference in the size of the foot should also be
noted. Foot size discrepancies are seen in hemihypertrophy,
neurological disease, and vascular disease.
Fig. 2.3 Cover-up test of the proximal left tibia. (a) A varus or neutral To determine if a discrepancy is due to a femoral or tibial
alignment is a positive test. (b) A valgus alignment is a negative test.
difference, the child should sit on the exam table with the
Courtesy of Jon R. Davids, M.D.
hips and knees flexed 90°, the patellas forward, and feet
unsupported. If there is a femoral discrepancy, the knees will
appear to be at a different level when viewed from above; if a
tibial discrepancy is present, the feet will be at different levels
when viewed from the front. In many cases, there is a discrep-
ancy in both segments.
Prone Exam
The prone exam (with measurement of hip rotation, thigh–foot
angle, and foot alignment) is an excellent qualitative assess-
ment of rotational conditions affecting the lower extremity.
Femoral Version and Antetorsion

Hip rotation in the prone position is the net result of soft-
tissue rotation contracture, acetabular version, and femoral
version (anteversion or retroversion). Axial rotation of the
acetabulum and femur within two standard deviations of the
Fig. 2.4 The lower extremity length is measured from the anterior
age-related mean value is considered normal and is termed
superior iliac spine to the medial malleolus with a metal tape measure
version; values outside two standard deviations are termed
torsion [4].
the alignment of the upper part of the lower leg in children Hip rotation should be evaluated with the child prone and
presenting with bow legs between the ages of 1 and 3 years the hip extended because the hip functions largely in extension
[17]. The lower extremity is positioned with the patella for- during normal gait. Also, hip flexion in the supine position
ward and the lower leg and foot are covered by the examiner’s relaxes the anterior hip capsule, causing an inaccurate assess-
hand. A positive test (bowing in the upper tibia) or neutral test ment of rotation [16]. The child should be positioned prone,
(a straight thigh–upper leg axis) is an indication for radio- with one hand lightly on the pelvic area to detect what degree
graphic evaluation. A negative test (slight valgus at the upper of rotation rocks the pelvis off the table. The knee is flexed
tibia) indicates physiologic bowing. A positive cover-up test 90°. The leg serves as an indicator of rotation, measuring its
has a high sensitivity; a negative cover-up test has a high angulation from vertical [6] (Fig. 2.5). Normal age-dependent
specificity and negative predictive value [17] (Fig. 2.3). values for internal rotation and external rotation have been
Limb length is measured from the anterior superior iliac published [2, 4, 6] (Fig. 2.6).
spine to the medial malleolus with a tape measure (Fig. 2.4). The normal hip arc of motion, the sum of internal and
If a limb length discrepancy is found, a cooperative child external rotations, is approximately 80°–100° [6, 19], with
should stand with blocks underneath the short extremity to greater external rotation than internal rotation until the age of
determine what size lift will level the pelvis. Older children 3–5 years [4, 7, 20]. Due to the in utero position, infants
20 J.J. Gugenheim
Fig. 2.5 Assessment of hip

rotation with 45° internal and 45°
external rotation. With kind
permission from Springer
Science+Business Media: Paley
D. Principles of deformity
correction. Berlin, Heidelberg;
2002 [23]
epiphysis (SCFE), coxa vara, and proximal focal femoral

Lateral rotation
80° deficiency (PFFD). Jacquemier et al. defined normal age-
Medial rotation
Anteversion related values of acetabular version in a study of 143 chil-
60°
dren between ages 1 and 15 years [22]. Because the
significance of abnormal acetabular version in infants and
children is unknown at present, radiographic studies are
40° rarely done to measure acetabular version; therefore, the true
prevalence of acetabular retroversion in children is largely
unknown. Coxa vara and PFFD usually exhibit a decreased
20°
arc of rotation (less than 90°) on the prone exam and are
detectable on the supine exam as decreased hip abduction or,
10° in unilateral cases, a limb length discrepancy.
A severe valgus deformity of the proximal tibia will cause
an apparent increase in external rotation and decrease in
internal rotation without affecting the total rotational arc,
1 3 5 7 9 11 13
whereas a severe varus deformity will cause an apparent
Fig. 2.6 Values for hip rotation and anteversion, according to age increase in internal rotation and decrease in external rotation
(horizontal axis). Adapted from [4] (Fig. 2.7), but this is rarely significant in an evaluation of
pediatric patients [23].
often have an external rotation contracture of the soft tissues In conclusion, in the vast majority of cases of infants with
around the hip at birth that gradually resolves by age 18 more external rotation than internal rotation in the prone
months. This soft-tissue contracture can mask the underlying position with the gradual diminution of the discrepancy, the
femoral antetorsion. This physiologic soft-tissue contracture etiology is a physiologic external rotation contracture of
is at its maximum value at birth and is the usual etiology for infancy (PERCI) especially between birth and age 18 months.
external rotation exceeding internal rotation until age 18–24 Increased internal rotation compared to external rotation is
months [20]. Femoral antetorsion (abnormally high value for often indicative of femoral antetorsion.
anteversion) manifests as an increase in hip internal rotation In most cases, a qualitative clinical examination, based on
and a decrease in external rotation without affecting the total comparing hip internal and external rotations, is sufficient
rotational arc. It is the most common cause of intoeing in without a quantitative imaging study. Imaging to quantify
early childhood, tends to occur more in girls, and is usually femoral version is indicated only if hip rotation is signifi-
symmetric [8]. As the physiologic soft-tissue external rota- cantly asymmetrical, operative correction is planned, or ver-
tion contracture resolves, internal rotation of the hip increases sion must be measured to determine the cause of failure of
[8]. For this reason, parents often report that their child prior surgical procedure around the hip [4]. Most authors
became more pigeon toed or walked with more intoeing at conclude that femoral anteversion is not harmful [8, 16, 24],
age 18–24 months. although it may require surgical correction when associated
Other etiologies of excessive external rotation include with excessive external tibial torsion [4, 19], abnormal
acetabular retroversion, as seen in Down syndrome [21], and acetabular version [19, 25], neuromuscular disease [26], or
femoral retroversion, as seen in slipped capital femoral developmental dysplasia of the hip (DDH) [25, 26].
Fig. 2.7 A 30° valgus deformity

of the proximal tibia results in an
incorrect assessment of 75°
external rotation (instead of 45°)
and 15° internal rotation (instead
of 45°). Adapted with permission
from Lippincott Williams and
Wilkins/Wolters Kluwer Health;
Jacquemier M, Jouve JL, Bollini
G, Panuel M, Migliani
R. Acetabular anteversion in
children. J Pediatr Orthop.
1992;12(3): 373–5 [22]
The TFA averages 5° internal (range 30° to +20°) in

infants and averages 10° external (range 5° to +30°) by the
age of 8 years [8]. Katz reported internal tibial torsion on the
left side more frequently than the right in a series of 54
females and 45 males [27]. Normal age-related values have
been published [2, 4]. Staheli et al. assessed the normal range
and interobserver error. They found that the normal range,
defined as within two standard deviations, is quite large, with
a standard deviation of 4.9°–8.9°. They concluded that val-
ues from 5° to 30° were within the range of normal during
childhood [2].
Tibial torsion can also be diagnosed and quantified by
determining the transmalleolar axis (TMA). With the child in
the sitting position with the hip flexed and patella facing for-
ward, the angle between the TMA and the coronal plane is
assessed [28, 29]. Measurement can also be done in the prone
position by locating the transmalleolar axis, drawing a line
perpendicular to this axis, and then measuring the angle this
Fig. 2.8 The thigh–foot angle (TFA) assesses tibial torsion. (a) line forms with the long axis of the thigh. The result obtained
External tibial torsion. (b) Internal tibial torsion. Adapted with kind by the TMA method yields a value a few degrees greater than
permission from Springer Science+Business Media: Paley D. Principles
of deformity correction. Berlin, Heidelberg; 2002 [23]
the TFA method, but the TFA is easier to measure [2]. While
the TMA method measures only the tibial and fibular rota-
tions, the TFA method is also affected by hindfoot abnormali-
Tibial Torsion ties. More accurate quantification by diagnostic imaging is
Tibial version is normal in children and, by definition, torsion rarely indicated unless corrective osteotomies are being
is abnormal [2, 4]. However, the term tibial torsion will be planned. Since internal tibial torsion is a normal condition in
used here to include both normal and abnormal values because infants and children and normally resolves without treatment,
of its common usage. The thigh–foot angle (TFA) is the angle corrective osteotomies are rarely indicated.
formed by the longitudinal axis of the thigh with the longitu-
dinal axis of the hindfoot. The TFA is measured in the prone Axial Foot Deformities
position with the hip in neutral rotation, the knee in 90° flex- The alignment of the foot as a possible cause of intoeing or
ion, and the ankle and foot in neutral dorsiflexion (Fig. 2.8). out-toeing can also be assessed with the child in the prone
Although this assessment of the TFA is a qualitative diagnos- position. The examiner should note whether the medial and
tic test for tibial torsion, a goniometer may be used for quanti- lateral borders of the foot are straight (Fig. 2.9). The heel
fying this angle and to compare on subsequent exams. External bisector line, a line through the midline axis of the hindfoot
tibial torsion is diagnosed and quantified with a positive value and forefoot, passes through the second web space in a normal
if the foot is externally rotated on the leg; internal tibial torsion foot. Originally described by taking a photocopy of the foot, a
is diagnosed and quantified with a negative value if the foot is method of making a permanent paper document, the child is
internally rotated on the leg. held in a weight-bearing position on the photocopy machine.
22 J.J. Gugenheim
out disorders such as hip dysplasia and SCFE in a child

presenting with a gait abnormality and pertinent history and
clinical exam [8].
Assessment of Limb Length
Several techniques have been developed for the measurement

of limb length discrepancies, including orthoroentgenograms
[31], slit scanography [32], standing teleroentgenograms
Fig. 2.9 The heel bisector line passes through the second web space in [23, 33], scanograms [34], computerized tomography (CT)
the normal foot (10A) and lateral to the second web space in metatarsus scanogram [35], and ultrasound [36]. The ideal method
adductus. Adapted with permission from Smith JT, Bleck EE, Gamble
JG, Rinsky LA, Pena T. Simple method of documenting metatarsus should be accurate, readily available in the physician’s office,
adductus. J Pediatr Orthop. 1991;11(5):679–80 [30] and technically easy. Its cost and radiation dosage should be
low. Its measurements should exhibit good reproducibility
(Table 2.3).
The method can also be used while examining the child prone A single-exposure standing teleroentgenogram taken with
[30]. If the heel bisector line passes lateral to the second web the patella straight forward with a distance of 10 ft between
space, the forefoot is adducted (MTA). If the line passes the beam source and the film offers the best combination of
medial to the second web space, valgus is present. A concave benefits and fewest shortcomings (Fig. 2.10). It can easily be
medial border of the midfoot also indicates MTA. If MTA is performed. Radiation exposure is greater than the CT scano-
present, the hindfoot can then be held with one hand while gram technique [35] but less than the three-view scanogram
the examiner’s other hand abducts the forefoot to determine [33]. With the advent of digital radiography and picture
whether the MTA is rigid or supple. A normal foot should archiving and communication systems (PACS), the image
be flexible and exhibit slight forefoot abduction with gentle can be manipulated to minimize radiation exposure and film
manipulation. storage is not a problem. By using a large distance between
the beam source and the film, parallax and magnification
(of up to 5 %) are introduced. Measurements can be made
Diagnostic Imaging directly with a ruler or with computer software [33]. The
image is made in the weight-bearing position. Angular defor-
Whether to do imaging studies after a history and physical mities and bone pathology that may contribute to angular
exam often presents a dilemma. The expense and radiation deformity and length discrepancy such as osteochondromas,
exposure with imaging should be avoided if the diagnosis is enchondromas, or malunited fractures are detectable (see
obvious from the history and physical exam and the imaging Table 2.2). The contribution of each segment (pelvis, femur,
will not affect the decision making. Any limb length dis- tibia, and foot) can also be quantified. Intraobserver and
crepancy, asymmetric angular deformity, or angulation out- interobserver measurement reliability is excellent with a
side the range of normal for age requires a full-length teleroentgenogram [34].
standing anterior–posterior image of both extremities to A standardized radiographic technique avoids distortion
quantify the angular deformities and/or limb length discrep- and magnification due to divergence of the X-ray beam and
ancy. Davids et al. noted that routine radiographic screening is reproducible so that serial radiographs can be compared.
of children with bowed legs is not cost effective and exposes A cassette with a variable grid to include the hip, knee, and
them to unnecessary radiation and recommended using the ankle is used. The cassette measures 132.5 × 38.5 cm for
cover-up test to screen children between ages 1 and 3 years. larger patients and 94.5 × 38.5 cm for smaller children. The
Children with a negative cover-up test do not require radiog- beam is centered at the knee with the beam source at least
raphy [17]. Guidelines for performing imaging studies for 10 ft from the cassette. Correct patient positioning requires
rotational conditions are less clear. Children with rotational patellas straight forward for the anteroposterior (AP) view
components more than two standard deviations outside the and 90° orthogonal to the AP position for the lateral view.
mean, short stature (less than twenty-fifth percentile), asym- The knees should be in full extension, with the body weight
metric hip abduction or rotation, a positive Trendelenburg distributed equally on both feet. If there is a leg length
sign, marked limb asymmetry in length or alignment, or dis- discrepancy, a block is placed under the shorter extremity to
comfort during gentle range of motion of the lower extrem- level the pelvis and to keep the knee extended with the
ity should be considered for diagnostic imaging. A pelvic weight equally distributed on both feet. In severely obese
radiograph including a lateral view may be needed to rule children or in the presence of severe angular deformity,
2
Table 2.3 Comparison of methods for assessing leg length discrepancya

Incorporation
Approximate Approximate of height of Typical
radiation charges (US Radiographic the foot and availability
Methods Reliabilityb Accuracyb Magnification exposure (mrads) dollars) deformity analysis pelvis in the USA Weight bearing
Clinical
Clinical Evaluation Including Imaging
Supine tape measure—“Real” + + None None Office visit Not applicable No Yes No
(ASAS to malleolus)
Supine tape measure— + + None None Office visit Not applicable Partial Yes No
“Apparent” (umbilicus to
malleolus)
Standing blocks ++ + None None Office visit Not applicable Yes Yes Yes
Imaging
Teleoroentgenogram ++++ +++ ~5% 42 $95c Yes Yes Varies Yes
Orthoroentgenogram +++ +++ Minimal 200 $110c No No Varies No
Scanogram ++++ +++ Minimal 200 $110c No No Varies No
Computed radiography ++++ +++ Varies with Varies with $137d Varies with Varies with Varies Varies with technique
technique technique, less technique technique (scanogram vs.
(scanogram vs. exposure than (scanogram vs. teleoroentgenogram)
teleoroentgenogram) standard teleoroentgenogram)
radiography
Microdose digital radiography +++ ++++ None 2 $75c Yes Yes No Yes
Ultrasound +++ ++ None None Not reported No No No Yes
CT scan (digital localization ++++ ++++ Minimal 60 $60c Minimal None Varies No
image)
MRI ++++ +++ Minimal None Not reported Not reported Not reported No No
a
Adapted with kind permission from Springer Science + Business Media: Sabharwal S, Kumar A. Methods for assessing leg length discrepancy. Clin Orthop Relat Res. 2008;466 (12)2910–22
b
Please see text for detailed analysis. For simplicity, the increasing number of “+” signs indicates greater degree of reliability/accuracy
c
Machen MS, Stevens PM. Should full-length standing anteroposterior radiographs replace the scanogram for measurement of limb length discrepancy? J Pediatr Orthop B. 2005;14:30–37
d
Sabharwal S, Zhao C, McKeon JJ, McClemens E, Edgar M, Behrens F. Computed radiographic measurement of limb-length discrepancy. Full-length standing anteroposterior radiograph
compared with scanogram. J Bone Joint Surg Am. 2006;88:2243–2251 [33]
23
24 J.J. Gugenheim
51 in
(130 cm)
Level
of knee
10 ft
(305 cm)
Fig. 2.10 A single-exposure standing full-length radiograph with patella forward is used to evaluate limb length and deformity. Adapted with kind
permission from Springer Science+Business Media: Paley D. Principles of deformity correction. Berlin, Heidelberg, 2002 [23]
it may be necessary to perform separate AP radiographs of Slit scanography utilizes an X-ray beam that is tightly
the two lower extremities. A magnification standard, such as collimated to a thin transverse slit that exposes the film as the
a 30 mm ball bearing, can be affixed to the extremity at the X-ray tube is moved from one end of the limb to the other [33].
main site of interest or at the knee when assessing leg length. Since the beam is always perpendicular to the film, theoreti-
The ball bearing should be positioned at the level of the cally magnification and parallax should be eliminated. Direct
bone, the same distance from the cassette as the bone. This measurement using a tape measure is quite accurate and reli-
technique quantifies magnification, which is usually about able [32]. Disadvantages include the facts that the patient is
5 % [33]. The ball bearing or another magnification marker nonweight bearing and must remain still for the exposure and
must be affixed to the extremity at the level of the bone, not the equipment is not readily available in most clinicians’
directly on the cassette (Fig. 2.11a, b). Placing a ruler with offices. Like the standard teleroentgenogram, it requires a
radiopaque graduations on the cassette may not accurately special cassette (38.5 × 94.5 cm or 38.5 × 132.5 cm).
quantify magnification because the ruler is closer to the film A three-view scanogram consists of three images with a
than the bone. ruler with radiopaque graduations placed vertically in the
Slit scanography and CT scanograms also exhibit reason- midline. Images of the hip, knee, and ankle are made with
able accuracy and reliability [18], but they require special the beam perpendicular to the joint at each exposure to elimi-
technology which may not be available in most clinicians’ nate parallax and magnification. The technique is easily per-
office setting. CT scanograms have the lowest radiation formed in most clinicians’ offices, requiring only a ruler with
exposure compared to other radiographic methods and can radiopaque markings and standard radiography equipment.
be performed in the presence of flexion contractures. The CT Its accuracy is comparable to the modified standing tele-
scanogram can be performed very rapidly and magnification roentgenogram [33, 34]. Disadvantages include the slightly
error is not an issue. However, since the scan is usually higher radiation dose [33], the necessity for the patient to
performed in a nonweight-bearing position, it is impossible to remain still for all three images, the necessity to avoid any
measure the contribution of the foot to the leg length discrep- tilt of the ruler, and the necessity to avoid asymmetric posi-
ancy. Furthermore, it is difficult to manipulate the CT image tioning of the extremities in abduction or adduction.
for planning of deformity correction, although advances in digi- Deformity analysis cannot be performed, and midshaft
tal imaging software may eliminate this problem in the future. pathology may be missed with a scanogram. Moreover, the
Cost, availability of CT scanners, and need for scheduling contribution of the foot to the limb length discrepancy cannot
continue to be obstacles to its use. be measured. Because of these shortcomings, scanograms
to avoid any movement while the three exposures are made.

Higher radiation dose is also an issue.
In conclusion, the modified standing teleroentgenogram
affords the most advantages for initial screening, since it will
reveal the site of the discrepancy and any angular deformity
and diaphyseal pathology. For follow-up, the standing tele-
roentgenogram, slit scanography, CT scanograms, or three-
view scanograms may be performed, depending on the
equipment and the absence of angular deformities or foot
height discrepancy. If angular deformity and/or foot discrep-
ancy is present, or deformity analysis is planned, the modified
standing teleroentgenogram should be repeated.
Version Diagnostic Imaging
The presence of version (normal axial angulation) or torsion

(abnormal version greater than two standard deviations from
the mean value) is usually apparent from the physical exam
without the need for diagnostic imaging. If the amount of
version on physical exam is normal for the child’s age, and
the diagnosis is obvious and the imaging will not affect deci-
sion making, then no imaging studies are indicated. However,
if the diagnosis is in doubt, if the suspected condition is out-
side the normal range for age, or if corrective surgery is
being planned, imaging for diagnosis and quantification may
be indicated. Since computerized tomography (CT) is usu-
ally necessary, one must weigh the advantages of informa-
tion gained from the study against the disadvantages of
Fig. 2.11 (a) AP and lateral alignment radiographs of a 16-year-old radiation exposure and cost.
girl with Ollier disease and a limb length discrepancy. She felt level
with a 4-cm block under the right foot, but this block undercompen- Acetabular Version
sated for her discrepancy. (b) The lateral view is taken with the knee in
maximum extension. Although the knee does not appear to extend com- Computerized tomography measurements show that the nor-
pletely, the lack of full extension is due to a juxtaarticular proximal mal acetabulum is anteverted approximately 13° in children
tibial deformity, as quantified by proximal posterior tibial angle (PPTA). from ages 1 to 15 years, with a large range of normal between
Note the 30-mm ball bearings at the levels of the knee to quantify ages 1 and 2 years [21, 22]. Visser et al. reported mean acetab-
magnification
ular anteversion of 6.5° in a newborn and 16.5° in adults [37].
Excessive acetabular anteversion is often seen in children
are not recommended for the initial imaging technique, but with DDH, and acetabular retroversion has been reported in
offer convenience and accuracy for follow-up of femoral children with Down syndrome [21]. McKibbin developed an
and/or tibial length discrepancies [33]. “instability index” based on the sum of the acetabular version
Orthoroentgenography is a radiographic technique of angle and the femoral anteversion angle. Values less than 20°
performing three exposures of the lower extremity on a long or greater than 58° were associated with hip instability. He
cassette. Similar to the scanogram, the beam for each expo- hypothesized that the version of the femur and acetabulum
sure is centered over the hip, knee, and ankle to minimize must develop in a coordinated manner [25]. Tönnis and
magnification and parallax. By using rectangular collimation, Heinecke reported increasing pain and osteoarthritis in adult
the three exposures can be performed to obtain a full-length hips with a McKibbin instability index at the upper and lower
image from the hip to the ankle [31]. Direct measurement and limits of normal [19]. Currently there are no longitudinal
deformity analysis on the image can be performed. However, studies of the development of acetabular version in normal
the technique has many of the same disadvantages as the and abnormal hips in children. Techniques for qualitative
scanogram, including the inability to perform the study in a assessment using a single AP radiographic image of the pel-
weight-bearing position, inability to measure the contribution vis have been described in adults but not in children [38, 39].
of the foot to the length discrepancy, and need for the patient Until the relevance of quantifying acetabular version in
26 J.J. Gugenheim
landmarks at the greater trochanter and long axis of the neck

eliminates any contribution from the shaft. Even at the distal
femur, the reference line or plane has been defined differently
in various studies: as the flexionextension axis of the knee
[43], as the plane bisecting the femoral condyles [42], and as
a line tangent to the posterior aspect of the femoral condyles
[19]. Because studies show that femoral antetorsion (exces-
sive version) normally resolves and does not predispose to
osteoarthritis, surgery is rarely indicated [8, 16, 24].
Fig. 2.12 Femoral version is the angle formed by the femoral neck and Antetorsion may not resolve in children with spastic cerebral
the plane of the femoral condyles. Adapted with kind permission from palsy; in some such patients, corrective derotation osteoto-
Springer Science+Business Media: Paley D. Principles of deformity mies may be indicated [26].
correction. Berlin, Heidelberg; 2002 [23]
When planning corrective femoral derotation osteoto-
mies in children with cerebral palsy, accurate localization of
children is documented, computerized tomography and its the site of torsion proximal and distal to the greater trochan-
high radiation exposure to the pelvic organs are probably not ter is important. Kim et al. defined two components of fem-
indicated with certain exceptions, such as preoperative plan- oral anteversion based on CT imaging: supratrochanteric
ning for reconstruction of a hip dislocation associated with torsion (STT) in the proximal femur and infratrochanteric
syndromes or following a failed initial hip surgery. torsion (ITT) in the distal femur. In normal children, the
STT declines with age; the ITT showed a random distribu-
Femoral Version tion that did not decline with age for a net decrease in femo-
Femoral version is the angle formed by the axis of the femoral antetorsion. In children with cerebral palsy, the ITT
ral neck with the tangent of the posterior aspect of the femo- increased and the STT decreased, and the overall femoral
ral condyles or transcondylar axis at the knee. The normal antetorsion remained essentially unchanged. If derotation
femur is anteverted, with the neck extending anteriorly from osteotomy is performed, it should be performed at the site of
the coronal plane (Fig. 2.12). Normal values for infants and the torsion to avoid creating a new iatrogenic deformity and
children have been published, with an average value of 30° lever arm disease: muscle fatigue with or without a joint
to 40° at birth, declining to 8° in adult men and 14° in adult contracture [46]. Therefore, CT imaging may be indicated
women [2, 4, 6, 19, 26]. to localize the site of the torsion when planning derotation
Computerized tomography is currently the most accurate osteotomies.
method of quantifying femoral version [40]. The equipment
is readily available but exposes children to ionizing radia- Tibial Torsion
tion. The morphologies of the femoral neck and greater tro- Similar to femoral version, tibial torsion rarely requires
chanter sometimes make measurement difficult [41]. Various imaging for diagnosis or quantification. Computerized
other radiographic techniques have been used, including tomography has been described to measure tibial torsion as
biplanar radiography (RyderCrane [41] and Magilligan [42] the angle formed by a line through the proximal tibial juxta-
techniques), fluoroscopy [43], CT scan, sinus wave biplanar articular area and the distal bimalleolar axis or distal articu-
conventional radiography, modified Heimann biplanar con- lar surface [47]. Similarly ultrasonography measures the
ventional radiography, and limited 3D volumetric tomogra- tibial torsion by the angle between the plane of the posterior
phy [40]. In order to minimize radiation exposure, ultrasound aspect of the proximal tibia and the plane of the posterior
techniques have also been developed [44, 45]. One series aspect of the distal tibia [28]. These imaging techniques
showed that ultrasound measurements yielded lower values assess tibial anatomy, but because they use different bony
than the Magilligan technique, with larger discrepancies at landmarks, the values obtained differ slightly. Unlike clinical
greater degrees of torsion [44]. In contrast, another study in assessment of the thigh–foot angle or bimalleolar axis, CT or
patients between 12 and 44 years of age found an anteversion ultrasound negates any effect of foot pathology or mobility
value of 10° higher than the correct value and recommended of the fibula. Ultrasound does not expose the child to radia-
ultrasound as a screening tool with radiographic methods if tion. Although experimentally produced tibial torsion
surgery is contemplated [45]. increased pressure at the tibiotalar joint in cadavers [48], cur-
Different anteversion values arise due to the method of rently there is no known detrimental effect of physiologic
defining the distal reference line or plane to be compared to tibial torsion in young children. Therefore, there is hardly
the proximal line or plane along the long axis of the femoral any indication for performing such imaging studies in
neck. Measuring the anteversion as a relationship between asymptomatic individuals.
Coronal Plane Angulation
Distinguishing physiologic bowing of the lower extremities Distal femoral

in infants from infantile tibia vara (Blount disease) is a fre- 1 metaphyseal angle
quently encountered reason for orthopedic evaluation. Early
3 cm
and accurate diagnosis is important in order to avoid unnec-
essary treatment and radiation exposure in infants with phys-
iologic bowing while preventing progression of the deformity 2 cm
in infants with tibia vara. The child with a negative cover-up
test; normal height, weight, and BMI for age; negative fam-
ily history for orthopedic disorders; and no other remarkable
physical findings rarely needs a radiograph, but should be
followed for resolution of the bowing [17].
2 cm
Several studies have attempted to use radiographic mea-
surements to distinguish normal physiologic bowing and
3 cm
tibia vara. The ideal radiographic imaging technique would 2
have high sensitivity (such as not incorrectly missing a case
Proximal tibial
of tibia vara and neglecting treatment) and high specificity
metaphyseal angle
(such as not incorrectly classifying a case of physiologic
bowing as tibia vara and treating a child unnecessarily in
infants before age 2 years). Langenskiöld and Riska classified
six stages of infantile tibia vara, based on the radiographic Fig. 2.13 The distal femoral metaphyseal-diaphyseal angle (FMDA)
α1 and the proximal tibial metaphyseal-diaphyseal angle (TMDA) α2
appearance of the proximal tibia [49]. The classification of O’Neill and MacEwan. Adapted with permission from O'Neill DA,
system has several shortcomings. The radiographic changes MacEwen GD. Early roentgenographic evaluation of bowlegged chil-
are not seen before age 2 years; Langenskiöld stage is not dren. J Pediatr Orthop. 1982;2(5):547–53 [51]
related to prognosis and does not consistently guide treat-
ment [50]. Interobserver agreement is good for the early and
late stages but poor for the intermediate stages [50]. Salenius developed radiographic signs of tibia vara [52]. Using
and Vankka described normal mean and standard deviations Levine’s and Drennan’s radiographic technique, Feldman
of the radiographic femoral-tibial angles for infants and chil- and Schoenecker developed guidelines with improved sensi-
dren [14]. Although varus angles after age 2 years are abnor- tivity and specificity by using 9° and less as the criterion for
mal, this method does not distinguish physiologic varus at physiologic bowing and 16° or greater as the criterion for
the upper limit of normal from tibia vara before age 2 years. tibia vara. For patients with an MDA between 9° and 16°, the
O’Neill and MacEwen measured the distal femoral metaph- authors considered bracing only if there is instability with
yseal-diaphyseal angle (FMDA) and the tibial metaphyseal- walking and recommended evaluating the patient at 4-month
diaphyseal angle (TMDA). The distal femoral metaphyseal intervals until the bowing resolves or the characteristic signs
angle (FMDA) is formed by a line perpendicular to the line of Blount disease are seen [53]. McCarthy et al. used the
connecting the medial and lateral edges of the physis and a femoral-tibial ratio (FTR), defined as the femoral MDA
line drawn through the midpoint of the femoral shaft at divided by the tibial MDA, to differentiate between infantile
points 2 and 3 cm proximal to the epiphysis. The proximal tibia vara and physiologic bowing. If the tibial MDA is
tibial metaphyseal angle is measured in a similar manner greater than the femoral MDA, the femoral-tibial ratio is less
(Fig. 2.13). Children with a TMDA greater than the FMDA than one, indicating probable infantile tibia vara instead of
or fibula longer than the tibia were more likely to have a physiologic bowing. If the FTR was less than one, indicating
persistent varus deformity which did not resolve by age 30 physiologic bowing, the authors reported a false-negative
months [51]. Levine and Drennan measured the metaphy- rate of 10 % (one in ten cases of infantile tibia vara would be
seal-diaphyseal angle (MDA) at the proximal tibia, formed missed) and a false-positive rate of 7 % (approximately 1 in
by the intersection of a transverse line through the proximal 14 cases of bowing would be incorrectly diagnosed as infan-
tibial metaphysis and a line perpendicular to the long axis of tile tibia vara). They found good inter- and intraobserver reli-
the tibial shaft in children between ages 11 months and 30 ability, with little effect of rotation position for the radiograph
months (Fig. 2.14). Only 3 of 58 patients with an MDA less [54]. Stricker and Faustgen emphasized the importance of
than 11° developed radiographic changes of tibia vara, neutral limb rotation when measuring the TMDA and the
whereas 29 out of 30 patients with an MDA of 11° or more tibial-femoral angle [55].
28 J.J. Gugenheim
Table 2.4 The six deformity components

The three angulation components are:
Apex medial (valgus) Angulation in the AP (XY)
Apex lateral (varus) plane
Apex anterior (procurvatum) Angulation in the sagittal
Apex posterior (recurvatum) (YZ) plane
External rotation Angulation in the XZ plane
Internal rotation
The three translation components are:
Translation along the X axis Medial translation, lateral
translation
Translation along the Z axis Anterior translation,
posterior translation
α Translation along the Y axis Lengthening, shortening
Skeletal deformity is a vector. Like all vectors, deformity

has magnitude, direction, and location.
Magnitude of a skeletal deformity has six components:
Three angulations and three translations (Table 2.4).
Although these components of the deformity may coexist,
they are independent of each other. The six components are
measured in an XYZ three-dimensional coordinate system.
Fig. 2.14 The tibial metaphyseal-diaphyseal angle (MDA) of Levine A deformity may have any or all of these components.
and Drennan. Adapted from [52] A deformity with components in more than one plane is an
oblique plane deformity, not a biplanar deformity. The mag-
Because none of these methods guarantees perfect sensi- nitude of the deformity in the oblique plane (the true plane of
tivity and specificity, a combination of methods should be the deformity) is greater than the magnitude of any of its
utilized, including measuring the FMDA and TMDA on an orthogonal components.
alignment radiograph in neutral rotation. An FTR less than In order to document the presence of a deformity on a
one or an abnormally large TMDA warrants serious consid- radiograph, there must be at least one of the following:
eration of the diagnosis of early infantile tibia vara. However, mechanical axis deviation (MAD), abnormal joint orientation
there is no single value of an abnormally high TMDA that angle(s), and limb length inequality. Conversely, to document
will differentiate between physiologic bowing and infantile accurate correction radiographically, it is both necessary and
tibia vara with perfect sensitivity and specificity. sufficient to correct the mechanical axis of the extremity, joint
orientation angles, and limb length inequality. Abnormal
axial angulation (malrotation) may be quantified by com-
Quantitative Deformity Analysis puted tomography if necessary, but it is usually assessed by
physical examination.
The physician should use radiographic imaging to make a
diagnosis, quantify the deformity, and plan correction of the Mechanical Axis and Anatomic Axis
deformity. A single-exposure weight-bearing teleroentgeno- The entire lower extremity, the tibia, and the femur have a
gram or similar digital image from the hip joint to the floor is mechanical axis in the coronal plane. The mechanical axis of
used to identify and quantify coronal (AP) and sagittal (lat- the lower extremity is a straight line from the center of the
eral) plane deformities. This technique minimizes distortion femoral head to the center of the ankle. The mechanical axis
and magnification due to divergence of the X-ray beam and is the weight-bearing line during quiet standing. In a normal
is reproducible so that serial radiographs can be compared. lower extremity, this line passes 010 mm medial to the center
Measuring traditional full-length radiographs was reported of the knee [23]. The femur and tibia each has a mechanical
to be more reliable than measuring paper images or digital axis. The femoral mechanical axis is a straight line from the
radiographs [56]. center of the femoral head to the center of the knee; the tibial
After adequate imaging, the clinician must analyze the mechanical axis is a straight line from the center of the knee
deformity to quantify the components of the deformity. to the center of the ankle mortise. No matter how deformed a
Accurate analysis is necessary to make a diagnosis and plan femur or tibia is, whether due to a malunion or a develop-
surgical correction if necessary. mental deformity, the mechanical axis is, by definition,
Table 2.5 Hip–knee–ankle angle (degrees), tibial-femoral angle axis, the anatomic axis is unaffected by abnormalities of the
(degrees), and mechanical lateral distal femoral angle (mLDFA) head or neck, such as coxa vara or avascular necrosis of the
(degrees)a
femoral head. By definition, the femoral mechanical axis
Age Hip–knee–ankle Tibial-femoral intersects the distal femur at the center of the knee, midway
(years) angleb angleb mLDFAc between the femoral condyles. The femoral anatomic axis
Mean and Mean and Mean and intersects the distal femur more medially, at approximately
standard deviation standard deviation standard deviation the junction of the concave intercondylar notch and convex
1<2 3.6 ± 5.6 3.8 ± 4.2 94.8 ± 3.8
medial femoral condyle (Fig. 2.15). The tibial anatomic axis
2<3 2.5 ± 3.2 9.1 ± 3.0 90.7 ± 3.4
is parallel and slightly medial to the tibial mechanical axis,
3<4 2.0 ± 2.8 9.0 ± 3.2 89.0 ± 2.5
but the difference is insignificant, so for most purposes the
4<5 1.7 ± 1.7 8.6 ± 2.0 87.5 ± 2.5
5<6 2.3 ± 2.6 9.0 ± 3.3 88.3 ± 1.1
two axes of the tibia can be considered identical.
6<7 1.9 ± 2.1 7.6 ± 2.0 87.7 ± 2.3 Although using the mechanical or anatomic axis to ana-
7<8 0.4 ± 2.9 6.7 ± 2.6 86.8 ± 2.2 lyze deformity should yield similar results, the mechanical
8<9 0.7 ± 2.4 6.0 ± 1.9 88.1 ± 1.9 axis method is preferred for several reasons: The goal of cor-
9 < 10 0.1 ± 2.8 6.9 ± 2.5 88.1 ± 1.9 rection is to restore the mechanical axis of the entire extrem-
10 < 11 0.6 ± 2.1 6.4 ± 2.1 87.9 ± 1.8 ity. Also, in a severely curved femur or tibia, the deformed
11 < 12 0.2 ± 2.1 6.4 ± 1.7 87.2 ± 1.8 bone will not have a single anatomic axis. In the operating
12 < 13 0.2 ± 2.5 6.5 ± 2.3 87.0 ± 2.1 room, if a sufficiently large film cassette is not available to
13 < 14 0.1 ± 2.3 6.7 ± 2.4 87.5 ± 1.9 visualize the entire femur, the anatomic axis method may
14 < 15 0.1 ± 2.3 6.6 ± 2.6 87.4 ± 1.9 need to be used to verify adequate correction.
15 < 16 1.0 ± 2.1 5.3 ± 2.1 87.7 ± 1.6 The femur and tibia have an anatomic axis in the sagittal
16 < 17 1.1 ± 2.2 5.3 ± 1.7 87.6 ± 1.5 plane as well. In the lateral radiographic view, the mechani-
17 < 18 0.1 ± 2.6 5.9 ± 2.2 87.7 ± 1.6 cal axes of the femur and tibia are almost never used; only
a
Adapted from Sabharwal et al. [13, 57] the anatomic axis is used for deformity analysis. Similar to the
b
Negative values indicate valgus angulation; positive values indicate
varus angulation
AP anatomic axis, the lateral anatomic axes of the femur and
c
Angles less than 90° indicate valgus angulation and greater than 90° tibia are straight lines determined by a series of mid-diaphyseal
indicate varus points. Since the femur has a slight bow in the sagittal plane,
the line is determined by a series of mid-diaphyseal points
a straight line from the center of the joint at the proximal end closest to the section of interest.
to the center of the joint at the distal end. Deformity analysis should be done in a stepwise manner
The mechanical axes of the femur and tibia intersect at (Fig. 2.16). The first step is to measure the lengths of the
the center of the knee to form the hip–knee–ankle angle. In entire lower extremity, femur, and tibia bilaterally. The sec-
adults, the mechanical axes of the femur and tibia are almost ond step in determining the presence or absence of deformity
collinear, forming a mean angle at their intersection at the is to draw the AP mechanical axis of the extremity and then
knee of 1.2 ± 2.3° varus [57]. Thus the mechanical axis of the answer the question: Does the anteroposterior (AP) mechani-
lower extremity nearly coincides with the mechanical axis of cal axis pass through the center of the knee? The distance
the femur and mechanical axis of the tibia with very slight from the AP mechanical axis to the center of the knee in mil-
deviation medial to the center of the knee. limeters is the MAD. The mechanical axis normally passes
In children, however, the normal hip–knee–ankle angle through the center or slightly medial to the center of the knee
ranges from 3.6 ± 5.6° varus from age 1 to 2 years to 2.5 ± 3.2° (defined as medial MAD) for children and adults with the
valgus from age 2 to 3 years, and then declines to 0.3° varus exception from ages 4 to 6 years, when it passes very slightly
after age 7 years (Table 2.5). The changes in the lower lateral to the center of the knee (lateral MAD) [13]. If there is
extremity alignment, as quantified by the hip–knee–ankle AP MAD outside the normal range, there is deformity. Medial
angle, beginning as varus until age 2 years, changing to val- MAD indicates lower extremity varus; lateral MAD indicates
gus at age 2–3 years, and stabilizing as very slight varus from valgus. The presence of MAD outside the normal range
age 7 years onward, correlate closely with similar changes in confirms the presence of deformity, but the absence of MAD
the alignment of the distal femur, and, to a lesser degree, of does not confirm the absence of deformity (such as due to
the proximal tibia [13]. abnormal but compensatory joint orientation angles).
The femur and tibia also have an anatomic axis. The ana-
tomic axis is defined as a straight line determined by a series of Joint Orientation Angles
mid-diaphyseal points. The anatomic axis is where an intra- The third step to discover deformity or to confirm correction
medullary nail is placed. In the femur, the AP anatomic axis of deformity is measurement of the joint orientation angles.
differs from the AP mechanical axis. Unlike the mechanical Normal joint orientation angles and mechanical axis within
30 J.J. Gugenheim
Fig. 2.15 Standard Standard Measurements

measurements for joint
orientation angles and Frontal Sagittal
mechanical axis deviation.
Adapted with kind permission
from Springer Science+Business
NSA = 130°
Media: Paley D. Principles of mLPFA = 90°
(124°–136°)
deformity correction. Berlin, (85°–95°)
Heidelberg; 2002 [23]
aMPFA = 84°
(80°–89°)
aLDFA = 81°
(79°–83°) 1
aPDFA = 83° 3
mLDFA = 88° (79°–87°)
(85°–90°)
JLCA = 2°
(1°–3° medial)
mMPTA = 87° aPPTA = 81° 1
5
(85°–90°) D = 10 mm (77°–84°)
(4–16 mm)
MAD = 10 mm nedial
(3–17 mm)
1 aADTA = 80°
mLDTA = 89° 2
(78°–82°)
(86°–92°)
normal values
(range ±1SD)
range of normal are both necessary and sufficient to rule out of the two supplementary angles at this site. The third letter
deformity or to confirm correction of deformity. If either is is F (femur) or T (tibia). The final letter is A, the abbreviation
abnormal, there is deformity. The femur and tibia have a for angle.
proximal joint orientation angle and a distal joint orientation Figure 2.15 shows the normal joint orientation angles for
angle on both the AP and lateral radiographs. The joint orien- the lower extremity in adults. However, the age-specific
tation angle is formed by the intersection of the joint orienta- pediatric values should be used for children less than 7 years
tion line with the axis, either mechanical or anatomic, of the of age [13].
respective bone. The joint orientation lines for the femur and A deformed femur or tibia has a deformity magnitude,
tibia are described in Table 2.6. deformity direction, and deformity level.
The joint orientation angles are abbreviated by five let- The magnitude of the deformity is the magnitude of the
ters. The first letter is a lower case “a” or “m,” which desig- angle formed by the intersection of the proximal axis and
nates anatomic or mechanical, respectively, depending on distal axis of the bone. In a normal bone, the proximal and
which axis is used to form the angle. Usually a (anatomic) or distal axes of the bone form an angle of zero; the two axes
m (mechanical) is only used to differentiate between the two are collinear.
techniques of determining the joint orientation angles of the The proximal and distal axes of the bone can be drawn by
femur in the AP plane. In the lateral view of the femur, and using the long segment method or the short segment method
AP and lateral views of the tibia, the joint orientation angle (Figs. 2.17 and 2.18).
is formed by the anatomic axes. The second letter is either M The long segment method is used if the deformity is near
(medial), L (lateral), A (anterior), or P (posterior), which the middle of the bone (mid-diaphyseal). The anatomic axis
designates the location of the angle with respect to the axis of (a series of mid-diaphyseal points) of each segment is
the bone so that the normal value of the angle is 90° or less. drawn, and the angle formed by the intersection of these
This nomenclature is not used for the femoral neck–shaft lines determines the magnitude and level of the deformity in
angle because of the long-standing convention of measuring the plane in which it is measured. The joint orientation
the relationship between the neck and the shaft as the larger angles at each end of the bone, formed by the intersection of
Measure the standard Measure the lengths If asymmetric,

lengths of the lower → of the femurs → there is limb length
extremities from floor and the tibias discrepancy
to proximal femur
Draw the mechanical If abnormal,

axes of the lower → there is deformity
extremities
Draw the AP mechanical

axes of the femur and
tibia
Draw the lateral

anatomic axes of the
tibia and distal femur
Draw the AP and lateral Measure the

joint orientation → joint orientation →
lines of the proximal angles
and distal tibia
If abnormal,
↓ there is deformity
Draw the AP and lateral Measure the

joint orientation → joint orientation →
lines of the distal femur angles
and lateral joint
orientation of the distal femur
Fig. 2.16 Steps for detecting deformity on a radiograph of the lower extremities
Table 2.6 Joint orientation lines of the femur and tibia (see Fig. 2.17)
AP plane
Proximal femur Line from tip of the greater trochanter to the center of the femoral head (mechanical axis method). Longitudinal axis of
the femoral neck (anatomic axis method)
Distal femur A line tangential to the femoral condyles
Proximal tibia A straight line from the lateral corner to the medial corner of the tibial plateau
Distal tibia A line across the subchondral bone of the distal tibia at the ankle mortise
Lateral plane
Proximal femur Neck–shaft angle but rarely used in the lateral plane
Distal femur A line connecting the anterior and posterior extent of the distal femoral physes or the site of the closed physis
Proximal tibia A line across the subchondral bone of the proximal tibial plateau
Distal tibia A line between the anterior and posterior corners of the distal tibia
32 J.J. Gugenheim
the distal short segment mechanical axis, as determined by

the mechanical lateral distal femoral angle.
Because the AP mechanical and AP anatomic axes of the
femur differ, the AP proximal femoral axis is drawn by first
drawing the proximal joint orientation line (center of the
␪ femoral head to the tip of the greater trochanter) and then
drawing a straight line distally from the center of the femoral
head with a mechanical lateral proximal femoral angle
(mLPFA) equal to the contralateral side if the contralateral
femur is normal or the population normal value (90°) if the
contralateral side is abnormal. One must not use the mechan-
ical axis at one end of the femur and anatomic axis at the
other end.
Oblique Plane Deformities

A deformity seen in both the AP and lateral views is an
oblique plane deformity. Biplanar deformity is a misnomer
because the deformity is only in a single plane (the plane of
maximum deformity) between the AP and lateral planes
(Fig. 2.19). Using the previously described techniques, the
magnitudes of the AP (coronal) plane and lateral (sagittal)
plane deformity are measured. The true magnitude of
the deformity can be determined by either the graphic or the
trigonometric method. The graphic method uses the
Fig. 2.17 The long segment method quantifies mid-diaphyseal defor- Pythagorean theorem (Fig. 2.20):
mities. It is θ, the angle formed by the intersection of the anatomic axes
of the proximal and distal segments of the femur. Adapted with kind
permission from Springer Science+Business Media: Paley D. Principles q = Ö q2 AP + q2 lat
of deformity correction. Berlin, Heidelberg; 2002 [23]
where θ = magnitude of the true or resultant angle of
deformity
the joint orientation line and the respective axis, must be θAP = magnitude of the angular deformity in the AP plane
checked. If abnormal, there is juxtaarticular deformity in θlat = magnitude of the angular deformity in the lateral plane
addition to the mid-diaphyseal deformity. Notice that the true angular deformity is greater than
If, however, one segment is too short (too close to the either orthogonal (AP and lateral) component for oblique
adjacent joint to draw a straight mid-diaphyseal line) or if plane deformities.
the joint orientation angle of a long segment is abnormal The graphic method, which utilizes the Pythagorean
(a juxtaarticular deformity), the short segment method is theorem, is a good approximation, but the exact magnitude of
used. The magnitude of the deformity is the magnitude of the the resultant angle can be calculated using the trigonometric
angle formed by the intersection of the long and short seg- method from the equation
ments. The axis of the long segment of the bone being mea-
sured is determined by drawing the axis as a series of q = tan1 Ö tan 2 qAP + tan 2 qlat
mid-diaphyseal points, and the short segment axis is drawn
by utilizing the normal joint orientation angle, determined where tan1 = arc tangent
either by the contralateral limb joint orientation angle, if nor- tan θAP = tangent of the angular deformity in the AP plane
mal, or by the population normal value. The short segment tan θlat = tangent of the angular deformity in the lateral plane
axis must be drawn at its position on the joint orientation line The graphic method is easier to use and yields an approxi-
in a normal skeleton. mation within 0° to 2° for physiologic angular deformities.
This method of determining the long anatomic axis and The direction of the deformity is the orientation plane of
short anatomic axis of the bone is valid for the femur and the true (resultant) angular deformity. It is the plane of maxi-
tibia in both the AP and lateral views. In certain situations in mum deformity magnitude. The true orientation can be
the femur, such as diaphyseal bowing or surgeon’s desire to approximated by using the graphic method (see Fig. 2.20).
restore the mechanical axis to normal, the mechanical axis Although it is only an approximation, it is quite accurate
method is used by drawing the proximal mechanical axis and within the range of physiologic deformities. The orientation
Fig. 2.18 The short segment method quantifies the deformity when eral distal femoral angle (mLDFA) is 101°, which is abnormal. (d) The
one segment is too short to draw the anatomic axis. The axis of the short proximal and distal mechanical axes of the femur are drawn from the
segment is drawn from the joint orientation lines, and the intersection of joint orientation lines. The intersection of the axes forms the angle θ,
these two axes quantifies the deformity angle θ. (a) The mechanical the magnitude of the deformity. Note that this method uses the mechan-
axis of the lower extremities is drawn, confirming the presence of a ical axes of both segments of the femur. One must not use the anatomic
deformity, as quantified by the mechanical axis deviation (MAD). (b) (diaphyseal) proximal axis with the mechanical distal axis. Adapted
The mechanical axes of the femur and tibia are drawn. (c) The joint with kind permission from Springer Science+Business Media: Paley
orientation lines of the femur and tibia are drawn. The mechanical lat- D. Principles of deformity correction. Berlin, Heidelberg; 2002 [23]
angle α can be measured by drawing two perpendicular lines The level of the deformity is the location of the intersection
representing the magnitudes of the AP and lateral angula- of the proximal and distal axes. If the level differs on the AP
tions. The angle between the resultant diagonal lines and the and lateral radiographs, there is either translation or there is
horizontal axis represents the orientation of the oblique plane an additional angular deformity on one of the views.
deformity, the direction of the true deformity angle. This The third component of angulation in the XZ plane is
angle is the angle formed by the intersection of the plane of rotation around the Y axis, currently quantified with imaging
maximum deformity with the coronal plane. studies. Since the magnitude is usually obtained by CT soft-
The trigonometric method for measuring the orientation ware, the measurement will not be discussed here.
angle α yields the exact value and can be calculated from the Translation, if present, may occur in one, two, or three
formula planes. The magnitude of translation is the length of the line
drawn perpendicular from the axis (either mechanical or ana-
tan qlat
a = tan1 tomic) of one segment to a point on the axis of the other
tan qAP segment. Developmental and congenital deformities do not
34 J.J. Gugenheim
Fig. 2.19 (a, b) Oblique plane

angular deformity of the tibia.
Radiographs show angulation on
the AP (22°) and LAT (21°)
projections of magnitude. This is
not a biplanar deformity but
rather a single plane of
angulation where the true plane
of angulation lies between the
frontal and sagittal planes. (c)
Oblique radiograph obtained
perpendicular to the plane of
angulation shows the maximum
angulation (31°). With kind
permission from Springer
Science+Business Media: Paley
D. Principles of deformity
correction. Berlin, Heidelberg;
2002 [23]
energy of impact. Like angulation, the translation compo-

nents in the orthogonal planes can be used to calculate the
true magnitude of translation and its orientation. Unlike
angulation, the graphic method using the Pythagorean theo-
rem yields the exact magnitude and orientation instead of an
approximation.
Box 2.1. Pitfalls

• For maximum accuracy, use metal tape measure,
not cloth.
• During the prone exam, stabilize the pelvis on the
exam table to avoid rocking the child side to side,
which will give a false high estimation of hip
rotation.
• Do not draw the AP mechanical axis at one end of
the femur and the AP anatomic axis at the opposite
end. The mechanical and anatomic axes are inter-
changeable elsewhere in the lower extremity, but
Fig. 2.20 The graphic method can be used to calculate the resultant not in the AP femur.
oblique plane deformity and to determine the plane of the deformity. A
vertical vector with a magnitude of 22° represents the apex anterior defor-
mity. A horizontal vector of 21° represents the apex lateral (varus) defor-
mity. The resultant angulation (30°) is represented by the diagonal of the
rectangle formed by the AP and lateral angulations on the graph. The plane
of the angular deformity is oriented 45.5° from the coronal plane. With
Laboratory Studies
kind permission from Springer Science+Business Media: Paley
D. Principles of deformity correction. Berlin, Heidelberg; 2002 [23] Serum and urine chemistry studies are rarely needed to
evaluate lower extremity deformities. However, if a rachitic
disorder is present, serum and urine studies will be helpful in
have a translation component. Translation with angulation is determining the etiology (Table 2.7).
associated with high-energy fractures, where one fragment is In summary, many youngsters are brought to the physi-
angulated and translated with respect to the other by the cian’s office by concerned parents, often very anxious to get
Table 2.7 Chemical findings in various forms of rickets [58]

Serum Urine Miscellaneous serum and urine values
Alk 25(OH) 1,25(OH)2
Type of rickets Ca2+ P Phos PTH Vit D Vit D %TRP Ca2+
Vitamin D-deficient rickets ↓ or → ↓ ↑ ↑ ↓ ↓ ↑ ↓
Dietary phosphate deficiency → ↓ ↑ → → → → →
Gastrointestinal rickets ↓ ↓ ↑ ↑ ↓ ↓ or → ↓ ↓ ↓ Absorption from gastrointestinal tract
Vitamin D-resistant rickets Amino acids, sugar, water, base in urine
I. Phosphate diabetes → ↓↓ → → → ↓↓ →
II. Reduced 1,25(OH)2 vitamin D ↓ ↓ ↑ ↑ → ↓↓ ↓ ↓
production
III. End-organ insensitivity ↓ ↓ ↑ ↑ ↑ or → ↑ or → ↓ ↓
IV. Renal tubular acidosis ↓ ↓ ↑ ↑ ↑ or → ↑ or → ↓ ↑ Na ↓, K ↓, C1 ↑; acidosis alkaline urine
Renal osteodystrophy ↓ ↑ ↑ ↑↑ ↓↓ ↓↓ ? ↓↓ BUN ↑
↑ increased, ↓ decreased, → unchanged or normal, Alk Phos alkaline phosphatase, BUN blood urea nitrogen, Ca calcium, Cl chloride, K potassium,
Na sodium, P phosphate, PTH parathyroid hormone, TRP tubular reabsorption of phosphate, Vit D vitamin D forms
treatment. When evaluating a child or adolescent with a 8. Lincoln TL, Suen PW. Common rotational variations in children.
J Am Acad Orthop Surg. 2003;11(5):312–20.
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9. Jonson SR, Gross MT. Intraexaminer reliability, interexaminer reli-
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10. Woerman AL, Binder-Macleod SA. Leg length discrepancy assess-
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ment: accuracy and precision in five clinical methods of evalua-
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Decision Making in Lower Extremity
Deformity Correction 3
Mark L. Miller and J. Eric Gordon
situations and it is important that the surgeon discuss the

Introduction various options and potential complications with the patient
and/or family prior to making a final decision.
Lower extremity deformities can vary widely with regard to
their etiology, magnitude, integrity of the soft-tissue enve-
lope, and natural history. Because of this variation, it is dif- Clinical Evaluation
ficult to create a formulaic method of approaching the patient
with a lower limb deformity. This difficulty is compounded When evaluating a patient, there is a temptation for surgeons
by differences in the patient’s environment, surgeon’s skills, to look at radiographs of the patient and make decisions
resources available to treat the patient, and ability of the regarding the ultimate treatment without taking into account
patient or family to comply with a treatment program. other factors. Like other areas of medicine, it is important for
Because of these differences, it is important to assess how the surgeon to obtain a history of the problem, perform a
each of these factors may influence the treatment method careful physical examination, and obtain appropriate labora-
that is ultimately chosen and to follow a consistent approach tory studies and imaging prior to making a diagnosis and
in analyzing the deformity and planning the surgical correc- developing a treatment plan.
tion. The nature and location of the deformity should guide When obtaining a history, it is important to elicit from the
the surgeon’s choice of implant, not vice versa. patient or family a chronologic account of the development
When approaching lower extremity deformities, it is of the deformity as well as current symptoms of pain, insta-
important to develop a comprehensive problem list outlining bility, and functional limitations. The surgeon should under-
the pathology associated with the involved limb. In some stand the limitations that the patients and their caretakers
situations these lists may be quite short, outlining only a perceive as well as their expectations of treatment. The pres-
simple uniplanar angulatory deformity. In other situations, ence of joint instability, limitation of motion, pain, neuro-
however, these lists can be quite complex, combining multi- logic symptoms, signs of infection, and previous treatment
ple deformities in different planes and locations, soft-tissue are crucial to elicit from the patient or family. Past medical
issues, joint instability, and psychological and family issues history, such as coagulopathy or bleeding issues, compro-
that can be extremely challenging. mised immune system, and chronic medical conditions, can
It is important to remember that even with a consistent also influence the decision making.
approach to decision making, it is not uncommon that mul- Examination of the patient generally starts with obser-
tiple approaches to similar clinical deformities and symp- vation of the individual’s gait. Factors that contribute to
toms may be appropriate and that there may not be a single the ultimate plan include general conditioning (for
“best” approach. Surgeon skills and patient/caretaker prefer- instance, is the patient obese?). The examination should
ences can often influence the final decision making in these also include an assessment of motion and stability of the
major lower extremity joints. Limitation of joint motion or
instability can have a direct impact on both the etiology
M.L. Miller, MD • J.E. Gordon, MD (*) and the treatment of deformities. For example, a hip adduc-
Department of Pediatric orthopedic Surgery, tion contracture can lead to the development of knee val-
St. Louis Children’s Hospital, One Children’s Place
gus in patients and attempts to correct the knee valgus
Suite 4S60, St. Louis, MO 63110, USA
e-mail: [email protected]; without addressing the hip adduction contracture can lead
[email protected] to gait problems after correction of the knee deformity as

38 M.L. Miller and J.E. Gordon
well as postoperative recurrence. Children with congenital Table 3.1 Important elements of a problem list to describe in
limb length discrepancies frequently have hip, knee, and/ planning a lower extremity deformity correction
or ankle instability of the shorter extremity, which can Uniapical deformity versus multiapical deformity
have a substantial impact on subsequent limb lengthening. Coronal, sagittal, and rotational parameters of each deformity
Additionally, rotational profile should be assessed by Soft-tissue envelope/vascularity of bone
physical examination, documenting femoral anteversion [1] Patient/caretaker perception of problem
Patient comorbidities
and tibial torsion clinically.
Prior treatment successes and challenges
Imaging studies provide information about the static
deformity in both the anteroposterior and lateral planes. It is
crucial when evaluating the radiographs that the surgeon
ascertains not only the presence of malalignment, but also some parts of the world there is a strong cultural bias against
the location of one or more deformities. This can be best accepting amputation as part of what otherwise might be a
accomplished by either mechanical axis planning or ana- preferred treatment.
tomic axis planning utilizing full-length radiographs of the
entire lower extremity including the hip, knee, and ankle.
This portion of the evaluation has been addressed in the pre- The Problem List
vious chapter. Components of the deformity can include
angulation, translation, length, and rotation. Of these compo- The history, physical examination, imaging studies, patient
nents, angulation, translation, and length can be largely and family perceptions of the problem, and dynamics of the
described from the radiographs. Rotation is often best ascer- situation should be brought together by the surgeon to form a
tained by examining the patient, although specialized rota- problem list (Table 3.1). This list should summarize the defor-
tional studies such as CT scans and MRI can complement the mities, pertinent pathology, and comorbidities as they impact
physical examination in selected situations. the treatment of the deformity, patient perceptions of the prob-
One must elucidate why the patient and/or family are lem, and potential obstacles to treatment whether they are
seeking medical care for their deformity. Is the patient sim- related to the patient or the environment in which treatment is
ply bothered by the appearance of the extremity or is the occurring. It is important to remember that the problem list is
patient having pain? Often families seek care for growing simply a problem list and not necessarily a surgical agenda.
children because of concerns not about the current state of After a problem list has been developed, the anatomy and
the limb but because of concerns that the deformity will biology of the underlying deformity must be considered.
worsen or that pain or disability will result at a later time. It In particular the surgeon should decide whether the location
is important to engage the patient and caretaker regarding the of the deformity is more amenable to acute or gradual cor-
natural history of the deformity and counsel them as to how rection based on the anatomy of the neurovascular structures
the surgical correction may or may not address their con- and postoperative or posttraumatic scarring. Are the physes
cerns. Occasionally the patient or caretaker may be seeking open, leaving the option of growth modulation to correct the
care for a complaint that is only minimally or not related to deformity? The presence of active or prior infection is
the patient’s deformity. another important factor to be considered, as are factors such
One must also evaluate the social situation of the patient as the presence of a pseudarthrosis.
as well as the psychological issues and family dynamics When considering the problem list in conjunction with
when planning correction of a deformity. In particular, the the anatomy and biology of the problem, the surgeon should
surgeon should take into account the ability of the patient be able to outline options for an appropriate treatment agenda
and family to comply with a proposed treatment regimen. for addressing the deformity comprehensively over time.
For example, gradual correction of deformities utilizing a Some of these plans may be relatively simple comprising an
circular external fixation system in a patient with autism acute correction of a uniapical deformity. Other cases may
might be ill advised. The ability of patients and families to require a much more complex plan, including a series of
comply with postoperative protocols should be evaluated interventions over a number of years such as for an infant
prior to finalizing a surgical plan and prearrangements for with proximal femoral focal deficiency.
the anticipated need for physical therapy and follow-up visits
may be necessary in certain situations. The surgeon’s skill
set and available medical resources should also be taken into Surgical Indications: General
account when developing a plan. Relevant cultural norms
should also be considered and may dictate pursuing recon- The decision about whether or not to correct a given defor-
structive options that might otherwise be contraindicated and mity can be quite individual for each patient and there are no
lead the surgeon and patient to accept perhaps less function absolute rules. Relative indications for surgical correction
in order to be more culturally acceptable. For example in of a deformity include the presence of pain or a deformity
3 Decision Making in Lower Extremity Deformity Correction 39
Table 3.2 Relative indications for surgical correction of lower only the femur or the tibia and the other bone is normal,
extremity deformity correction should nearly always occur within the affected
Persistent pain bone. If both bones are abnormal but the majority of the
Mechanical axis in zone 2 with symptoms deformity is within one of the bones, there may be some con-
Mechanical axis in zone 3 or greater with or without symptoms sideration to correcting only the bone with the majority of
Uncompensated symptomatic hindfoot deformity the deformity if less than 5° of abnormality is present in
Sagittal plane deformities impeding gait and function
either the LDFA or MPTA. If greater than 5° of abnormality
is noted in both the LDFA and MPTA, we prefer to address
the deformity at both sites.
For sagittal plane deformities about the knee, one must
consider both the bony deformity within the distal femur
and proximal tibia as well as the soft-tissue constraints
about the knee joint. The goal of treatment of sagittal defor-
mity about the knee is functional range of motion in both
extension and flexion. My preference is to develop a surgi-
cal plan that achieves knee extension within 5° of full exten-
sion and without hyperextension of greater than 5° as well
as knee flexion of at least 90°. For patients with limited
extension, one must consider concomitant hamstring, ilio-
tibial band, or posterior capsule releases while correcting
the bony sagittal deformity. Likewise, one can consider
quadriceps lengthening to augment a deformity correction
in a patient with limited knee flexion.
Surgical Indications: The Ankle
The decision to correct ankle alignment must be made in con-

junction with a careful examination of the hindfoot. Patients
Fig. 3.1 Depiction of zone of mechanical axis deviation at the with substantial deformity of the ankle may be clinically well
knee. Zone 1 is within the tibial spine. Zone 2 is within the tibial aligned with a compensatory hindfoot deformity (Fig. 3.2).
condyles. Zone 3 is within the knee joint width away from the cen- Patients with such well-compensated deformity and with
ter of the knee joint. Zone 4 is greater than one knee joint width limited subtalar motion may not be well served by a decision
from center of knee joint [22]
to correct the ankle and thus “create” a more visible defor-
mity by uncovering the fixed hindfoot abnormality.
with a natural history of substantial progression (Table 3.2).
In general, current or anticipated limb length discrepancies
greater than 2 cm should be addressed. Relative Contraindications
Contraindications to surgery are necessarily somewhat

Surgical Indications: The Knee vague and include a number of patient-related factors
including unrealistic expectations on the part of the patient
Angulatory deformities about the knee should generally be and caretakers, their inability to follow through with the
corrected if the mechanical axis falls within zone 2 and the necessary outpatient components of a treatment program, as
patient is symptomatic and should be corrected if well as situations where the risks of surgical intervention
the mechanical axis is beyond zone 2 (Fig. 3.1) even if the outweigh the potential benefits to the patient (Table 3.3).
patient is asymptomatic. When the deformity about the knee For instance, patients with severe mental illness or with
requires correction, the joint orientation angles such as the limited ability to comprehend the treatment plan may not
lateral distal femur angle (LDFA), the medial proximal tibial have the ability to comply with more complex treatment
angle (MPTA), and the joint line congruency angle (JLCA) protocols including gradual correction using external fixation.
should be measured. If the JLCA is more than 5° in conjunc- Although more complex treatment protocols may be con-
tion with a bony deformity, we prefer to address the ligamen- traindicated in these patients, simplified alternatives may
tous laxity as well. If the deformity about the knee involves represent better choices. For example, for some patients the
Fig. 3.2 Radiographs (a) and clinical images (b) of a patient with distal tibial valgus and compensatory hindfoot varus. Note the radiographic
valgus of the left ankle and the apparent normal clinical alignment of the left hindfoot
Table 3.3 Relative surgical contraindications to performing a circular external fixator. In these cases a tibial osteotomy
lower extremity deformity correction
can be performed while leaving the fibula intact and secur-
Unrealistic patient/family expectations ing it distally to the tibia. The angulatory deformity is then
Patient/family unable to comply with postoperative protocol corrected while lengthening gradually, thus transporting the
Potential complications of treatment outweigh benefits to patient fibula distally in relation to the proximal tibia resulting in
tightening of the lateral collateral ligament (Fig. 3.4).
difficulties involved in complying with instructions and a

physical therapy program associated with a limb lengthen- Physeal Modulation or Ablation
ing may be able to comply easily with the less strenuous
instructions and therapy associated with a closed femoral Physeal modulation can take many forms. Physeal bar resec-
shortening (Fig. 3.3). tion following traumatic or developmental partial arrest can
be performed successfully in situations where a partial arrest
of the physis, as documented by progressive deformity, is
Surgical Options present that involves less than 50 % of the physis with more
than 2.5 cm of growth remaining at the involved physis and
The plan for each deformity may comprise any of a number more than 2 years of growth expected [2, 3]. Advantages of
of different techniques that might be applicable in a given physeal bar resection include the relative simplicity involved
situation (Table 3.4). There is no compelling reason to avoid in postoperative care and relatively rapid patient recovery
combining techniques when clinically applicable. General and return to activity (Fig. 3.5). Disadvantages of physeal
categories that should be considered include soft-tissue sur- bar resection include a high failure rate and frequent late clo-
gery, physeal bar resection, growth modulation, acute cor- sure of the physis necessitating close follow-up and further
rection with internal or external fixation, or gradual surgical treatment.
correction with external fixation. A brief description of the Complete epiphysiodesis by percutaneous drilling or utiliz-
techniques follows with a general description of indications ing either plates or staples can be performed in skeletally
and contraindications. immature individuals who have adequate growth remaining to
contribute to the correction of a limb length discrepancy [4].
This has been recommended in situations where the projected
Soft-Tissue Modification limb length discrepancy is between 2 and 5 cm. The technique
however can be utilized in larger limb length discrepancies as
Soft-tissue surgery can be effective either as a solitary pro- part of a comprehensive treatment plan. Temporary hemiepi-
cedure or in conjunction with bony surgery. Modification of physiodesis using medial and lateral staples or plates has been
the soft tissues is not limited to procedures that directly reported [5] but is not widely utilized due to concerns about
approach the soft tissues themselves but can also be an permanent physeal closure and possibility of rebound growth
intended consequence of bony procedures. In particular, following implant removal.
tightening of the lateral collateral ligament at the knee can Hemiepiphysiodesis by plate, screw, drilling, or Blount’s
be performed by translating the fibular head distally either staples should be considered in patients who are skeletally
with a fibular osteotomy and gradual bone transport with an immature and who have adequate growth available to rea-
external fixator or in cases of adolescent tibia vara utilizing sonably expect correction of an angulatory deformity [6].
Fig. 3.3 Femoral shortening

case study. (a) Preoperative
radiographs of a 17-year-old
female with a 7 cm left greater
than right limb length
discrepancy due to a right-sided
posttraumatic femoral growth
arrest. (b) Intramedullary saw
completing distal cut within
femur. (c) Intramedullary saw
beginning proximal cut within
femur 7 cm proximal to distal
cut. (d) Completion of proximal
intramedullary cut. (e)
Intramedullary wedge splitting
the medial cortex of the
intercalary segment. (f)
Completion of splitting
intercalary segment. (g)
Reduction of proximal and distal
segment with solid
intramedullary nail. (h)
Postoperative radiographs
demonstrating healed femur after
closed femoral shortening
These deformities can be in the coronal, sagittal, or oblique Acute Correction with Osteotomy
planes (Fig. 3.6). Physeal modification can be utilized even
in situations where the physis is abnormal [7] such as chronic Osteotomies with acute correction are particularly indicated
renal failure and hypophosphatemic rickets. Deformities in angulatory deformities in patients with minimal limb
such as proximal tibial valgus following a Cozen’s-type frac- length discrepancies allowing correction of the deformity
ture which tend to recur following osteotomy correction are without placing undue stress on the neurovascular structures.
particularly amenable to treatment with plate hemiepiphys- Most often the acute correction is combined with rigid inter-
iodesis. Following correction of the deformity in a patient nal fixation although it can be utilized in conjunction with
who has substantial growth remaining, the metaphyseal external fixation or Kirschner wire fixation and cast immobi-
screw can be removed percutaneously and the plate and lization. The choice of implants is determined by the site of
epiphyseal screw can be left in place allowing continued the osteotomy and can potentially include intramedullary
growth (Fig. 3.7). If the deformity recurs, the metaphyseal fixation, screw fixation, locking plates, or non-locking plates.
screw can be replaced percutaneously resulting in re- Intramedullary fixation can be helpful when combined
hemiepiphysiodesis and repeat correction. with percutaneous diaphyseal osteotomy techniques such
as in patients with an isolated femoral rotational deformity
Table 3.4 Available techniques to [8, 9]. Intramedullary stabilization can also be helpful when
perform deformity correction angulatory deformities exist in the diaphyseal region after
Soft-tissue modification either traumatic malunion or in cases of hypophosphatemic
Physeal modulation or ablation rickets with residual femoral lateral bow (Fig. 3.8). In these
Osteotomy with acute correction cases often the percutaneous osteotomy can correct both
Osteotomy with gradual correction the apparent genu varum deformity and the coxa vara that
Fig. 3.4 Case example of

adolescent tibia vara treated with
circular external fixator and
tightening of the lateral collateral
ligament. (a, b) AP and lateral
preoperative radiographs of a
12-year-old male with tibia vara.
(c, d) Immediate postoperative
radiographs depicting a ring
circular external fixator with
fixation of the fibula distally into
the tibia and no proximal
transfibular fixation. (e)
Postoperative radiographs after
fixator removal showing distal
transport of the fibula relative to
the proximal tibia, thus
tightening the lateral collateral
ligament
Fig. 3.5 Case example of a 9-year-old female with a posttraumatic placed on the epiphyseal side to limit reformation of bar. (f) Immediate
physeal bar formation in the right distal femur. (a) Standing AP demon- postoperative standing AP. (g) Standing radiographs demonstrating
strating right distal femur physeal bar. (b, c) Coronal and sagittal slice growth within the distal femoral physis 2 years postoperatively. (h)
of a CT scan showing lateral distal femoral bar with less than 20 % of Standing anteroposterior radiograph demonstrating continued growth
physis involvement. (d) Fluoroscopic image showing curettage of phy- with migration of the cranioplast proximal to the physis with resultant
seal bar. (e) Postoperative AP after physeal bar resection. K-wires are recurrence of the physeal bar associated with a limb length discrepancy
placed in order to follow eventual growth; radiolucent cranioplast is and valgus deformity 3 years postoperatively
Fig. 3.6 Oblique plane deformity of the right distal femur in a 6-year- medial distal femur, promoting distal femoral varus and procurvatum.
old male with arthrogryposis. (a) Standing AP demonstrating right dis- (e, f) Standing AP and maximal extension lateral views 18 months post-
tal femoral valgus. (b) Lateral of the right knee in less than maximal operatively with resultant correction of deformity. The patient had full
extension showing a knee flexion contracture of approximately 25°. (c, knee extension
d) AP and lateral of the knee showing hemiepiphysiodesis of the antero-
can produce a significant abductor lurch during gait. The in the epiphyseal region that require rigid control of the
disadvantage of intramedullary fixation is the limitation in osteotomy. One specialized use of these screws is to stabi-
the magnitude of the correction that can be obtained. lize intra-articular osteotomies in the proximal tibia. This
Intramedullary stabilization results in a comparatively can be performed in cases of medial tibial plateau depres-
straight diaphyseal segment when combined with a diaphy- sion due to delayed treatment of early-onset tibia vara with
seal osteotomy. Intramedullary stabilization can also be uti- a resulting “pagoda” deformity [10]. These tibial plateau
lized with blocking screws in conjunction with acute osteotomies can be effectively stabilized after an acute cor-
correction of a supracondylar distal femoral or proximal rection using cannulated screws with an incomplete osteotomy
tibial osteotomy but this use is technically difficult for the and allograft cortical bone as a structural graft (Fig. 3.9).
surgeon who is not familiar with the use of such screws. Plates provide a rigid method of stabilizing osteotomies
Proper mechanical alignment of the proximal and distal following an acute correction. They are particularly useful in
segments can also be obtained with temporary external fix- stabilizing metaphyseal osteotomies after correction of either
ator assistance prior to intramedullary reaming and fixation angulatory or rotational deformities. Plates utilized can be
with an intramedullary nail. Other contraindications to conventional plates, locking plates, or blade plates [11]. In
intramedullary fixation include open physes with substan- particular, blade plates and locking plates (Fig. 3.10) can be
tial remaining growth in the proximal tibia and the inability used to stabilize osteotomies that have little or no inherent
to pass an intramedullary nail thorough an obstructed intra- stability and locking plates can be utilized with a submuscu-
medullary canal. In addition intramedullary nail should be lar technique that may minimize soft-tissue dissection.
generally avoided in the presence of active infection. Advantages to their use include the ability to begin early
Screw fixation provides the surgeon with the ability to motion at adjacent joints, rigid fixation, and immediate cor-
stabilize osteotomies with some inherent bony stability rection of the deformity (Fig. 3.11). Disadvantages to the use
while achieving rigid correction of the deformity. Cannulated of conventional plates are the relatively larger amount of
screws are particularly helpful when performing osteotomies soft-tissue dissection that is necessary, the lack of ability to
Fig. 3.7 Four-year-old male

with a proximal tibial valgus
deformity after proximal tibia
fracture (Cozen’s fracture). (a)
Standing AP after cast treatment
for right proximal tibia fracture.
(b) 6-month postinjury film
demonstration proximal tibial
valgus deformity. (c)
Fluoroscopic image showing
placement of hemiepiphysiodesis
plate medially. (d) Immediate
postoperative standing AP. (e)
9-month postoperative
radiographs showing normal
mechanical axis. (f) Metaphyseal
screw is removed after deformity
correction in order to facilitate
replacement of
hemiepiphysiodesis if deformity
recurs
adjust the correction postoperatively, and the difficulties monolateral external fixation, circular external fixation utilizing
associated with soft-tissue coverage in areas with little over- the classic Ilizarov-type hinges and distractors, or computer-
lying soft tissue with resulting issues with infection. controlled six-strut circular external fixation. Most recently,
self-lengthening nails have combined some of the advan-
tages of gradual correction with internal fixation [12]. Self-
Gradual Correction with Osteotomy lengthening nails however provide no ability to correct
angulatory deformities gradually but do allow for acute cor-
The workhorse of complex deformity correction is gradual rection of angulatory deformities with gradual lengthening.
correction. Gradual correction is effective in treating defor- Advantages of gradual correction include minimal soft-tissue
mities with components of angulation, rotation, translation, dissection and early weight bearing with the ability to deal
and/or length. Gradual correction can be achieved with with problems that arise during the postoperative period and
Fig. 3.8 Use of intramedullary nail for deformity correction. (a) of pediatric femoral nail. As the osteotomy was performed at the isthmus
Standing AP of 12-year-old female with X-linked hypophosphatemic and deformity, this results in an opening wedge osteotomy medially. (e)
rickets and midshaft bowing of both femurs at the isthmus. (b) Postoperative standing AP demonstration correction of right femoral
Fluoroscopy demonstrating percutaneous diaphyseal femoral osteotomy deformity. A proximal tibial osteotomy with gradual correction using a
using drill. (c) Completion of diaphyseal femoral osteotomy. (d) Insertion circular external fixator was used to correct the tibial deformity
Fig. 3.9 Twelve-year-old male with Blount’s disease and proximal simulating correction of intra-articular deformity. (e, f) Intraoperative
tibia intra-articular deformity. (a) Standing AP demonstrating intra- fluoroscopy and postoperative radiographs demonstrating fixation with
articular proximal tibial deformity and medial growth arrest large fragment screws after structural bone graft placement. (g) Healed
(Langenskiold type VI). (b, c) Fluoroscopy demonstrating drill holes osteotomy sites with persistent proximal tibial varus. Tibial plateau
along the path of intra-articular osteotomy for tibial plateau elevation, elevation corrects intra-articular deformity but often does not correct
completion of osteotomy with an osteotome. (d) Laminar spreader overall varus deformity completely
Fig. 3.10 Fourteen-year-old female with Blount’s disease. (a) Standing restoration of mechanical axis. (d) Lateral fluoroscopic view. (e, f)
AP demonstrating right proximal tibia varus deformity. (b) Oblique Postoperative AP and lateral view showing correction of deformity.
osteotomy heading from distal medially to proximal laterally with (g) 18-month postoperative standing AP demonstrating deformity
care to preserve lateral cortex. (c) Fluoroscopic image demonstrating correction
Fig. 3.11 Sixteen-year-old female with distal femoral valgus deformity. blade plate correction of deformity. (d) Postoperative radiographs demon-
(a) Standing AP demonstrating distal femoral valgus deformity. (b, c) AP strating restoration of mechanical axis. Of note, patient had a concomitant
and lateral fluoroscopic images showing distal femoral osteotomy with derotational distal tibial osteotomy for excessive external tibial torsion
Fig. 3.12 Thirteen-year-old

female with tibial pseudarthrosis,
status post-multiple attempts at
correction and healing with Peter
Williams rod. (a, b) AP and
lateral of the right tibia
demonstrating deformity and
occurrence of a new distal tibial
pseudarthrosis. (c, d) AP and
lateral views of Ilizarov
application with multilevel
osteotomy, proximal osteotomy
for tibial lengthening, and distal
tibial osteotomy for deformity
correction and bone transport
into pseudarthrosis site. (e, f) AP
and lateral views after frame
removal and pseudarthrosis
healing
the ability to “fine-tune” the correction to optimize final systems tend to be disliked by patients and issues with pain
alignment [13–16]. Disadvantages of gradual correction and pin tract problems are often noted [17–21]. As the lower
include complex postoperative care requiring substantial limb deformities become more complex, the degree of required
compliance with a physical therapy protocol and rigid adher- patient compliance and the physician learning curve also
ence to a follow-up schedule by the patient and family with the increase substantially. The trade-off, however, is the increas-
possibility of damaging adjacent joints by the injudicious use ing ability to deal with complex three-dimensional deformi-
of distraction at an osteotomy site. In addition, external fixation ties and achieve accurate corrections (Figs. 3.12 and 3.13).
Fig. 3.13 Ten-year-old male with posteromedial bowing and limb otomy was performed for lengthening and a midshaft osteotomy for
length discrepancy. (a, b) Standing AP and lateral view demonstrating deformity correction. (d, e) AP and lateral view demonstration of heal-
deformity and limb length discrepancy. (c) AP radiograph showing ing of regenerate and correction of deformity. (f) Standing AP showing
application of bi-level circular external fixator. A proximal tibial oste- restoration of mechanical axis and correction of leg length discrepancy
have become more readily available and accessible, and the

Summary complexity of deformities that can safely be addressed has
increased dramatically. It is still important to start with a com-
In summary, decision making in lower extremity deformity prehensive evaluation of each patient and his or her environ-
correction is a necessary but arduous task. One must develop ment and apply the principles of deformity correction to
a complete problem list which addresses the pertinent diffi- develop a customized treatment plan.
culties associated with the patient’s deformity, perceived
problem, and ability of the caretaker and patient to implement
the plan of care. This problem list then should help guide the References
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Growth Modulation for Angular
and Length Correction 4
Peter M. Stevens
The appeal of temporary, reversible, epiphysiodesis is that,

Introduction while being more cost effective, it circumvents the above list
of iatrogenic problems. Haas was the first to document this,
As pediatric orthopedists, we are often consulted to evaluate using a wire loop, initially in dogs and eventually in human
and manage evolving deformities of the lower extremities subjects [7, 8]. Soon thereafter, in 1947, Blount introduced
that, left untreated, may have lifelong, adverse impacts upon his staple for correction of angular deformities and limb
gait and the spine. Generally speaking, the era of “corrective length inequality and this technique was widely practiced for
shoes,” orthotics, and bracing has yielded to surgical man- decades [9]. Because the physis is dynamic and the forces of
agement, because the natural history of pathological defor- growth so powerful, there was a propensity for staples to
mities is inexorable progression. In 1933, Phemister eventually (or precipitously) bend, break, or migrate
introduced the concept of open epiphysiodesis for the man- (Fig. 4.1). The use of multiple staples failed to consistently
agement of angular deformities and limb length inequality address these problems. Furthermore, staple removal often
[1]. With the advent of fluoroscopy, this method evolved to proved challenging, sometimes resulting in local bone loss
the comparatively less invasive percutaneous growth arrest that could compromise the reversibility of this technique.
[2–4]. However, the acknowledged drawback of such abla- This placed doubt upon the wisdom and necessity of using
tive methods is that they are permanent and, hence, irrevers- such rigid implants; their popularity has therefore declined.
ible. Therefore, open or percutaneous epiphysiodesis must Frustrated by some of the aforementioned problems with
be delayed until adolescence and perfectly timed, in order to stapling, the concept of using an extra-periosteal, non-
avoid over- or under-correction of a given deformity. The locking plate, serving as a tension band, was developed in
vagaries of determining skeletal maturity and of parental 2002, and introduced in 2004 [10, 11]. Since then, similar
compliance and timely follow-up may make this goal opti- implants have been introduced into the market, worldwide
mistic [5]. The same drawbacks pertain to other experimen- (Fig. 4.2). It is not critical which implant one selects; rather
tal methods of chemical, electrical, or radiofrequency it is the tension band principle that counts. However, one
ablation, which are permanent and irreversible. should avoid locking plates because the rigid construct will
Given these limitations many still consider osteotomy to predispose to implant failure.
be the “gold standard” for progressive angular deformities In resource-challenged regions of the world, the 8-plate or
such as Blount’s disease, rickets, and idiopathic genu val- facsimiles may not be available. In such an environment, an
gum. Some feel compelled to cut bone(s) in order to correct option is to take a 1/3 semi-tubular plate or a pelvic recon-
associated torsional deformity. However, the potential disad- struction plate and cut it into 2-hole segments [12–14]. The
vantages of osteotomy include blood loss, neurovascular potential disadvantages relate to the relative bulkiness or
compromise, immobilization, over- or under-correction, and stiffness (will not reverse bend) when compared to the real
malunion or nonunion [6]. Comparative cost, cast immobili- device. Some favor the use of quad plates (4-hole) in heavy
zation, weight-bearing restrictions, unsightly scars, and dif- children, particularly with Blount’s disease. Inevitably, a
ficult hardware retrieval must be considered as well. variety of plate shapes have been proposed (step-off, hinge
plates, H-plates) and the controversy is unresolved as to
P.M. Stevens, MD (*) whether titanium or stainless steel implants are superior.
Department of Orthopedics, University of Utah,
Unfortunately, there is currently no literature to support or
100 North Mario Capecchi Drive #4550, Salt Lake City,
UT 84113, USA refute these ideas. Suffice it to say that any device that is flex-
e-mail: [email protected] ible and permits differential growth can work.

52 P.M. Stevens
Fig. 4.1 A 4-year-old girl, with

posttraumatic tibia valga
(Cozen’s phenomenon), treated
by stapling. Rigid implants are
subject to failure through
bending, breakage, or migration,
as a result of the inexorable
forces of physeal growth. Note
the extrusion of her single staple;
occurring inexplicably, 9 months
following implantation
Deformities of the Lower Extremities
General
With the notable exception of physiologic varus and valgus,

lower extremity deformities often progress with growth. While
angular deformities at the knee are most obvious and perplex-
ing, one must examine the patient for limb length discrepancy,
rotational deformities, and ligamentous laxity that represent
confounding factors. Also, examine the hip and ankle clini-
cally and, when indicated, radiographically, for coexisting or
compensatory angular deformities. If the deformity is unilat-
eral, then the normal leg serves as a comparison. However,
oftentimes deformities are bilateral and not necessarily sym-
metrical. One must be cognizant of remaining growth and the
predicted natural history of a given problem. If a deformity is
thought to be physiologic or borderline in severity, a follow-up
after 6 months may clarify this and affect the timing of inter-
vention. If there is concurrent deformity of the hip and/or
ankle, simultaneous guided growth may be employed [17].
Fig. 4.2 Since the introduction of the 8-plate in 2004, many facsimiles
have been produced worldwide and used successfully for guided
growth. The common denominators for success are low profile and Angular: Frontal
non-locking
Physiologic
An alternative strategy, popularized by Metaizeau,
involves the insertion of large-diameter (6.5 or 7.3 mm) can- It is well recognized that children under the age of two may
cellous screws across one or both sides of the physis (percu- present with symmetrical and painless bowing of the legs,
taneous epiphysiodesis using transphyseal screws, or PETS) associated with ligamentous laxity and intoeing. With obser-
[15]. While also minimally invasive, the theoretical objec- vation alone, all components of this deformity will spontane-
tion is the necessity of violating the physis with a large ously correct, without treatment. While there is no need for
threaded implant. Series reports of PETS are largely con- intervention, parental education, reassurance, and periodic
fined to the adolescent age group [16]. It has not been follow-up are tantamount to success. Likewise, physiologic
described in younger children or in sagittal or oblique plane genu valgum should be managed expectantly, without brac-
deformities. Thus, PETS has comparatively narrow ranges of ing, physical therapy, or shoe modification, up until the age
age and applications and its potential reversibility remains in of 6. These self-limiting conditions are a contraindication to
question. surgical intervention of any kind [18–20].
4 Growth Modulation for Angular and Length Correction 53
Fig. 4.3 (a) This 19-month-old girl with Schmid-type chondroepiphy- remains asymptomatic, with neutral alignment. Note the indirect reso-
seal dysplasia, presented with a waddling gait and hip pain (due to lution of her ankle varus. Her femoral neck fractures remain healed; the
stress fractures of the femoral neck). (b) 13 months following bilateral, plates will be left in situ, pending further growth. Proximal femoral
3-level guided growth (outpatient/no cast), her mechanical axis neutral; osteotomies appear to be unlikely
her gait has become normal and hip pain abated. (c) At age 6, she
Pathologic such as ankle valgus or coxa vara that may also lend them-
selves to the same principle and technique. Timely guided
In contradistinction to the above-noted problems, the patho- growth may prevent or forestall the need for distal tibial or
logic deformities may be unilateral or bilateral and multi- proximal femoral osteotomies. In some patients, multilevel
level. The etiology may be idiopathic, developmental guided growth, to simultaneously address concomitant defor-
(Blount’s), acquired (Cozen’s), metabolic (rickets), genetic mities, is efficient and well tolerated [17] (Fig. 4.3).
(neurofibromatosis, hereditary multiple exostoses, Schmid
dysplasia), or dysplastic (fibrous dysplasia, Ollier’s), to
name a few. Deformities may occur in the frontal, sagittal, or Treatment
oblique plane, compounded by torsion and ligamentous lax-
ity. They are insidious in their progression, and refractory to Timing/Angular
attempts at orthotic management or physical therapy.
Regardless of the underlying etiology, individually and When dealing with progressive angular deformities, the previ-
collectively these deformities constitute the indication for ously preferred method of epiphysiodesis—now largely of his-
guided growth, as the treatment of choice, instead of osteot- toric interest—involved permanent arrest by either open [1] or
omy (salvage procedure). Clinically, genu valgum is often percutaneous techniques [21, 22]. These required serial scano-
associated with anterior knee pain and a circumduction gait; grams and bone age determinations with calculations using
patellar instability may ensue. It is documented by measur- various methods [23–25]. Guided growth with tension band
ing the intermalleolar distance when standing and prone. plates is more forgiving in terms of timing and calculations. On
Genu varum may also be seen with knee pain and a wad- a standing AP teleroentgenogram, the relative limb lengths and
dling, Trendelenburg-positive gait, often in association with mechanical axis are documented. The latter normally will
intoeing, ligamentous laxity, and lateral thrust. Furthermore, bisect the horizontal knee at an 87 degree angle. The mechani-
when the deformity is unilateral, progressive, relative limb cal axis should fall within the center two quadrants (zones +/−
length discrepancy is common. 1) of the knee (Fig. 4.4). When it is in medial or lateral 2 or 3,
While knee deformities represent, by far, the most common surgical intervention is generally warranted. If two levels of
indication for guided growth, there are specific deformities deformity, for example femur + tibia, are identified, then pan
54 P.M. Stevens
Table 4.1 Anatomic angles [26]

Abbreviation Anatomic angles Degrees
MPFA Medial proximal femoral angle 90
LDFA Lateral distal femoral angle 81
PMTA Medial proximal tibial angle 87
LDTA Lateral distal tibial angle 87
PPTA Posterior proximal tibial angle 81
PDTA Posterior distal tibial angle 90
JCA Joint congruence angle 0–3
Apex medial
fect timing or resorting to osteotomy. The risks of delaying sur-

gical realignment include compensatory deformity of the hip,
knee, or ankle, ligamentous laxity, and patellar instability.
Technique/Angular
Under tourniquet control, the designated physis is approached,

through a 3-cm incision. The dissection is deepened through
fascial and submuscular planes, down to the bone, preserving
the periosteum. A Keith needle is inserted into the physis,
checking its position in the frontal and sagittal planes, with the
fluoroscope. The plate is then inserted, placing the small cen-
Fig. 4.4 The mechanical axis (dotted red line) comprises a vertical line ter hole over the Keith needle to align it with the physis. It is
connecting the center of the femoral head to the center of the ankle; desirable to position the plate midsagitally, unless an oblique
normally, this bisects the knee. If you divide the knee into quadrants,
medial zones (−) 1–3 reflect increasing varus and lateral zones (+) con-
plane deformity is being corrected. For example, a knee flexion/
stitute valgus. While zone 1 may warrant observation, zones 2 or 3 call varus deformity may call for a single anterolateral tension
for treatment, in order to protect the physes and the knee band plate. One plate per physis is usually sufficient. The
smooth 1.6-mm guide pins are inserted; they need not be par-
genu guided growth is indicated, in order to preserve a horizon- allel, but must avoid the physis and joint. Each hole is pre-
tal knee joint line. drilled to a depth of 5 mm in order to facilitate advancement of
In children older than 10, the anatomic angles, as codified the 4.5-mm cannulated screws, without undue torque.
by Paley and Sabharwal et al. (Table 4.1), can be useful to While screw length is arbitrary, and sizes may be mixed, the
monitor during correction; these reference joint/shaft orien- longest practical screw is usually recommended. Each is
tation for the femur and tibia [26, 27]. The joint congruence inserted to be snug with the plate. It is then important to remove
angle is typically 0° (parallel lines), indicating ligamentous the guide pins, which may be binding on the screw, and further
balance/integrity. There may be concomitant deformity in tighten each screw to countersink within the plate. This should
both the femur and tibia. By envisioning the knee joint hori- be documented fluoroscopically. This critical maneuver will
zontally, one can readily detect multilevel deformities. For avoid 3-point bending stresses, reported in some patients with
example, in patients with Blount’s disease, there may be con- Blount’s disease, which could produce stress fracture of the
comitant varus or valgus deformity of the distal femur that screw. This is a potential technical error that may compromise
must be taken into account [26]. any screw, be it titanium or stainless steel, cannulated or solid.
To benefit from guided growth, the age of the patient is Once the hardware is secured, the wound is closed in layers and
not critical, provided that there is at least a year of predicted a soft dressing applied. Most patients are discharged from
growth remaining. Guided growth has been safely and success- same-day surgery with (optional) crutches. Full weight-bearing
fully utilized in patients ranging from 18 months to 18 years in and activities are encouraged as tolerated.
chronologic age. The latter figure reflects the delayed/prolonged
growth observed in some children with neurologic delays or
metabolic syndromes. Commencing as young as age 4, patients Follow-Up
with progressive or symptomatic deformity (such as Klippel-
Trenaunay-Weber, Beckwith-Wiedemann, neurofibromatosis), Routine follow-up is recommended every 3 months (for
it may be preferable to correct the problem, even if it requires angle) or every 6 months (for length) until correction is
repeated guided growth subsequently, rather than trying for per- achieved (Fig. 4.5). For angular correction, most deformities
Fig. 4.5 (a) This 13-year-old girl presented with “patellofemoral dys- deformities completely corrected within 10 months of pangenu plate
plasia” manifest by bilateral patellar instability. (b) Clinically, her inter- application and her patellae stabilized, without direct intervention. (d)
malleolar distance measured 20 cm. Her mechanical axis is lateral zone Radiographic comparison of before vs. 10 months after pangenu guided
3. It was apparent that idiopathic genu valgum (femur + tibia) was the growth, whereupon the plates were removed. (e) “Sunrise” radiographs
proximate cause of her patellar dislocations and recognized that an demonstrating improvement in the patellofemoral sulcus depth, pre-
MPFL reconstruction or patellar tendon transfer would not suffice to sumably as a consequence of normalizing the mechanical axis and
solve her problems. (c) Having hit her adolescent growth spurt, her directing the patella in front of the femur
56 P.M. Stevens
will be resolved within 12–18 months. Certain syndromes Often in children with lower limb spasticity bilateral,
and bone dysplasias such as achondroplasia may require FKFD results in a progressive crouch gait that is refractory to
more time. There is no proven limit as to how long one can bracing and physical therapy. The shortened stride length
safely restrict one side of the physis. Since growth is unabated and anterior knee pain, often seen with radiographic evi-
by the presence of a unilateral flexible tether, premature dence of patella alta +/− avulsion fractures, may preclude
physeal closure is unlikely. Clinical correction is corrobo- sustained ambulation. Supracondylar osteotomy of the femur
rated by full-length, weight-bearing teleroentgenogram, not- +/− patellar advancement has enjoyed recent popularity.
ing limb lengths and the mechanical axis. In children younger However, recurrent deformity is common and represents a
than 10, or with delay in skeletal maturity, it may be advis- major drawback.
able to allow slight overcorrection of the mechanical axis An alternative approach is to employ guided growth of the
into medial or lateral zone 1. In the event of rebound/recur- distal anterior femur to gradually correct the sagittal or
rent deformity, the process may be repeated. Once the screws oblique plane deformities. It is important to place the plates
have reached maximum divergence, the narrow waist of the medial and/or lateral to the sulcus (intracapsular, but nonar-
plate accommodates reverse bending of the plate, facilitating ticular) in order to avoid irritation of the patella [28]. While
further correction. This characteristic may not be shared by the initial postoperative pain and crepitus may exceed the
the more rigid quad plates. expectations of the parents and physical therapist, these
symptoms abate as the legs gradually straighten. In contra-
distinction to osteotomies or external frames, knee flexion
Angular: Sagittal/Oblique range is not compromised. Postoperative bracing [usually
floor reaction] may still be helpful until the crouch deformity
Knee fully corrects. Typically, this takes 12–18 months following
guided growth treatment. Overcorrection into recurvatum is
Fixed knee flexion deformity (FKFD) may be associated rare, as is recurrence. In the authors’ experience, simultane-
with conditions including cerebral palsy, spina bifida, and ous or subsequent advancement of the patellar tendon is usu-
arthrogryposis. It is important to differentiate between ally not required because knee pain typically abates as the
dynamic and fixed contracture, because the treatment is very knee gains extension and patella alta is rendered unimport-
different. Both conditions will limit the straight leg raise; ant. Furthermore, concomitant hamstring lengthening is also
with the former, the popliteal angle will change as the hip is unnecessary. In fact, as the knee straightens, the hamstrings
flexed in the supine position. In addition to the typical supine may actually pull upon the ischium, extending the pelvis and
straight leg raise, it is helpful to examine the patient prone, alleviating the hip flexion deformity, while preserving hip
with the hip extended, relaxing the hamstrings. If the knee extensor power. Finally, apparent equinus is improved indi-
remains flexed and the foot does not reach the table, this rectly as the knee extends and the heel may reach the floor.
indicates a fixed rather than dynamic knee deformity that Thus, by primarily addressing the FKFD with anterior ten-
will not respond to hamstring recession (Fig. 4.6). sion band plates, “single-event, multilevel” correction is
realized. In contradistinction to supracondylar osteotomy,
posterior capsulotomy, or frames, knee flexion is preserved
while extension is gained (Fig. 4.7).
Another application for sagittal plane correction of the
proximal tibia is shown in Fig. 4.8. In this posttraumatic
deformity, there was no defined physeal bar. Had one been
identified, guided growth may be employed, at the time of
epiphysiolysis, to expedite angular correction.
Ankle/Frontal
Progressive ankle valgus is common and insidious sequelae

of a variety of conditions (Table 4.2). The detection is more
Fig. 4.6 Examination in the prone position places the hip in maximum challenging than knee deformities because of the relatively
extension, thereby eliminating the hamstrings as a source of deformity. short height of the foot. It may be mistaken for an
With fixed knee flexion deformity (45° shown here) the ankle does not
overcorrected club foot, planovalgus, or pronated foot. The
rest on the table. A hamstring recession will not resolve this deformity.
An extension osteotomy may be averted with guided growth of the ante- critical radiographic study is a weight-bearing AP of the
rior distal femur ankles. The normal lateral distal tibial angle (LDTA) should
Fig. 4.7 (a) This 7-year-old patient with congenital fixed knee flexion plates to the anterior distal femur, one on each side of the sulcus. (c) In
deformity (FKFD) had a previous extension osteotomy (arrow) at age contradistinction to osteotomy, or frame application, knee extension is
3, with the inevitable recurrence of her deformity. Anterior femoral achieved, without losing correction. Regarding patella alta, if the
staples migrated and had to be removed. (b) Intraoperative images show patient is ambulatory and pain free, there is no requirement that the
the technique of sagittal plane correction by applying tension band patellar tendon be advanced
be approximately 90°. As the deformity progresses, there is reaction force shifts laterally. This situation does not lend
often lateral pain around the ankle and hindfoot due to itself to correction with orthoses.
impingement of the distal fibula upon the talus or calca- A simple method of treatment is guided growth of the dis-
neus. The distal fibular epiphysis may enlarge in response tal medial tibia. While a transphyseal screw can work, one is
to the increased weight-bearing load imposed as the ground applying a rigid implant across the open and very dynamic
58 P.M. Stevens
Fig. 4.8 (a) This 8-year-old girl suffered a proximal tibial transphy- diverging screws. (c) 12 months postoperatively, the screws continue
seal fracture on a trampoline. While the fracture healed uneventfully, to diverge, as a result of redirected growth. The opposite knee is shown
she presented with a symptomatic, 20° recurvatum deformity. The pre- for comparison. She has become asymptomatic. When the recurvatum
operative lateral view demonstrates posterior proximal tibial angle of is fully corrected, the metaphyseal screw will be removed, percutane-
102°, with the normal value being 81°. (b) 6 months following poste- ously. Her growth will continue to be monitored on a regular basis and
rior guided growth, to effect correction in the sagittal plane, note the the screw may be reinserted p.r.n.
Table 4.2 Etiology of ankle valgus

Dysplastic Neuromuscular Genetic Posttraumatic
Club foot Cerebral palsy Rickets (or metabolic) Fracture
Postaxial hypoplasia Spina bifida Down syndrome Osteotomy
Tibial pseudarthrosis Neurofibromatosis MHE
physis. This may lead to implant failure or difficult removal. manner, a posterior plate may address calcaneus deformity
It is preferable to utilize a flexible, extra-periosteal tension of the ankle. If the deformity is greater than 15°, however, an
band that will produce more rapid correction and is simple to osteotomy may be required.
remove. In comparison to the knee, the time to correction of
the ankle is slower, often taking 18–24 months. It is recom-
mended to overcorrect into slight varus (LDTA of up to 95°), Complications of Guided Growth
in anticipation of rebound valgus, which is relatively common.
The concerns expressed regarding hardware prominence Perioperative complications pertaining to tension band plate
have noit borne out in practice. However, if the child is wear- insertion are decidedly rare, provided that the routine pre-
ing an AFO, it is desirable to bend or pad the brace p.r.n. to cautions are exercised. These include protecting and preserv-
avoid rubbing. ing the periosteum, and avoidance of violating the physis or
joint with the screws. With respect to the latter, it may be
helpful to perform an arthrogram in young children, in order
Ankle/Sagittal to outline the entire chondroepiphysis (Fig. 4.9).
There have been reports of broken screws. Most com-
At the distal tibia, one may insert an anterior plate for resid- monly this occurs in Blount’s disease and involves the
ual fixed equinus or a posterior plate for calcaneus deformity, metaphyseal screw, not at the head/shank junction, but where
rather than resort to supramalleolar osteotomy. In a similar the exposed screw shank enters the cortex. This may occur
Fig. 4.9 (a) A 3-year-old girl with Cozen’s posttraumatic genu valgum, tibia for length inhibition and distal medial tibia for valgus. (e)
presenting 18 months following proximal tibial fracture. Note the small 15 months later, her length discrepancy measures 1.2 cm and the ankle
hemangioma on the instep (arrow). (b) An arthrogram was employed to valgus has resolved. (f) Note the asymmetrical growth line in the left
better delineate the chondro-epiphyses and aid in placing the guide distal tibia, with correction of the ankle valgus to neutral. The strategy
wires and cannulated screws. (c) Full correction of the angular defor- is to wait for 9 months, allowing slight overcorrection into ankle varus,
mity took 20 months, whereupon the implant was removed. Follow-up and then remove the metaphyseal screws from the distal and proximal
continued to monitor for recurrent valgus and LLD. (d) At age 7, she is plates. Following 6 months of reprieve from guided growth, allowing
noted to have a 2.5 cm discrepancy and symptomatic ankle valgus. growth to resume, the metaphyseal screws may be reinserted. Follow-up
These were attributed to her congenital hemangioma causing growth will be continued until maturity, repeating this process of screw
imbalance. This was managed via bifocal guided growth—proximal removal/reinsertion as indicated
regardless of the material (stainless vs. titanium), design Without corroborative evidence, these choices reflect sur-
(cannulated vs. non-cannulated), or size of the screw geon preference.
(Fig. 4.10). This situation is best avoided by anticipation and The issue of rebound growth imbalance and recurrent
awareness. Steps to avoid this situation include the follow- deformity, which is more common under the age of 10, is not
ing: (1) remove guide pins and alternately tighten the screws a true complication. Rather, it is an educational challenge,
to bend the plate in situ, or (2) remove plate and increase its requiring the surgeon to establish parental expectations,
convex bend to better contour to the lateral cortex of the maintain vigilance, and pursue meticulous follow-up. The
tibia. When encountered, broken screws may be percutane- rebound deformity phenomenon can generally be detected
ously exchanged, as needed, rather than resorting to osteot- within 12 months of implant removal. Properly informed,
omy. Other options to consider are the use of dual plates or most parents (and patients) would prefer repeat guided
quad plates, and the choice of solid (non-cannulated) screws. growth to any osteotomy.
60 P.M. Stevens
see the growing child once or twice a year to document any

progression and intercede accordingly. While shoe lifts may
be prescribed, they are compensatory and few children will
consistently wear them.
Pathologic
Whether the etiology is congenital, developmental, or

acquired, if the predicted discrepancy will exceed 1.2 cm at
maturity, then surgical treatment may be warranted. It is
important to see the growing child at least annually, in order
to identify the trend (static vs. progressive), educate the par-
ents, and anticipate the predicted discrepancy at maturity.
The most practical annual radiograph to obtain is the tele-
roentgenogram, which is a full-length, weight-bearing AP of
the legs [29]. It is helpful to level the pelvis with an appropri-
ate height wooden block under the shorter leg. If one uses a
calibration marker, then relative limb lengths may accurately
be measured, along with the mechanical axis [30].
Traditionally, shoe lifts were employed until adolescence
when open or percutaneous epiphysiodesis was undertaken.
Some authors still favor permanent epiphysiodesis over
reversible instrumented techniques [31]. However, problems
inherent in determining skeletal maturity have frequently led
Fig. 4.10 If there is no contact between the plate and tibial metaphysis, to over- or under-correction, especially when using perma-
the screw be subject to 3-point bending, regardless of the screw size and nent and irreversible techniques. With the advent of reversible
material. This 4.5-mm solid stainless screw demonstrates such a techni-
cal error techniques—formerly using staples and more recently, extra-
periosteal tension band plates—the timing is much more for-
giving and the philosophy has changed accordingly.
Management
Weighing the potential for rebound growth, causing recurrent Treatment: Length
deformity (more common under the age of 10), may be desir-
able to permit slight overcorrection (of varus into medial zone Timing: Over vs. Under the Chronologic
1 valgus—or vice versa). This will extend the “mileage” of a Age of 10
given procedure. If rebound is deemed likely, one option is to
remove just the metaphyseal screw (Fig. 4.11) and continue The typical application for guided growth inhibition is dur-
observation, reinserting the screw if and when rebound is ing adolescence, hoping to achieve limb equalization at skel-
documented. In other words, the modular nature of reversible etal maturity. Regardless of the implants chosen (staples,
guided growth lends itself to a myriad of conditions and situ- tension band plates, Metaizeau PETS), there is a latency
ations. Frequent minimally invasive, outpatient surgeries may effect before longitudinal growth is actually inhibited. This
be preferable to major, more invasive techniques. should be taken into account and epiphysiodesis undertaken
6–12 months in advance of what was formerly deemed
“ideal.” Some authors prefer drilling the physis, in order to
Length: Anisomelia avoid implants and the potential need for removal [31].
However, by using reversible technology, there is leeway
Physiologic because the implants may be removed if correction occurs
before skeletal maturity is reached.
It is widely accepted that limb length discrepancy of up to Comparatively young children with “gigantism” due to
1.2 cm is well tolerated by most adults, requiring no treatment. specific conditions such as Trevor’s Disease, Klippel-
When lesser discrepancies are identified, it is appropriate to Trenaunay-Weber, hemangioma, Beckwith-Wiedemann
Fig. 4.11 As a sequela of DDH,

this 12-year-old girl became
symptomatic with a 2.5-cm limb
length discrepancy. Three years
following guided growth, her
limb lengths are equal
syndrome, and neurofibromatosis may be candidates (start- Treatment/Length

ing as young as age 4) for intermittent longitudinal restraint
of the overgrown limb, via guided growth (Fig. 4.12). Despite utilizing the same implants and surgical approaches,
Similarly, children with limb dysplasia, such as postaxial there are subtle differences in technique. Specifically, the
hypoplasia, may be considered for serial/intermittent guided screws are placed in a divergent orientation within the plate
growth of the normal, longer limb. The goal is to forestall, (Fig. 4.13). This will mitigate against screws bending and
minimize, or perhaps obviate limb lengthening, with all of may reduce the aforementioned latency period.
its associated risks.
In patients undergoing major long bone resection/limb
salvage for oncologic disease, intermittent guided growth of Complications/Length
the contralateral limb may mitigate some of the problems
encountered with expanding implants. When employing this Length correction is less dramatic than angular. Consequently,
strategy, it is important to respect the 2-year window of follow-up every 6 months is sufficient for monitoring prog-
opportunity, because, if a given physis is restrained indefi- ress. The proximal lateral tibia may pose problems and lead
nitely, the physis could permanently shut down. Therefore, to iatrogenic genu varum. This problem may be encountered
one strategy is to remove the metaphyseal screws, wait for whether one uses staples or tension band plates. If such an
6 months, and reinsert them (see Figs. 4.9 and 4.12). This angular drift is noted, one should remove the medial tibial
may serve as an adjunct to contralateral limb lengthening, or plate [or metaphyseal screw], allowing gradual normaliza-
circumvent the need for it altogether. tion of the mechanical axis, and reimplant when the alignment
62 P.M. Stevens
Fig. 4.12 (a) This girl presented with hemangioma causing a progres- 2-cm discrepancy and varus drift of the mechanical axis into zone 2.
sive LLD (4.5 cm), left hip and back pain, and projected 6- to 7-cm This was managed by reinserting the lateral metaphyseal screw in the
discrepancy (if untreated) at maturity. (b) She started at 4 + 10 years tibia and percutaneously drilling the proximal fibular physis. When
with guided growth of the proximal tibia. The fibula was not promi- the axis returned to neutral over the ensuing 8 months, medial screws
nent, and thus was not included. (c) 2 years later, her discrepancy were added. (g) A summary of guided growth procedures as of age
measured 3.5 cm. The empirical tolerance of physeal restraint is 11 + 4, when her limb lengths became equal for the first time in her
2 years. (d) Therefore, the tibial metaphyseal screws were removed life. The financial savings and reduced risk, as compared to osteot-
and femoral plates added. (e) Asymptomatic at age 9, with a 2.5 cm omy/shortening of the involved leg, or frame/lengthening of the normal
discrepancy (would have been 5–6 cm by now). (f) At age 10, with a right leg, are obvious
Fig. 4.12 (continued)

64 P.M. Stevens

Fig. 4.13 When effecting

angular correction, the screws are
placed more or less parallel to
each other and there is no time
limit. Most angular deformities
correct within 12–18 months.
When placed for length
inhibition, the screws are placed
divergent to each other. This may
reduce the lag time and avoid
screw bending. After 2 years of
growth inhibition, one may
remove the implant, or just the
metaphyseal screw(s), wait for
6 months, and repeat the process
p.r.n.
is neutral (see Fig. 4.12). In some cases, unopposed fibular 6. Steel HH, Sandrow RE, Sullivan PD. Complications of tibial oste-
otomy in children for genu varum or valgum. Evidence that neuro-
growth may contribute to the deformity. If this is suspected,
logical changes are due to ischemia. J Bone Joint Surg Am.
or if the fibular head is relatively prominent, it may be desir- 1971;53(8):1629–35.
able to percutaneously drill the proximal fibular physis. 7. Haas SL. Retardation of growth by a wire loop. J Bone Joint Surg
Am. 1945;27:25–36.
8. Haas SL. Mechanical retardation of bone growth. J Bone Joint Surg
Am. 1948;30A(2):506–12.
Summary 9. Blount WP, Clarke GR. Control of bone growth by epiphyseal sta-
pling: a preliminary report. J Bone Joint Surg Am. 1949;31A(3):
The classic method of epiphysiodesis, described eight 464–78.
10. Stevens PM. Guided growth: 1933 to the present. Strategies Trauma
decades ago, has evolved to a more versatile, less invasive,
Limb Reconstr. 2006;1(1):29–35.
and reversible method, such as employing a low-profile ten- 11. Stevens PM. Guided growth for angular correction: a preliminary
sion band plate. This technique lends itself to the correction series using a tension band plate. J Pediatr Orthop. 2007;27(3):
of both angular deformities and limb length discrepancy and 253–9.
12. Böhm S, Krieg AH, Hefti F, Brunner R, Hasler CC, Gaston
may safely be applied in very young children, without the
M. Growth guidance of angular lower limb deformities using a one-
risk of permanent physeal closure [provided the periosteum third two-hole tubular plate. J Child Orthop. 2013;7(4):289–94.
is protected]. Bilateral and multilevel deformities may be 13. Jamil K, Abdul Rashid AH, Ibrahim S. Guided growth implants for
addressed, on an outpatient basis. The complication rate and low-income to middle-income countries. J Pediatr Orthop B.
2013;22(6):608.
relative cost are very low, compared to osteotomy. This
14. Lin TY, Kao HK, Li WC, Yang WE, Chang CH. Guided growth by
method should be considered the treatment of choice for a a stainless-steel tubular plate. J Pediatr Orthop B. 2013;22(4):
wide variety of conditions, reserving osteotomy for salvage 306–10.
situations. 15. Métaizeau JP, Wong-Chung J, Bertrand H, Pasquier P. Percutaneous
epiphysiodesis using transphyseal screws (PETS). J Pediatr Orthop.
1998;18(3):363–9.
16. Lykissas MG, Jain VV, Manickam V, Nathan S, Eismann EA,
References McCarthy JJ. Guided growth for the treatment of limb length dis-
crepancy: a comparative study of the three most commonly used
1. Phemister DB. Operative arrestment of longitudinal growth of surgical techniques. J Pediatr Orthop B. 2013;22(4):311–7.
bones in the treatment of deformities. J Bone Joint Surg Am. 1933; 17. Stevens PM, Novais EN. Multilevel guided growth for hip and knee
15:1–15. varus secondary to chondrodysplasia. J Pediatr Orthop. 2012;
2. Bowen JR, Leahey JL, Zhang ZH, MacEwen GD. Partial epiphys- 32(6):626–30.
iodesis at the knee to correct angular deformity. Clin Orthop Relat 18. Salenius P, Vankka E. The development of the tibiofemoral angle in
Res. 1985;198:184–90. children. J Bone Joint Surg Am. 1975;57(2):259–61.
3. Canale ST, Russell TA, Holcomb RL. Percutaneous epiphysiodesis: 19. Heath CH, Staheli LT. Normal limits of knee angle in white
experimental study and preliminary clinical results. J Pediatr Orthop. children – genu varum and genu valgum. J Pediatr Orthop. 1993;
1986;6(2):150–6. 13(2):259–62.
4. Ogilvie JW. Epiphysiodesis: evaluation of a new technique. 20. Kling TF, Hensinger RN. Angular and torsional deformities of the
J Pediatr Orthop. 1986;6(2):147–9. lower limbs in children. Clin Orthop Relat Res. 1983;176:136–47.
5. Blair VP, Walker SJ, Sheridan JJ, Schoenecker PL. Epiphysiodesis: 21. Inan M, Chan G, Littleton AG, Kubiak P, Bowen JR. Efficacy and
a problem of timing. J Pediatr Orthop. 1982;2(3):281–4. safety of percutaneous epiphysiodesis. J Pediatr Orthop. 2008;
28(6):648–51.
66 P.M. Stevens
22. Bowen JR, Johnson WJ. Percutaneous epiphysiodesis. Clin Orthop 27. Sabharwal S, Zhao C, Edgar M. Lower limb alignment in children:
Relat Res. 1984;190:170–3. reference values based on a full-length standing radiograph.
23. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method J Pediatr Orthop. 2008;28(7):740–6.
for predicting limb-length discrepancy. J Bone Joint Surg Am. 28. Klatt J, Stevens PM. Guided growth for fixed knee flexion defor-
2000;82-A(10):1432–46. mity. J Pediatr Orthop. 2008;28(6):626–31.
24. Moseley CF. A straight-line graph for leg-length discrepancies. 29. Machen MS, Stevens PM. Should full-length standing anteroposte-
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25. Heyworth BE, Osei DA, Fabricant PD, Schneider R, Doyle SM, length discrepancy? J Pediatr Orthop B. 2005;14(1):30–7.
Green DW, et al. The shorthand bone age assessment: a simpler 30. Stevens PM. Radiographic distortion of bones: a marker study.
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569–74. 31. Stewart D, Cheema A, Szalay EA. Dual 8-plate technique is not as
26. Paley D. Principles of deformity correction. Berlin: effective as ablation for epiphysiodesis about the knee. J Pediatr
Springer; 2002. Orthop. 2013;33(8):843–6.
Physeal Bar Excision
5
Karl E. Rathjen and Anthony I. Riccio
Growth disturbance can occur from physical loss of the

Introduction physis, altered physeal physiology without loss of physeal
structure, or disruption of normal physeal architecture.
As with any clinical dilemma in the growing child, manage- Disruption of normal physeal architecture may produce an
ment of a physeal growth disturbance begins with careful osseous bridge across the cartilaginous physis, which is
consideration of the natural history of the pathologic pro- termed a physeal bar (Fig. 5.1). However, it is important to
cess. Appropriate treatment of a physeal arrest starts with realize that physeal growth disturbance may produce defor-
understanding the patient and disease characteristics that mity without physeal arrest (Fig. 5.2). The etiology of a phy-
may act in concert to create limb deformity. Knowledge of seal growth disturbance appears to have some bearing in the
the nature and characteristics of the arrest, an understanding successful restoration of growth following resection of a
of normal physeal behavior, and an appreciation of factors physeal bar. In the authors’ experience, growth arrests asso-
influencing future growth allow the clinician to assess the ciated with fractures and infantile Blount’s disease carry a
potential for deformity if a physeal arrest was left untreated. better prognosis for resumption of growth following epi-
Appreciation of these factors is also essential for generating physiolysis than arrests resulting from infection, radiation,
realistic expectations for restoration of normal growth fol- or intraosseous space-occupying lesions [11]. As the treat-
lowing surgical intervention. To ensure optimal outcome, the ment strategies can differ based upon the etiology of a growth
treatment choice must be tailored to the individual patient disturbance, an understanding of the nature of the physeal
and, in many instances, the individual physis. disturbance is essential.
Etiology and Prognosis Trauma
Physeal injury can occur via both direct and indirect mecha- It is well documented that the physis’ cartilaginous nature
nisms. While physeal fractures account for the majority of provides a path of minimal resistance to forces traversing an
physeal arrests and premature physeal closures, growth dis- extremity. The majority of physeal fractures course through
turbance is also reported following osteomyelitis, vascular the hypertrophic zone of the growth plate, which is mechani-
insult, radiation exposure, as well as neoplastic and tumor- cally the weakest zone of the physis and therefore the most
like conditions [1–12]. Infantile Blount’s disease is yet susceptible to disruption. Physeal fractures that progress
another cause of physeal growth disturbance. Unlike other from the hypertrophic zone into the adjacent metaphysis
etiologies, physeal arrest in infantile Blount’s disease is (Salter–Harris II fractures) course away from the germinal
likely the result of sustained altered physiologic function and proliferative zones and therefore away the regions of
within the physis itself [3, 13, 14]. maximal growth and metabolic activity. As a result, these
fractures generally have a lower rate of physeal arrests than
those that course from the hypertrophic zone, through the
K.E. Rathjen, MD (*) • A.I. Riccio, MD germinal and proliferative zone and towards the epiphysis
Department of orthopedic Surgery, Texas Scottish Rite Hospital
[15] (Salter–Harris III and IV fractures) [16, 17] (Fig. 5.3).
for Children, University of Texas Southwestern Medical Center,
2222 Welborn Street, Dallas, TX 75219, USA In addition to the Salter–Harris classification, fracture
e-mail: [email protected]; [email protected] location can be prognostic for the development of a growth

Fig. 5.1 AP radiograph of
bilateral ankles. Note the “Harris
growth arrest” lines (arrowheads)
on both sides. On the right ankle
the Harris line is asymmetrical
and terminates medially at the
area of physeal arrest or “bony
bar” (arrow)
Fig. 5.2 (a) A 2-year-old with a history of neonatal sepsis. MRI at this femur. (c) With restoration of normal limb alignment and mechanical
age showed no physeal bar and a relatively normal cartilaginous epiph- forces, the secondary ossification of the distal femur begins to ossify.
ysis. Despite the significant diaphyseal deformity and appearance of the This is an excellent example of the Heuter-Volkmann principle. (d)
secondary ossification center, the patient was felt to have mild (albeit 9 years postoperatively, there has been relatively normal growth. Note
long-standing) growth disturbance without physeal arrest. (b) the absence of angular deformity, the pelvic obliquity associated with
Intraoperative radiograph with opening wedge osteotomy and arthro- limb length inequality, and the premature closure of the ipsilateral distal
gram which demonstrates normal cartilaginous anatomy of the distal femoral growth plate
Fig. 5.3 Salter–Harris type II, type III, and type IV injuries. Note that the germinal zone of the physis is disrupted in the type III and IV injuries
5 Physeal Bar Excision 69
Neoplasm and Tumorlike Conditions
Growth disturbance from physeal injury has been reported

in the presence of both malignant and benign neoplasms
[18, 19].
Physical encroachment of the growth plate by these
lesions is the usual mechanism of physeal damage, although
additional injury can occur during treatment (surgical or
radiation). Growth disturbance resulting from space-
occupying lesions can be challenging to treat as it is often
difficult to predict the degree of altered growth resulting
from irradiation and the behavior of physeal tissue pre-
served following tumor resection or a limb salvage opera-
tion. Small physeal bars are only rarely encountered,
usually obviating epiphysiolysis as a surgical option.
Completion epiphysiodesis and staged or concurrent defor-
mity correction or limb lengthening procedures are more
commonly required.
Vascular Insult
Limb hypoperfusion due to either severe systemic hypoten-

sion or local limb ischemia resulting from vascular injury,
altered venous outflow, or compartment syndrome can result
in altered physeal physiology and growth disturbance
[20–22].
This relatively rare cause of abnormal growth plate func-
Fig. 5.4 AP bilateral lower extremities of a 4-year-old with a history of tion has been associated with both partial and complete phy-
neonatal sepsis. Note the shortening and angular deformity of the right
seal arrests. A small proportion of growth arrests without an
leg. Note also that the central growth arrest has created intra-articular
abnormality of the right knee. Note the irregularities in the left proximal obvious attributable cause may in fact have resulted from
and distal femoral epiphyses unrecognized transient vascular injury insufficiently follow-
ing local trauma without fracture or injury remote to the area
arrest. Fractures involving the distal femoral physis and, to a of arrest in the ipsilateral limb.
lesser extent, those involving the medial malleolus are noto-
rious for relatively high rates of growth arrest [11, 17].
Other Causes of Physeal Injury
Infection Rare etiologies of growth plate disturbance include burns,

frostbite, electrical injury, radiation exposure, and repeti-
Osteomyelitis and septic arthritis can lead to both physeal tive stress injuries. Growth disturbance due to repetitive
growth disturbance and growth arrest. As with trauma, physeal stress is commonly encountered in the young athlete.
destruction from musculoskeletal infection can result in both Altered proximal humeral physeal growth is well
complete and partial growth disturbance resulting in angular described in children who engage in extensive overhead
deformities, altered joint morphology, and limb length differ- throwing activities [23, 24]. Fortunately, cessation of the
ences [7]. Widespread systemic neonatal sepsis can result in offending activity usually results in restoration of normal
multifocal physeal arrests and severe deformity (Fig. 5.4). physeal function.
70 K.E. Rathjen and A.I. Riccio
Assessment of the Abnormal Physis
Timely identification of a growth disturbance, differentiation

between partial and complete physeal arrests, determination
of the location and extent of a physeal bar, and an accurate
assessment of remaining growth of the involved physis are
all necessary for complete assessment of the abnormal phy-
sis and selection of appropriate treatment.
Timely Identification
Early identification of a physeal growth disturbance usu-

ally affords the clinician a wider array of management
options and can simplify management by allowing for
treatment of the growth arrest without concomitant man-
agement of an associated deformity. Careful serial radio-
graphic follow-up of physeal injuries is recommended
until the clinician is confident regarding the resumption of
normal growth. Even when normal growth ensues, radio-
graphic surveillance until skeletal maturity is advised as
physes with a history of injury are known to close prema-
turely [25]. If identification of a growth disturbance is
delayed and a significant acquired deformity has devel-
oped, both the deformity and the growth disturbance usu-
ally require treatment.
Fig. 5.5 AP bilateral lower extremities of a 9-year-old who sustained a

distal femoral physeal fracture 2 years earlier. Note the premature com-
Partial Versus Complete Physeal Arrest plete closure of the left distal femoral physis and the pelvic obliquity
associated with mild limb length inequality
The effect of a bar (bony, cartilaginous, or fibrous) on the
adjacent physeal growth varies with location and size of the
arrest. Large bars and those bars associated with extensive Location of Physeal Arrest
injury to surrounding physeal cartilage usually retard growth
of the entire physis causing a complete growth arrest Partial growth arrests are classified based upon their location
(Fig. 5.5). Such bars usually result in a limb length discrep- within the physis. Determination of location of a physeal bar
ancy without associated intra-articular or angular deformity. is important, as it is predictive of the potential deformity that
Smaller central bars and those that are more peripherally might ensue if left untreated. Furthermore, the choice of sur-
located usually result in a partial physeal growth disturbance. gical approach for a bar resection is largely dependent upon
As the bar causes local restriction of growth in the presence its location.
of normal physeal function remote to the bar, angular defor- Physeal bars are generally classified as central or periph-
mity, limb length difference, tenting of the physis, and intra- eral [10]. Peripheral bars, as the name suggests, involve an
articular deformity can ensue (see Fig. 5.4). outer edge of the growth plate. If left untreated, peripheral
Resumption of growth following resection of a physeal bars are likely to create marked angular deformity in a child
bar relies upon the integrity and health of the normal physis with considerable growth remaining. We typically classify
surrounding the physeal bar. There is no role for physeal bar peripheral bars as Type A arrests (Fig. 5.6). Central bars are
resection in the presence of a complete growth arrest of the sub-classified based upon the amount of healthy physis sur-
entire physis; therefore, differentiating between a partial and rounding the arrest. Type B arrests are surrounded circum-
complete disturbance is the first step in developing a man- ferentially by normal physeal cartilage. As the normal physis
agement strategy. continues to grow around a Type B bar, tenting of growth
Fig. 5.6 Anatomic classification of physeal arrest. Type A: Peripheral. (ed). Tachdjian’s Pediatric Orthopedics: From the Texas Scottish Rite
Type B: Central surrounded by normal physis. Type C: Central travers- Hospital for Children. 5th ed. Philadelphia: Elsevier; 2013
ing the physis completely. Reprinted with permission from Herring JA
plate, and eventually the epiphysis, can develop. This may planes and that this exercise can ensure that the surgeon has
ultimately result in distortion of the underlying articular a comprehensive understanding of the three-dimensional
cartilage. A Type C arrest is a variation of a central bar in anatomy of the physis [3, 25, 28–31]. This nuanced knowl-
which the area of arrest traverses the entire growth plate in edge will likely aid the surgeon who attempts resection of a
one direction with healthy physis present on the remaining physeal arrest.
two sides. This type of arrest is often seen following Salter–
Harris III and IV fractures of the medial malleolus in which Plain Radiographs
physeal bars frequently extend from anterior to posterior Standard orthogonal radiographs are an appropriate initial
with normal physis found medially and laterally (an example diagnostic study for any patient with a suspected physeal
is shown in Fig. 5.10). arrest. Though advanced imaging modalities allow for more
Resection of a bar while minimizing concomitant damage accurate determination of the presence, extent, and exact
to the surrounding normal physis is of paramount importance location of a physeal arrest, much information can be gar-
regardless of the type of arrest encountered. The importance nered from plain radiography. Physeal narrowing and areas
of classification lies in the fact that accomplishing this goal of bridging can often be seen using plain radiographs prior to
in the presence of central bars (Types B and C) may be more the development of deformity (as demonstrated in Figs. 5.1
challenging than in those located peripherally. and 5.5). More readily determined, however, are the pres-
ence and orientation of growth arrest lines, which often are
the earliest heralds of altered growth [4]. These thin sclerotic
Extent of Physeal Arrest lines develop at the growth plate during most physeal insults.
If normal growth resumes, this line becomes progressively
Determination of the extent of physeal involvement in a par- distant from the physis. If normal symmetric physeal growth
tial growth plate arrest is the next step in developing an resumes, the arrest line will remain parallel to the physis. In
appropriate treatment strategy. Size of a physeal bar, typi- the presence of a growth arrest, the line (if the arrest is
cally documented as a percentage of the entire physis, has peripheral) or lines (if the arrest is central) will converge
been shown to correlate with successful outcomes following towards the area of abnormal growth. Identification of such
resection surgery [4, 11, 26, 27]. Successful restoration of convergence should alert the clinician to a likely arrest and
growth has been reported to be more likely in bars that com- will usually prompt additional imaging to assess the extent
prise less than 25 % of the involved growth plate [27]. While of the bar (see Fig. 5.1).
it is important to note that no firm cutoff exists as to a per-
centage of physeal area beyond which bar resection should Computed Tomography
not be attempted, restoration of meaningful growth follow- Computed tomography (CT) is often the imaging modality
ing excision of arrests that involve greater than 50 % of the of choice for assessment of a physeal bar. A CT scan can
growth plate is less likely. Although three-dimensional generally be obtained quickly and without sedation, which is
imaging is now nearly universally used to assess the extent often necessary for magnetic resonance imaging (MRI).
of a physeal arrest, we believe that it is important to under- Modern image reconstruction and manipulation software
stand the process of “mapping” a physeal arrest in two usually allow for excellent visualization of both normal and
Fig. 5.7 (a) AP bilateral lower extremities of an 8-year-old with infan- of a “map” which defines the area of physeal closure. (d) Early postop-
tile Blount’s. (b) Coronal MRI demonstrating a small physeal bar in a erative and final standing radiographs. Note the resumption of growth
proximal tibia (arrow). (c) Modern software can facilitate construction as demonstrated between the markers (arrows)
diseased physis, thereby allowing for very accurate determi- determined by which physis is involved and the age of the
nation of the extent and location of a physeal bar (see also patient. A number of studies have assessed longitudinal limb
Fig. 5.10a) [30]. growth and allow general estimates of yearly growth from
each long bone physis [35–37]. As physiologic or skeletal
Magnetic Resonance Imaging age may differ significantly from chronological age; the for-
MRI is a highly sensitive test for the detection and delinea- mer is important to consider when assessing growth poten-
tion of areas of physeal arrest. This study allows for the tial. Traditionally, this is most commonly achieved using
localization of both normal physeal cartilage and regions of standards for skeletal age published in atlas format using
bony bars. Furthermore, the use of three-dimensional MRI radiographs of the left hand and wrist [38]. Skeletal age can
utilizing fat-suppressed spoiled gradient-recalled echo also be determined using AP and lateral radiographs of the
sequences allows for manual reconstruction of the growth elbow [39, 40]. This information, in conjunction with the
plate, thereby allowing the clinician to accurately map an knowledge that extremities typically grow until a bone age of
area of growth arrest which can be invaluable in developing 14 in females and 16 in males, can provide estimations of the
a surgical plan for epiphysiolysis (see also Fig. 5.7c) [3, 29, amount of growth remaining in a particular physis.
31, 32]. Accurate mapping of a physeal bar should include
localization in relation to known and identifiable anatomic
markers which can be useful during surgical resection [33, Surgical Indications
34] (Fig. 5.7).
Traditional indications for physeal bar excision include a
growth plate arrest occupying less than 50 % of the involved
Growth Remaining physis in a child with more than 2 years or 2 cm of growth
remaining at the involved physis. Interestingly, there is sur-
The greater the growth potential in a physis, the more likely prisingly little scientific evidence to support this “dogma” [4,
it will resume growth following resection of a physeal bar, 12, 27]. Because of the modest results of physeal bar resec-
and the greater the benefit from the restoration of growth. tion in most modern reports, we believe that physeal bar
The amount of growth remaining from a particular physis is resection is ideally performed in patients in the first decade
of life, and patients closer to skeletal maturity (i.e., only 3 or Volkmann principal. If acute angular correction is performed,
4 years of limb growth remaining) may be better treated with careful consideration and planning must be given to deter-
completion epiphysiodesis, with or without a contralateral mine appropriate fixation of the osteotomy. Fortunately,
epiphysiodesis to mitigate any limb length difference. many of these patients are young and can be managed with
Consideration can be given to resection of larger bars in very relatively simple constructs including variations of “pins and
young children as success might spare the child from multi- plaster” (Fig. 5.11). Obviously, more extensive internal or
ple subsequent procedures. Lastly, excision of a physeal external fixation is another option.
arrest is ideally performed prior to the development of angu-
lar deformity. Once deformity is present, consideration must
be given to concomitant corrective osteotomy. Surgical Approaches (Tips and Tricks)
Once the need for osteotomy to correct angular deformity

Surgical Technique and Pitfalls has been assessed, the determination can be made regarding
the best approach to resect the physeal bar. Peripheral bars
Once the determination has been made to resect a physeal are simplest and are approached directly (Fig. 5.8). Central
bar, meticulous preoperative planning should address the fol- bars can be more challenging and can be addressed in a vari-
lowing considerations: need for concomitant deformity cor- ety of ways, including through a metaphyseal window or
rection, approach for bar resection, interposition material tunnel (perhaps most common) (Fig. 5.9), percutaneously
following resection, and radiographic markers in the epiphy- (ideal for Type C bars as illustrated in Fig. 5.10), and directly
sis and metaphysis to aid in assessment of future growth. through an osteotomy (most commonly an option when
performing concomitant angular deformity correction).
Although distraction through the physis prior to bar resection
Role of Osteotomy has been reported [41], we have no experience with this tech-
nique and have concerns that distraction across the physis
The decision to surgically correct any concomitant angular may further damage the germinal layer and lead to premature
deformity should be the first consideration when planning a closure. The surgeon should select and approach that will
physeal bar resection. In our experience, it is unlikely that a give him or her the confidence that he or she can reach the
successful bar resection will “overgrow” to correct angular entire extent of the bar. Most commonly the bony bar is
deformity. Furthermore, we believe that it would be unwise resected with a high-speed burr. It is paramount that the sur-
to plan for “guided growth” via physeal tethering in an geon visualizes normal physis separating the epiphysis and
injured physis. Thus, osteotomy and acute correction of metaphysis. Again this is relatively straightforward for
deformity are often the most attractive option when a physeal peripheral bars but can be more challenging for central bars.
bar is associated with clinically unacceptable angular defor- Fiber-optic lighted suction, dental mirrors, and arthroscopes
mity. Acute correction of the angular deformity to restore may aid in confirming a normal physis “360°” around the
normal alignment has the added theoretical benefit of elimi- resection of a central bar. Additionally, radio-opaque dye can
nating any asymmetrical forces across the physis which may be placed in the resection bed to allow fluoroscopic assess-
be contributing to abnormal physeal growth via the Heuter- ment of the extent of the resection (Figs. 5.9 and 5.11).
Fig. 5.8 Illustration of

peripheral physeal bar resection
Fig. 5.9 Illustration of central physeal bar resection. Reprinted with permission from Herring JA (ed). Tachdjian’s Pediatric Orthopedics: From
the Texas Scottish Rite Hospital for Children. 5th ed. Philadelphia: Elsevier; 2013
Fig. 5.10 (a) Coronal and sagittal CT scan demonstrating Type C cen- guide to resect the bony bar. Resection is confirmed with a curette. (d)
tral bar following a Salter–Harris IV fracture of the medial malleolus. Final appearance after radiographic markers are placed to facilitate
(b) Intraoperative fluoroscopy is used to place a guide pin through the postoperative assessment
area of physeal closure. (c) A cannulated reamer is placed over the
Interposition Material is no longer clinically available.) While a few recent investi-

gations have assessed the possibility of cartilaginous cells as
Once the surgeon is satisfied that the bar has been completely an interposition material, they are, to our knowledge, not yet
excised, interposition material should be placed in an effort clinically viable [42–44]. Subsequently, for peripheral bars
to limit recurrence. Interposition materials have included we ensure that the adjacent periosteum has been widely and
bone wax, fat, silicone, and cranioplast [4, 6, 9, 27]. (Silicone sharply excised from the resection area and apply bone wax
Fig. 5.11 (a) Lateral X-ray and MRI showing a recurvatum deformity can be used to document the extent of the resection. (e) Local fat is
and an anterior physeal bar (arrow) following a proximal tibial frac- harvested to use as an interposition material. (f) Lateral radiograph
ture. (b) Cannulated ACL reamer is used to create a tunnel through the before and after opening wedge osteotomy with allograft (arrow) to
posterior tibial metaphysis after placing a guide wire in the physeal correct angular deformity. Note the radio-opaque markers in the epiph-
bar. (c) Fiber-optic lighted suction, dental mirrors, and fluoroscopy are ysis and metaphysis to help with postoperative assessment of subse-
used to confirm adequate resection of the bar. (d) Radio-opaque dye quent growth
to the exposed metaphyseal and epiphyseal bone. For central physis and the etiology and anatomy of the physeal growth
bars we prefer local fat interposition. Although cranioplast arrest and have an understanding of skeletal maturation.
has the purported advantage of completely and permanently Because of the complexities of the factors involved, the out-
filling the resection void, we believe that it has several disad- come of attempted bar resection can be unpredictable. As
vantages when further reconstructive procedures are such, the surgeon should educate patients and families of the
required. Namely that it is difficult to remove and leaves a importance of close clinical follow-up as well as the likeli-
bony deficit post-removal, often exactly at the level of defor- hood of needing subsequent procedures. Despite the unpre-
mity, significantly complicating subsequent procedures. dictable nature of physeal bar resection, it can result in
meaningful and gratifying resumption of growth in care-
fully selected patients.
Radiographic Markers
The surgeon’s final intraoperative task is to place small References

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Acute Deformity Correction Using
an Osteotomy 6
Vrisha Madhuri and Sangeet Gangadharan
In this chapter, we discuss the indications, principles, posteromedial bowing of the tibia, it is wise to defer surgery
specific considerations, choice of hardware and complica- till at least after 3–4 years of age, since spontaneous correction
tions of acute correction of lower limb deformities using an of the deformity is noted to a large extent, and the complica-
osteotomy. tion rate and chance of recurrence are higher if done at a
younger age 1. Soft tissue as well as bony correction of
deformities in cerebral palsy also tend to fail if performed
Principles of Acute Deformity Correction before 8–10 years of age due to worsening spasticity with
growth and persisting muscle imbalance [2].
General Considerations The magnitude of correction should take into account the
possibility of progressive deformity with future growth.
It is generally preferable to perform an acute correction when Physeal injuries and conditions such as Blount’s disease
the magnitude of angular correction required is small and the where only a portion of the growth plate is affected may
deformity is in a single plane. In children, the bone gap created require fusion of the healthy (lateral) portion of the open
by an opening wedge osteotomy heals well due to better physis or overcorrection of the deformity (Fig. 6.1). In some
healing potential and a thick, biologically active periosteum. situations, deformity correction may be combined with treat-
The skin overlying the affected area should be healthy as acute ment of the pathology such as excision of a physeal bar
correction can cause skin necrosis because of stretch or (Fig. 6.2) [3, 4].
impaired vascularity. Long-standing deformities are associated
with soft-tissue contractures on the concave side of the defor-
mity and this must be taken into account when planning. Principles Pertaining to Correction of Lower
Limb Alignment
Age Considerations Minor changes in lower limb alignment following corrective

osteotomy often remodel in children. Bone remodelling fol-
The outcome of treatment of certain deformities depends on lowing correction occurs in both sagittal and coronal planes,
the age at which the corrective osteotomy was performed. although such remodelling is greater in the plane of joint
This is due to correction or worsening of the deformity or the movement and when the osteotomy is close to the growing
underlying bony pathology with increasing age. In some end of the bone. This remodelling occurs at the site of oste-
conditions there is a high prevalence of recurrence when cor- otomy in accordance with Wolff’s law [5]. This law infers
rection is done at an earlier age. For instance, in congenital that remodelling will occur on the compression side by laying
new bone. Pauwels postulated that malalignment of bone pro-
duces a differential growth at the physis which tends to realign
V. Madhuri, D Orth, MS, Orth, MCh Orth (L pool) (*) the shaft perpendicular to the major joint reaction forces [6].
Paediatric orthopedics Unit, CMC Hospital, This observation is similar to the epiphyseal pressure rule
Christian Medical College, Vellore, POR Office,
Thottapalayam, Vellore, Tamil Nadu 632004, India
of Heuter-Volkmann (growth is retarded by increased
e-mail: [email protected] mechanical compression] [7]. Any residual deformity
S. Gangadharan, DNB Orth
would tend to decrease by remodelling in 4–5 years following
Paediatric orthopedics Unit, Christian Medical College, Vellore, surgery [8]. Seventy-five percent of this remodelling occurs
Vellore, Tamil Nadu 632004, India at the physis and 25 % by bone drift or translation [9, 10].

Fig. 6.1 (a) Radiograph of a
7-year-old girl with Blount’s
disease. Apex of the deformity is
seen at the intersection of the two
lines (red lines indicate the
proximal and distal tibial
mechanical axes). (b)
Overcorrection by Rab’s
osteotomy. (c) Radiograph at
3-year follow-up shows good
alignment of the mechanical axis
of the tibia
Fig. 6.2 (a) Right-sided genu

varum in a 7-year-old boy with
hemi-epiphyseal arrest of the
right distal femur (red lines
represent the mechanical axes of
both lower limbs). (b) CT scan
shows a peripheral physeal bar
(white arrow). (c) Bar resection
and cement interposition were
performed in addition to
corrective osteotomy of distal
femur. (d) CT scanogram
(supine) shows restoration of the
mechanical axis and proximal
migration of cement indicating
restoration of growth at 18-month
follow-up. A supine CT
scanogram was taken in this case;
however, a standing full-length
radiograph is a more accurate
measure of alignment and should
be preferred
6 Acute Deformity Correction Using an Osteotomy 81
Thus a perfect anatomical alignment may not be necessary; of fibula. A distal tibio-fibular fusion should be performed
however long-term follow-up is required in children with to avoid ankle instability when a fibular pseudarthrosis is
growth remaining. anticipated following such resections [21].
• At least 10 % of the total fibular length should be pre-
served distal to the osteotomy to avoid ankle instability
Influence of Level of Osteotomy [22]. Our experience shows that proximal fibular migra-
tion occurs if the fibula does not unite, irrespective of the
In children, often the osteotomies are at a site distant from level of osteotomy.
the apex or center of rotation of angulation (CORA). When
planning an osteotomy, one should consider various factors
such as the proximity of the CORA to the adjacent physis Principles Pertaining to Specific Osteotomies
and/or joint, presence of pathological bone and neurovascu-
lar structures in the vicinity. Overall, metaphyseal osteoto- Opening Wedge Osteotomy
mies have a better healing potential than diaphyseal ones. The axis of deformity correction lies on the convex cortex.
The ease of rotational correction is the same at both levels in The overall length of the limb increases following deformity
case of femur, but the proximal shaft is preferable as large correction. The final length achieved will be equal to the sum
corrections at the distal third would be theoretically at the of the lengths of the convex border of the deformity
expense of an undesirable alteration in the patella-femoral (Fig. 6.3a). If the combined total correction exceeds 2.5–3 cm
dynamics [11]. Any corrective osteotomy or lengthening of lengthening, shortening may be required as a cautionary
should not be performed at the site of pathological bone to measure since significant lengthening could compromise the
avoid healing problems [12, 13]. The exceptions are where neurovascular status. The nerve stretch in certain circum-
the entire bone is pathologic like in osteogenesis imperfecta stances may be further influenced by the direction of rotation
or fibrous dysplasia. such as the risk of iatrogenic injury being somewhat less in
external than internal rotation when performing a proximal
tibial osteotomy because of tethering of the peroneal nerve at
Adjuvant Fibular Osteotomy: When the proximal fibula [23]. When the axis of correction lies
and Where? away from the osteotomy site further lengthening is achieved;
for instance in calcaneal lengthening osteotomy the axis of
The fibula acts as a strut to prevent large angular or rotational correction lies away from the calcaneum, i.e. at the talar head
corrections of the tibia. Thus, with opening wedge correction [24] (Fig. 6.4).
of the tibia, an adjuvant fibular osteotomy is often performed
along with tibial osteotomy. Closing Wedge Osteotomy
• Fibular osteotomy should be avoided in its proximal third The axis of deformity correction lies on the concave side of
to safeguard the common peroneal nerve [14]. In the mid- the deformity. The overall length of the limb increases fol-
dle third there is a risk of damage to peroneal vessels lowing deformity correction and is equal to the sum of the
[15–17]. concave borders of the deformity as shown in Fig. 6.3b.
• Classic recommendation of doing a distal tibial de- Generally, a closed wedge osteotomy in a straight bone
rotation without a fibula osteotomy is for rotational cor- reduces the limb length whereas the bone length increases or
rections of up to 35° [18]. However, de-rotational remains the same with an open wedge osteotomy, for instance
osteotomies of up to 55° internal and 45° external have with compensatory osteotomies for the proximal femur in
been reported without fibular osteotomy and without neu- Perthes disease and non-union of the neck of femur
rovascular compromise [19]. We however prefer a fibular (Fig. 6.3c).
osteotomy while doing a rotational correction of greater
than 30°. Dome Osteotomy
• We tend to perform osteotomy at the proximal and middle Dome osteotomy is ideal for deformities with CORA close
third junction in case of proximal tibial angular osteot- to the joint line such as in physeal or metaphyseal injuries
omy and close to distal and middle third junction in distal near the knee or ankle. The displacement of the osteotomy
tibial/rotational osteotomies. is directly proportional to the radius of correction. Dome
• A segment of the fibula should be resected when the osteotomy causes minimal length alteration as the CORA is
desired angular correction in tibia is considerable [20]. usually at or near the site of deformity in the metaphysis.
Fibula osteotomy, if performed at a site far away from The advantage of such an osteotomy is good bony contact
CORA, leads to wide displacement of the fibula and in such and intrinsic stability. However this technique is limited by
situation may require resection of the projecting portion its inability to correct rotational malalignment.
82 V. Madhuri and S. Gangadharan
Fig. 6.3 (a) Open wedge osteotomy in a deformed bone of length “l” bone (ACA axis of correction of angulation). (c) In a straight bone an
showing increase in length of the bone. The final length “L” is the sum open wedge angular osteotomy causes less shortening than a closed
of convex borders of the deformed bone. (b) Closed wedge osteotomy wedge as shown by the red lines. Hence, the former is preferred when
in an angular deformed bone showing increase in length of the bone. minimal lengthening is desired along with corrective osteotomy
The final length “L” is the sum of concave borders of the deformed
Angular Correction and Translation

An angular osteotomy involves shifting of mechanical axis
in the direction of correction of the deformity; that is, the
mechanical axis shifts medially in varus and laterally in
valgus osteotomy. Hence translation of the distal fragment
has been advised in order to restore the mechanical axis [23].
A valgus proximal femoral osteotomy requires lateralisation
of the distal fragment. However when doing an angular cor-
rection below the knee the direction of translation is oppo-
site, i.e. lateral translation for varus and medial translation
for a valgus osteotomy. Glard et al. showed that proximal
femoral varus osteotomy with medial translation in Perthes
disease did not affect the mechanical axis deviation at the
knee [25]. There are a number of studies which show remod-
elling of the femoral neck-shaft angle following varus
osteotomy with or without translation [25–30]. In a child, do
we need to translate the distal fragment when performing
angular correction to optimise the mechanical axis? Sanghavi
et al. found that after varus de-rotation osteotomy, the neck-
shaft angle tends to revert to normal depending on the age at
which the operation was undertaken [26]. Tercier et al.
Fig. 6.4 (a) Axis of correction of angulation (ACA) is located away showed that following proximal femoral osteotomy in
from the calcaneum, i.e. at the talar head. The effective lengthening Perthes, the resulting mechanical axis deviation is usually
along with open wedge osteotomy can be seen. (b) Preoperative and (c) subtle and clinically insignificant in children [27]. Following
intraoperative radiographic views of left calcaneal lengthening osteotomy
and the increase in talar head coverage brought about by it
varus osteotomy of the proximal femur in cerebral palsy
Fig. 6.5 (a) Effect of angular

correction on length of a straight
bone. Red solid lines indicate
length of the straight bone.
Increase in length is seen
following opening wedge
osteotomy without translation.
Translation has relatively less
effect on the final limb length.
“S” is the amount of shortening
effected following translation.
ACA axis of correction of
angulation. (b) Top: Radiograph
of a child with Perthes disease of
the right hip. Bottom: A varus
de-rotation osteotomy was
performed. The medial intact
periosteal hinge is shown (white
arrow)
60 % of the correction is lost after 8 years by remodelling [28].

In pelvic support osteotomy it has been shown that up to 30°
of valgus angulation at the osteotomy site does not cause any
significant change in mechanical axis [31]. Therefore it is
our assumption that translation of the distal fragment during
angular correction of proximal femoral deformities is not
mandatory in younger children unless the magnitude of cor-
rection is outsized, such as greater than 30°. This observation
is also supported by the acceptability of overriding/bayonet-
ing fracture reduction in younger children where after a
few years complete restoration of the anatomy occurs [32].
The advantages of not translating are that the periosteal
hinge medial or lateral stays intact and the final lengthening
achieved is greater than with translation (Fig. 6.5).
Oblique Osteotomy
An oblique osteotomy corrects multi-planar deformities at
the same time. A meticulous preoperative planning is essen-
tial as it involves correction of the deformity in various
planes through a single osteotomy. Lesser the obliquity of
the plane, lesser is the bony contact after angular correction
but easier and greater is the rotational correction such as in Fig. 6.6 In this example the amount of desired external rotational (20°)
Rab’s osteotomy (Fig. 6.6). and valgus angular correction (40°) (indicated by dotted lines) is plotted
on the graph. θ, the angle of inclination of proximal tibial osteotomy
with the horizontal lies between 60 and 65°. Adapted from [74]
Lengthening/Shortening Osteotomy
Lengthening osteotomy in a single stage is best achieved
either by a step cut/long oblique osteotomy to maintain length ratio as compared to the companion bones in case of
adequate contact while lengthening at the same time such as osteotomies of leg and foot [34]. Acute shortening of up to
in distal fibula, metatarsals or proximal ulna. Acute length- 3 cm can be performed safely in young adults with open
ening of 1.7 cm and 3.2 cm for the tibia and femur, respec- tibial fractures with bone loss [35]. Further shortening should
tively, has been performed with minimal complications in be gauged based on the clinical assessment of limb vascular-
adults [33]. Opening wedge angular correction can some ity. Fibular shortening osteotomy can be combined with
time offset the shortening caused by varus angulation and proximal tibia corrective osteotomy in achondroplasia to
gain in length to some extent, for instance in Perthes disease correct the lateral collateral laxity and fibular overriding [36].
(see Fig. 6.4a). It is important to keep in mind the change in The major disadvantage of lengthening/shortening osteotomy
with acute correction is that the amount of correction safely • Acute corrective osteotomy combined with a growth modu-
gained is limited. If the magnitude of angular deformity lation technique: This would however invoke a shortening
correction is considerable, either a shortening osteotomy effect due to arrest of either side of the physis.
or distraction osteogenesis is preferred to avoid significant • An opening wedge osteotomy would simultaneously
risk to the neurovascular structures and soft-tissue tightness address the existing limb length discrepancy.
(Fig. 6.7).
Mango Slice Effect
In the Indian subcontinent, raw mango slice, a savoury sea-
Special Considerations sonal snack for children, is split into multiple radial cuts for
better seasoning. This makes a good illustration for osteot-
Deformity Secondary to Physeal Injury omy with multiple open wedges for diaphyseal deformity
Angular deformity secondary to physeal insult is usually as seen following rickets and other metabolic disorders.
progressive until skeletal maturity. The following strategies The concept and execution are explained in the following
can be adopted for such corrections: illustration. Small corrections at two or more CORAs allow
• Overcorrection of the deformity to compensate for future lengthening and deformity correction spread over the
progression of deformity. length of bone (Fig. 6.8).
• Combination of acute corrective osteotomy and excision
of physeal bar and interposition. The Deformity Memory Effect
Paediatric congenital and developmental conditions such as
developmental coxa vara, fibular hemimelia and posterome-
dial bowing of tibia have a memory because of associated
soft-tissue contractures. Following an osteotomy, either the
deformity can recur by bending the callus or slow relapse
occurs due to abnormal forces on the growing physis [Heuter-
Volkmann]. In these situations the osteotomies should be
combined with soft-tissue lengthening or shortening of the
bone (Fig. 6.9).
Cozen’s Phenomenon
Certain fractures of the proximal tibia in a growing child are
associated with a proximal tibial valgus deformity. These
deformities may correct spontaneously over a few years, and
Fig. 6.7 (a) Radiograph of a 10-year-old girl with osteogenesis imper-
fecta and bowing deformity of both femora. Restoring the length of the
thus an acute corrective surgery for the tibial valgus should
femur is limited by the stretch on neurovascular structures and soft tis- be deferred for at least 3–4 years following the fracture
sues. (b) Hence, corrective osteotomies are combined with shortening [37, 38] (Fig. 6.10).
Fig. 6.8 (a) An intact mango

slice and (b) a slit, seasoned one
can be compared with multiple
opening wedge osteotomies in a
bowed tibia
Box 6.1
• Acute deformity is preferred in children and when
the magnitude of deformity or length discrepancy is
relatively small.
• Soft-tissue constraints should be addressed simulta-
neously where needed, to avoid recurrence.
• Knowledge of the natural history of the underlying
pathology is crucial before executing an acute
osteotomy.
• Anatomical alignment of mechanical axis is not
mandatory in younger children because of remodel-
ling potential.
• A dome osteotomy can be performed when the
CORA is juxta-articular.
• Fibular osteotomy may be necessary for tibial oste-
otomies for large correction.
Fig. 6.10 Cozen’s phenomenon. (a) Three-year-old boy with proximal

tibia fracture. (b) Radiograph after 4 years shows tibial valgus deformity
Indications
The indications for acute correction with an osteotomy in

children are varied and include correction of certain unipla-
nar or multi-planar skeletal deformities. The indications
include angular and rotational correction as well as treatment
of mild length discrepancies.
Site-Specific Osteotomies
Metaphysis
Frontal plane angular deformities around the knee [genu
varum or genu valgum] related to conditions such as Blount’s
disease, metabolic bone disease, physeal arrests and coxa
vara are often treated with acute correction via a metaphy-
seal osteotomy (Fig. 6.11).
Epiphyseal
Osteotomies may also be performed at epiphyseal level to
accomplish joint elevation such as medial hemi-plateau ele-
vation for Blount’s disease [39–41].
Fig. 6.9 The deformity memory effect. (a) Developmental coxa vara in Juxta-Apophyseal
a 15-year-old boy. (b) Corrective osteotomy and blade plate fixation.
Re-directional osteotomies of greater trochanter, calcaneal
(c) Radiograph at 16-month post-op follow-up shows reduction of
neck-shaft angle (red angles) in spite of the implant position remaining and tibial tuberosity improve muscle function by optimising
unchanged the lever arm dysfunction [42–45] (Fig. 6.12).
Fig. 6.11 CT scanograms

(supine) comparing (a) pre-op
and (b) post-op mechanical axis
alignment (dotted red lines) in a
14-year-old girl with bilateral
genu valgus. She underwent
bilateral varus osteotomies of
distal femora and fixation with
locked compression plates.
Supine CT scanogram was taken
in this case. However, a standing
full-length radiograph is a more
accurate measure of alignment
and should be preferred
Diaphysis bowing in the femur and tibia. The associated diaphyseal

The common indications for diaphyseal osteotomies include deformities are typically a generalised bow and multi-apical
metabolic bone disorders such as rickets, osteogenesis imper- in nature, requiring multilevel osteotomies for correction.
fecta, renal osteodystrophy and deformities secondary to trau- Severe forms of osteogenesis imperfecta often cause gro-
matic or pathological fractures due to osteomyelitis and cystic tesque diaphyseal deformities. The poor quality of underly-
lesions of bone. Posteromedial bowing of tibia and congenital ing bone warrants an intramedullary device to splint the bone
limb deficiency form a special subset of diaphyseal osteoto- after correction (see Fig. 6.7).
mies due to their propensity for complications due to associ-
ated soft-tissue inelasticity requiring special measures to avoid
complications, for instance, postponing the deformity correc- Certain Considerations Based on Location
tion till the child with posteromedial tibial bowing is older and of the Deformity
using a locked plate to prevent fractures or bowing of the regen-
erate that may cause recurrence of the deformity. Congenital Proximal Femur
pseudarthrosis of tibia is a rare disorder where the primary aim In order to avoid damage to the capital physis, most of the
of treatment is to achieve union along with deformity correc- proximal femoral osteotomies in children are carried out dis-
tion and limb lengthening (Fig. 6.13). tant from the site (apex) of deformity. Such acute correction
Metabolic bone disorders often exaggerate the natural reorients the femoral head in valgus or varus, extension or
curves of shafts of long bones, often leading to anterolateral flexion and rotation and is also used for shortening the
Fig. 6.12 (a) A 10-year-old girl with skeletal dysplasia with shortened Fig. 6.14 (a) Radiograph of a 6-year-old girl with bilateral dysplastic
abductor lever arm and abnormal articulo-trochanteric distance bilaterally. hips. (b) Open reduction was performed in conjunction with femoral
(b) Distal and lateral transfer of right greater trochanter was performed. shortening osteotomy to avoid avascular necrosis and de-rotation to
The increased length of abductor lever arm following trochanteric transfer correct anteversion and reduce the hip
improves abductor power and gait and eliminates trochanteric impinge-
ment. a, restored articulo-trochanteric distance
femoral segment in certain cases of long-standing dislocated

dysplastic hips.
Most of these osteotomies cannot be performed at the
CORA due to the presence of pathological bone at the site of
deformity as in proximal focal femoral deficiency or because
of proximity to the proximal femoral physis and the tenuous
blood supply to the femoral head [12, 13]. The common
example is Perthes disease where proximal femoral varus or
valgus osteotomies realign the articulation to either increase
femoral head coverage or avoid impingement by the
deformed head (Fig. 6.14). A combination of angulation,
shortening and rotation is sometimes necessary to achieve
optimal reorientation, for instance a proximal femoral oste-
otomy in a child with a congenital and neurogenic hip dislo-
cation. In developmental coxa vara realignment of the physis
to a more horizontal position prevents relapse.
Acute osteotomy of the intertrochanteric and sub-
trochanteric regions of femur orients the proximal femoral
physis more horizontally, thus converting shear forces to
Fig. 6.13 (a) Anteroposterior and lateral views showing congenital
compressive forces.
pseudarthrosis of tibia in a 6-year-old boy. (b) Six years following cor-
rective shortening osteotomy, resection of hamartomatous periosteum, Sub-capital realignment osteotomies are an example
bone grafting and revision intramedullary rodding of correction at the level of deformity (CORA) in slipped
Fig. 6.16 (a) Bilateral genu valgus in a skeletally mature girl.

(b) Bilateral closed wedge osteotomies and metaphyseal plate fixation
were performed. Closing rather than open wedge osteotomies were per-
formed to avoid common peroneal nerve injury. In femoral valgus
deformity corrections greater than 30° we prefer to decompress the nerve
acute corrective osteotomy. The correction of deformity

around the knee requires due consideration to neurological and
vascular structures due to their proximity to the osteotomy
site (Fig. 6.16).
Fig. 6.15 (a) Acute on chronic SCFE left hip in a 12-year-old girl.
(b, c) A modified Dunn’s osteotomy was done to realign the head and
avoid femoro-acetabular impingement Acute Correction of Length Discrepancy
Correction of minor shortening can be obtained by acute
lengthening. Brachymetatarsia correction by acute length-
capital femoral epiphysis but are rarely done because of the ening of up to 15 mm can be performed safely without any
risk of vascular compromise to the femoral head blood neurovascular compromise [34]. Trans-iliac osteotomy is a
supply (Fig. 6.15). rarely done procedure which achieves lengthening when
angular correction is not sought. In relative femoral neck
Osteotomies Around the Knee lengthening, displacement osteotomy of the trochanter is
Flexion deformity of the knee associated with intractable combined with reshaping of the head, neck and trochanter
joint contractures and muscle imbalance such as myelodys- to eliminate femoro-acetabular impingement and lengthen
plasia, arthrogryposis, cerebral palsy and poliomyelitis can the lever arm (Fig. 6.17). Acute lengthening of the distal
be corrected by supracondylar extension osteotomy of the fibula by a step-cut or long oblique osteotomy is another
femur. Such osteotomies reorient the arc of knee motion example [43].
allowing full apparent extension and improve the child’s
gait and function. A distal femur-shortening osteotomy can Reorientation Osteotomy of Acetabulum
counter the long-standing flexion contracture without sig- Ganz, triple, Dega’s and Salter’s osteotomy in dysplastic
nificantly affecting the hip extensor power. Correction of acetabulum augments femoral head coverage and redistrib-
coronal plane angular deformities by closed or open wedge utes hip joint force [45, 46].
is the commonest indication around the knee. While this is
now being rapidly replaced by the growth modulation tech-
nique, correction via an osteotomy has a role when the Osteotomy with Adjuvant Procedures
deformity is severe and growth potential is limited or where
rapid correction is desirable. Lower limb deformities in Sometimes acute correction of the deformity can be combined
children with certain skeletal dysplasias where growth mod- with another procedure. This can be unifocal such as physeal
ulation techniques play a limited role can also be treated by bar excision with corrective osteotomy in hemi-physeal

6-year-old boy with Choi’s type
IVa [44] post-septic sequel left
hip. (b) A neck-lengthening
osteotomy with distal transfer of
the greater trochanter shows
optimisation of the hip abductor
lever arm function. Red- and
yellow-dotted lines indicate
abductor lever arm and moment
arm, respectively. (c) Immediate
post-operative radiograph
arrests around the knee (see Fig. 6.2). Alternatively it could Box 6.2
be bifocal, where it is not optimal to do the angular correction • Apophyseal reorientation osteotomies (calcaneal,
at the same site as that of lengthening, for instance pelvic greater trochanter and tibial tuberosity) lengthen
support osteotomy with distal femoral lengthening [47, 48]. the lever arm for optimal joint function.
Guided growth procedures can also be performed along with • Metabolic bone disorders usually cause multi-
acute osteotomy, e.g. lateral proximal tibial guided growth apical deformities which often require acute
along with ipsilateral Rab’s osteotomy for a child with multilevel corrective osteotomies.
Blount’s disease [49]. To counter the muscle imbalance • Acute shortening osteotomy may be performed
brought about by cerebral palsy or meningomyelocele and primarily as in proximal femur to avoid avascular
other neuromuscular disorders, corrective osteotomy is com- necrosis or in conjunction with deformity correc-
bined with a muscle-balancing procedure, thereby avoiding tion to avoid stretch on neurovascular structures.
relapse. For example, patellar tendon reefing is done follow- • Various realignment osteotomies of the proximal
ing supracondylar extension osteotomy for fixed flexion femur are described to increase joint coverage,
deformity of the knee in cerebral palsy, and tibialis anterior reduce impingement, realign capital physis and
transfer to the heel is combined with a calcaneal osteotomy increase the arc of motion.
in a child with excessive ankle dorsiflexion and weak
plantarflexors.
Disease-Specific Indications Planning
In certain conditions like osteogenesis imperfecta, when the Rules Governing Osteotomy Planning
child is non-ambulatory, the indication for a corrective oste-
otomy is to aid seating on a wheelchair, better hygiene and Growth considerations have to be kept in mind when plan-
prevent further deformities rather than aligning mechanical ning deformity correction. Hence osteotomy planning in chil-
axis. A long-standing dislocated or destroyed painful hip dren is somewhat different from that of adults where accurate
in a child can be managed by a pelvic support osteotomy line diagrams and metaphyseal angles are drawn with a view
combined with excision of the femoral head. to achieve precise alignment of the knee mechanical axis.
While restoring the mechanical axis is important in children, are drawn (yellow-dotted lines) as shown in Fig. 6.18b. Red
the deformity correction involves aligning the proximal and lines form the anticipated mechanical alignment angles of
distal physes parallel to each other and perpendicular to the the tibia with the yellow-dotted lines tangential to the knee
ground rather than merely achieving exact anatomical align- joint and talar dome. We consider the talar dome as the refer-
ment. However in older children nearing skeletal maturity, ence line for distal mechanical axis since the epiphysis and
the principles of restoring the mechanical axis used for adults physis of the distal tibia are often misshapen in angular
are more applicable. While there are several methods for pre- deformity in growing children. The mechanical axes inter-
operative planning, we will illustrate our preferred planning sect each other at the CORA and the angle [α] thus subtended
methods for acute correction of the tibial and femoral defor- is the magnitude of correction of the deformity (Fig. 6.18b).
mities in children. When the CORA is located in the bone near the obvious apex
of deformity (area where the deformity visually appears to
be maximum), correction can be obtained by a single oste-
Planning for Tibial Deformity Correction otomy of magnitude “α” at this level.
Step 1: Finding the CORA Step 2: Finding the Axis of Correction

The aim of surgical correction via an osteotomy is to achieve for Two CORAs
near-normal mechanical alignment and joint orientation of In bowing deformities, the CORA could be located away
the lower extremity. After obtaining standing radiographs in from the obvious apex of deformity, suggesting the presence
the orthogonal planes, the joint lines at the knee and ankle of two or more CORAs [23]. In such situations, two points
Fig. 6.18 Steps in planning correction of tibial deformity. (a) Bowing convex or concave tibial border based on execution of an open or closed
deformity of the tibia due to metabolic bone disease. (b) Yellow-dotted wedge osteotomy, respectively. (d) Lines (yellow solid) are drawn parallel
lines drawn tangential to the tibial plateau and talar dome. Mechanical to the proximal and distal mechanical axes through these points. (e, f)
axes [red dotted] of proximal and distal tibia are drawn from the centre of The two points are connected by a line (red dotted), thus forming a trian-
the former landmarks. The point of intersection of these lines is the CORA gle with α1, α2, and β angles. An opening wedge osteotomy of “α1” mag-
of the deformity and the angle “α” subtended is the magnitude of correc- nitude proximally and “α2” distally is planned. (g) Final radiographs after
tion. (c) Two suitable sites for osteotomy “A” and “B” are plotted on the executing the osteotomies showing the corrected mechanical axis
(A and B) are plotted at the desired osteotomy sites on the Planning for Femoral Deformity Correction
cortex of the bone (Fig. 6.18c). These points form the centre Other than Coxa Vara
of the arc of correction of the deformity. Lines (yellow) par-
allel to the mechanical axis of the individual fragments [red] The mechanical and anatomical axes for the femur are differ-
are drawn through the proximal and distal points, respec- ent. There are no paediatric standards for proximal lateral
tively, as shown (Fig. 6.18d). These points are now con- femoral angle and the greater trochanter may be incom-
nected by a single line (dotted red) (Fig. 6.18e, f). The sum pletely ossified or unossified in young children. This requires
of the two acute angles, α1 and α2, subtended in the triangle an alternative planning for femoral deformity correction.
formed is equal to the total magnitude of correction “α”.
Explanation: Step 1: Plotting Mechanical Axis for Distal Femur
The obtuse angle “β” in the triangle = 180° − [α1 + α2] Standing orthogonal films are obtained in both anteroposte-
(sum of all angles in a triangle = 180°) rior and lateral view. The mechanical lateral distal femoral
Also, β = 180° − α (angles on a straight line add to 180°) angle (mLDFA = 88°) is drawn (see Chap. 2). This gives the
Hence, α = α1 + α2 distal mechanical axis [red line] as shown in Fig. 6.19b.
The equation proves that the magnitude of multiple wedge
osteotomies at the two desired points should sum up to the Step 2: Plotting Proximal Femoral
total magnitude of deformity correction. Mechanical Axis
The osteotomies can be thus planned at the marked points. As there are no standards for mLPFA for different age groups
It is prudent to confirm the mechanical axis alignment intra- in children we consider plotting the mechanical axis as
operatively with the Bovie cord method [50]. The cord is follows. The mid-diaphyseal line is drawn as shown in
stretched between the centre of the hip and talar dome fol- Fig. 6.19c. A line parallel to this is drawn from the centre of
lowing correction. The correction is then fixed using appro- capital femoral ossific nucleus [black dotted]. The proximal
priate fixation device. If desired, the CORAs could be femoral mechanical axis is a line angled 7° lateral to this line
identified on the concave border and closing wedge osteoto- (Fig. 6.19d). The point of intersection of these lines is the
mies carried out. CORA and the angle subtended gives the magnitude of cor-
Contrary to the popular practice, we do not draw the rection. The axis of correction should be located in the axial
mid-diaphyseal line as this would produce two fixed apices plane of the CORA depending upon whether an open, closed
at sites which may be unacceptable to undergo osteotomy. or neutral wedge osteotomy is desired. In case of bowing
The advantage of the described technique is that the oste- deformity with two or more CORAs step 2 shown in
otomy sites can be chosen by the surgeon in consideration Fig. 6.18e,f for tibial correction can be carried out. In case of
to adjacent structures like neurovascular bundle, physis, coxa vara, the principle of deformity correction lies in
pathological bone or unhealthy skin. aligning the capital femoral physis as horizontal as possible.
Fig. 6.19 (a) A femoral bowing deformity in a 4-year-old boy with unos- anatomical axis from the centre of capital femoral ossific nucleus.
sified greater trochanteric apophysis. (b) mLDFA and mid-diaphyseal line (d) The mechanical axis (red solid line) at the proximal femur is drawn
for the proximal femur are plotted (red solid line, mechanical axis; black from the same point at 7° to the black-dotted line. Intersection of the two
solid line, anatomical axis). (c) Black-dotted line is drawn parallel to the axes is the CORA and “α” is the magnitude of deformity correction
Fig. 6.20 (a) Illustration

showing mechanical axis
deviation (MAD) in a limb with
tibial and femoral deformities.
(b) Distal femoral corrective
osteotomy without addressing the
tibia deformity. Cross-checking
MAD with Bovie cord between
hip and talar dome as reference
points is not possible as tibial
axis is abnormal. (c) Cross-
checking correction of tibial
deformity is possible with Bovie
cord using the centres of tibial
plateau and talar dome as
reference points. (d) The
corrected tibial mechanical axis
allows the mechanical axis of the
limb to pass through the centre of
the knee joint when the femoral
head and the talar dome are the
reference points
In all other cases we wait for the neck-shaft angle to remodel If the femoral deformity is corrected first, the cord has only
once the mechanical axis is restored. In adolescents where one reference point, i.e. the centre of femoral head, the distal
the trochanter is ossified, mLPFA and mLDFA are plotted as reference point being outside the corrected mechanical axis,
described in Chap. 2. i.e. the centre of talar dome. On the other hand, the tibial
deformity can be corrected without using limb mechanical
axis for reference as the anatomical and mechanical axes are
Planning for Multiple Bony Deformities the same in case of tibia. Hence when ipsilateral femoral and
in the Same Limb tibial deformities are being addressed with acute osteotomy
correction, either at the same sitting or in a staged fashion, we
Children with deformities secondary to metabolic disorders recommend performing the tibial correction first (Fig. 6.21).
or osteogenesis imperfecta often present with multiple bony
deformities in the same limb. When planning such sequential
corrections, single or multi-staged, we perform the tibial cor-
rection first which has essentially the same mechanical axis Box 6.3
and anatomical axis. For checking the femoral alignment, if • Judicious preoperative planning with standing
the tibial alignment is normal, the Bovie cord is stretched radiographs or CT scanogram is essential before
between hip and ankle. Following correction of the femoral executing acute corrective osteotomy.
deformity, the cord should pass through the centre of knee if • Consider talar dome as a reference line for drawing
the mechanical axis of the lower extremity is restored. the mechanical axis of distal tibia as the distal tibial
However, when the tibia is also deformed, in spite of restor- epiphysis is often misshapen in deformities of the
ing the femoral mechanical axis, the cord may lie on the tibia.
medial or lateral side of the knee if the tibia has residual • Intraoperative confirmation of mechanical axis
varus or valgus deformity, respectively (Fig. 6.20b). There is alignment with a Bovie cord is prudent for accurate
no such problem when checking the tibia as the Bovie cord correction of deformities.
can be stretched between the centre of the knee and ankle, • When dealing with concomitant deformities of ipsi-
and if it lies along the axis of tibia, the tibial deformity cor- lateral tibia and femur, it is wise to correct the tibial
rection is confirmed independent of the hip reference point deformity first to avoid malalignment.
(Fig. 6.20c).
Fig. 6.21 (a) Preoperative

standing radiograph of a child
with spondylometaphyseal
dysplasia. He underwent serial
corrections of lower limb
deformities. (b, c) The tibial
deformity was addressed initially.
(d) This was followed by
correction of femoral deformity
Hardware Considerations Screws
Casts are often used following acute deformity correction in Screws are useful for small fragments and it is possible to do
young children. They are often used to maintain deformity post-operative adjustment in one plane only when a single
correction after no fixation or minimal fixation with screws screw is used, e.g. in Rab’s osteotomy (see Fig. 6.1) [53].
or wires [50]. The casts need to be well moulded to avoid They are also used for fixation in trochanteric (see Fig. 6.12)
loss of correction in cast. In the operating room, correction and tibial tuberosity displacements. They can cross the
of the deformity in the cast is checked by the cautery cord growth plate near or after physeal closure, as in osteotomy
method. A well-done cast wedging allows for fine-tuning of involving mal-united condyles and malleoli.
deformity correction (see Fig. 6.22). However, casts have to
be used with caution in acute correction as post-operative
swelling may lead to compartment syndrome. Dynamic Compression Plate
A contoured dynamic compression plate (DCP) in children

Kirschner Wires can hold the angular correction required for small deformi-
ties. With such internal fixation devices a predetermined cor-
Smooth Kirschner (K) wires are safe and easy to use espe- rection can be achieved by translating the bone fragments to
cially in infants and toddlers where plaster casts are often the plate. Rotational correction can be achieved by inserting
needed. The smooth wires are less traumatic to the physis and K wires on either side of the planned osteotomy at the desired
allow for fine-tuning of the correction in the post-operative angle and make them parallel at the time of fixation. This
period by wedging of the casts. They are also used when cor- technique is easy for the beginners as the proximal fragment
rective osteotomies are combined with other procedures such can be predrilled and tapped prior to osteotomy and is under
as physeal surgeries in the vicinity and where the anticipated control during osteotomy and fixation [54].
healing times are short and the forces across the osteotomy
site are not excessive. Examples of K wire use are Bowen’s
technique for tibia vara correction, supramalleolar osteotomy, Tension Band Wiring
calcaneal lengthening osteotomy, Salter’s innominate osteot-
omy and metatarsal osteotomies for hallux valgus correction Even large fragments such as proximal femur fragments dur-
[18, 51, 52]. ing coxa vara correction can be fixed in children with tension
Blade Plate
Specific osteotomy plates such as condylar blade plates with

preset angles can be used for proximal tibial as well as proxi-
mal and distal femoral metaphyseal corrections (see
Fig. 6.22). Minor adjustment in angular correction is possi-
ble by varying the angle of insertions/entry points in condy-
lar plates. Rotational correction can also be achieved by
directing the blade in appropriate anteversion. However,
these devices are less forgiving and require a thorough
understanding of the local anatomy and the constraints of the
implant design.
Locked Plates (LCP)
These are ideal for holding correction in osteoporotic bones

and in multiple osteotomies within a single bone. They per-
mit early weight bearing and promote improved bone heal-
ing due to reduced plate bone contact. Metaphyseal locked
plate allows better hold in a short fragment near the joint (see
Fig. 6.22). Paediatric varus and valgus hip LCP and distal
femoral LCP for osteotomies around hip and lower femur
allow multiple locked screw fixations, thus achieving not
only rigid fixation but also avoiding early or late joint pene-
tration as can be seen with some of the more conventional
plating systems.
Intramedullary Devices (Such as Rush Rods

and Fassier-Duval Telescoping Rods)
These devices are preferred when the bone quality is poor

and cannot be corrected completely by medical management
such as in osteogenesis imperfecta, metabolic disease and
resistant forms of rickets and renal osteodystrophy. The rate
of reported complications with telescoping rods has been
Fig. 6.22 Shows use of various implants at different sites. (a) Distal high [56, 57]. Rush rods can also be used for corrective val-
transfer of the greater trochanter fixed with tension band wiring. gus osteotomy in the proximal femur in proximal femoral
(b) Use of Kirschner wires and plaster cast for fixation of supramalleo- focal deficiency (PFFD). Overlapping rush rods can also be
lar corrective osteotomy. (c) Lateral closed wedge osteotomy of distal
femur and fixation with blade plate for genu varus deformity. (d) Use of
used to accommodate future growth in metabolic bone disor-
metaphyseal plate for juxta-articular fixation following medial closed ders and osteogenesis imperfecta (Fig. 6.23).
wedge osteotomy for correction of genu valgus deformity
External Fixation and Limb Reconstruction

band wiring (Fig. 6.22). It is a useful technique in closing Systems
wedge osteotomy. Another example is distal transfer of
greater trochanter [55]. There is lesser degree of physeal Acute correction with external fixation offers potential
damage when correction is desired in younger children advantages of allowing dynamic compression, distraction
wherein screws or plates cannot be used. following acute correction, permitting minor realignments
Fig. 6.23 (a) An 8-year-old boy with osteogenesis imperfecta pre-

sented a month after a trivial fall. Radiograph shows healing fractures
of both femora and broken Rush rod in right femur. (b) Bilateral double
Rush rodding was done to accommodate future growth of bone and
fracture beyond the bone was protected by the implant
post-operatively and having a lower incidence of peroneal

nerve palsy and compartment syndrome following a proxi-
mal tibial osteotomy in obese or adolescent patients [58].
The stability offered by circular external fixators often allows
patients to weight bear as tolerated. The fixator can span the Fig. 6.24 (a) Use of hydroxyapatite-tricalcium phosphate-calcium
physis till healing of the osteotomy and then be removed, silicate (HASi) scaffold as a wedge to maintain correction in calcaneal
thereby not significantly affecting growth. lengthening osteotomy. (b) Non-union and resorption of neck of right
femur following fracture in an 11-year-old girl treated by neck recon-
struction by autograft (arrow) and valgus subtrochanteric osteotomy.
Follow-up radiograph shows evidence of union
Bone Void Fillers
Autografts, allografts and varied combinations of commer- Adapted Fixation Techniques

cially available synthetic bone substitutes can be used as
fillers or scaffolds following an opening wedge osteotomy Fixator-Assisted Plating
as promoters of bone healing. Such fillers may also provide Maintaining accurate alignment can be difficult when using
some structural support in maintaining alignment following plate fixation following acute deformity correction. The
an acute correction. Ceramic-based bone graft substitutes fixator-assisted plating (FAP) technique, as described by
include hydroxyapatite, calcium phosphate, tricalcium Bar-On et al., uses a temporary external fixator to acutely cor-
phosphate or bioglass. Hydroxyapatite-based scaffolds are rect the deformity and stabilise it before application of the
the most preferred substitutes for bone reconstruction as plate [63, 64]. Fixator-assisted manipulation of osteotomy
natural bone has similar stoichiometry [59]. However, with fragments using either a tubular, rail or ring fixator allows easy
the exception of autografts, these substitutes do not have an placement of LCP or DCP by minimally invasive technique,
osteogenic potential. Synthetic bone graft substitutes are thus maintaining the biology and also decreasing the need for
often brittle leading to loss of correction and cannot be assistance during surgery. Indications for this technique
used to fill large defects in long bones [60]. Resorption of include angular or rotational correction of metaphyseal or
hydroxyapatite-based ceramics is faster in young children diaphyseal, multi-planar and multi-apical deformities.
[61]. In a recent study Balakumar et al. showed that
hydroxyapatite tricalcium phosphate calcium silicate scaf- Technique
fold (HASi) incorporates faster and can be used safely for The technique described by Bar-On et al. involves use of a
metaphyseal defects in children and adolescents [62] monoplanar external fixator and locked plates [63].
(Fig. 6.24). Metaphyseal plates are used for juxta-articular deformities
and straight narrow plates for diaphyseal deformities. Fixator-assisted deformity correction offers fine control
External fixator pins are introduced beyond the proximal and of alignment, eases correction and requires minimal assis-
distal ends of the selected locked plate. When this is not pos- tance during application of internal fixation. The main
sible, the half-pins can be closer together; however, their advantage over conventional techniques of correction is
placement should avoid the anticipated site of plate fixation. that there is less risk of losing correction. We often use a
The osteotomy is placed as close to the CORA as possible. similar technique when plating at the end of gradual
Bar-On et al. recommend two locked screws per segment. lengthening with a fixator to shorten time in external
Rozbruch described a similar technique in adults employing fixation.
a designated deformity correction frame such as Taylor spatial The technique, however, is difficult to use in smaller
frame or multiaxial correction frame [64]. The fixator children. A difficult exposure or plate introduction due to
placement must not impede subsequent internal fixation. presence of fixator can be avoided by judicious preopera-
The deformity is then incrementally corrected intraoperative planning and outlining plate position with a skin
tively with this fixator. marker [63]. Another problem is that a large pin tract may
We use a slight modification of the above techniques by act as a stress riser. This could be prevented by extending
placing the proximal and distal pins at right angles to the the plate beyond the fixator or providing cast protection for
mechanical axis and fixed to the clamps of a monolateral fix- a short period. The technique is also unsuitable for correc-
ator or a two-ring circular fixator. For tibial deformities, one or tions of large magnitude involving bone shortening as
two half-pins are passed parallel to the proximal and distal actual contact between the two fragments is lost. We have
joint lines, each under image intensifier in the plane of maxi- had no pin tack infection thus far when doing locked plate
mum deformity and preferably on the concave side of the following acute or gradual deformity correction using
deformity as distraction holds the alignment better. For the external fixation but there is a theoretical risk of deep
femur, the distal pin is inserted parallel to the joint line and infection.
proximal pin is inserted in the proximal shaft angled 7° caudad
to a perpendicular (valgus with respect to the anatomical axis Supracutaneous Locked Plating
in the anterolateral plane) to the femoral shaft (Fig. 6.25a). Supracutaneous plate is an attractive option for acute defor-
When rotational correction is required, the pins are aligned at mity correction or shortening in the presence of poor skin
the desired magnitude of rotational alignment in each frag- condition or infection [65–67]. The indications are similar
ment. In a large epiphysis like that of the femur, the pins can to those for using external fixation except that it allows
be passed through the distal femoral epiphysis if necessary. A fixation of small metaphyseal fragment when a metaphy-
two-ring Ilizarov frame with two wires in each fragment may seal locked plate is used. The additional advantages are its
be used in corrections close to the ankle (Fig. 6.26). lightweight, low profile and easy concealment under regu-
A hinged monolateral or ring fixator permits fine-tuning of lar clothing especially in a small child. We extensively use
correction before plate insertion. Acute correction by osteot- this technique for open fractures as well. Supracutaneous
omy is carried out at or near the CORA and alignment is con- locked plate can serve as a lightweight external fixator in
firmed in both planes. In case of multilevel deformity the presence of or prior to infection. The usual indications
correction or femoral corrective osteotomy, we use the Bovie are deformity correction secondary to osteomyelitis sequel,
cord technique to confirm final alignment. The locked plate is physeal arrest or pathological fractures. The technique
introduced by making longitudinal incisions proximally, dis- involves initial deformity correction at the non-union/
tally or through the osteotomy site [63]. One end is fixed with mal-union site. In the presence of a gap non-union shorten-
a K wire to achieve plate alignment in close proximity to the ing is affected and the fragments are held with a conven-
shaft of the bone following correction and fixed with distal tional or fixator-assisted technique (Fig. 6.27). One
and proximal locking screws. At this stage, there is the oppor- centimetre thick folded linen is inserted between the plate
tunity for minor translation of the underlying bone fragment and skin to aid screw length measurement. A supracutane-
by adjusting the screw lengths or use of a non-locking cortical ous locked plate holds this position with a minimum of four
screw in the distal or proximal fragment of the plate to achieve cortices (two locked screws) on either side. Following this
desired displacement of the fragment. Prominence of plate the fixator is removed. We do not hesitate to put one screw
may be avoided by pre-contouring the plate. Pre-contouring in the epiphysis when the fragment is very small as the
the plate with locked screws engaged in the plate is recom- plate is removed in a matter of few weeks to months and the
mended to avoid distortion of the screw holes. Alternatively a effect on growth is negligible. Pin care protocols are fol-
contoured DCP can also be used. Post-operatively toe touch lowed and removal is done when all four cortices at the
ambulation is usually permitted. osteotomy site are noted on radiographs.
Fig. 6.25 The saw bone model illustrates the technique of deformity fragment. (d) Osteotomy is carried out at or near the CORA and
correction with limb reconstruction system (LRS) and subsequent deformity corrected using the monolateral fixator. (e) A locked com-
plating. (a) Distal pin is placed freehand parallel to the knee joint line pression plate is aligned parallel to the shaft anterior to the fixator
(dotted line). (b) Second pin is placed using the LRS pin template. and temporarily stabilised with a K wire proximally and locked
(c) Second set of screws are angled 7° caudad to a perpendicular to screw distally. (f) Remaining two screws proximally and one distally
the underlying diaphysis (red-dotted line). This restores the mechanical are inserted through the locking holes and K wire removed. (g) LRS
axis of femur which lies at 7° to the anatomical axis of the proximal removed
Box 6.4. Fixation Techniques and Implants • An external fixator following corrective osteotomy
• Internal fixation with K wire and/or plaster cast offers added advantage of compression, distraction
allows minor post-operative realignment during and minor post-operative realignments.
moulding or by cast wedging. • Bone void fillers provide structural support and act
• Metaphyseal locking plates offer better hold of as promoter of bone healing as well.
short metaphyseal fragments following corrective • Fixator-assisted plating technique allows deformity
osteotomy. correction and stabilisation of fragments until plate
• Deformity correction in poor bone quality can be fixation is done.
stabilised with intramedullary devices which permit • A locked compression plate can be used as a low-
growth and provide internal splinting. profile and lightweight external fixator following
acute osteotomy.
Fig. 6.26 (a) Photographs show

use of Ilizarov frame in the
fixator-assisted technique for
reduction and maintaining
alignment of fracture fragments
in a 17-year-old boy with an open
fracture tibia. (b) Intraoperative
radiographs show attainment of
alignment and the connecting
Ilizarov rods, which are seen
overlying the skin
Complications the limb, especially if neurovascular bundle lies on the

concave side of the deformity. This iatrogenic risk is less
The complications in acute deformity correction include with a closing wedge than an opening wedge osteotomy.
complications of osteotomies, fixation failure and lengthening The degree of nerve stretch also depends on the mobility of
associated with acute deformity correction. The risk of com- the neurovascular bundle in the surrounding soft tissues and
plications may vary with age, the site of deformity, underlying is related to the magnitude of angular correction and its dis-
pathology, proximity to physis and adjacent neurovascular tance from the centre of correction (Fig. 6.28). The neuro-
structures. There is no consensus in literature on the amount of logic risk could further increase when rotational correction is
acute deformity correction and frequency of complications. attempted; for instance, acute internal rotational correction
Angular deformity correction has been noted to have more in the proximal tibia can often lead to common peroneal
complications than rotational corrections [68]. nerve palsy. Another cause of peroneal nerve palsy is a
fibular osteotomy, especially in the proximal third. Ideal
position for fibular osteotomy is closer to the lower and
Acute Complications middle third junction as the peroneal nerve is relatively less
mobile in the upper third [15]. Judicious use of intraopera-
Neurological tive SSEP monitoring and prophylactic nerve decompression
The rate of neurological complications following acute in certain high-risk instances could minimise the risk of
deformity correction is varied. There is evidence that acute iatrogenic nerve injuries [68].
correction of a proximal tibial varus deformity is associated
with a greater risk of peroneal nerve palsy than with gradual Compartment Syndrome
correction [69]. Some studies have shown no complications Concomitant acute lengthening with deformity correction,
whereas others have reported a variable rate of such injuries pressure on vessels or soft-tissue oedema following surgery
ranging from 5 to 20 % [68]. The neurological deficit could can lead to a compartment syndrome. When performing a
be due to direct intraoperative injury, ischemia, acute stretch proximal tibial osteotomy with acute correction, it is advis-
or fibular osteotomy [68]. Acute correction of a deformity able not to close the deep fascia after deformity correction.
via an osteotomy can potentially stretch the adjacent nerve(s) Prophylactic anterior compartment fasciotomy and insertion
when the osteotomy and correction result in lengthening of of a drain have also shown to decrease the chance of
Fig. 6.28 Illustration of valgus deformity of tibia. Effect of nerve

stretch brought about by an opening wedge osteotomy is shown. The
amount of stretch effected “L” on the nerve is directly proportional to
the magnitude of angular correction “θ” and this can be calculated by
q
the formula L = 2p R where “R” is the distance of the relatively
360°
immobile nerve segment at the level of osteotomy from “C”, the centre
of rotational correction. With kind permission from Springer
Science + Business Media: Paley D. Principles of deformity correction.
Berlin, Heidelberg; 2002
Fig. 6.27 (a) Picture of a 6-year-old boy with chronic osteomyelitis

left femur with a supracutaneous locked plate fixation. (b) Pre- and
post-operative radiographs show corrective angulation and shortening
Skin and Wound Problems
osteotomy following sequestrectomy When the CORA is away from the site of correction large
displacements of the bone occur and these could result in
risk to the skin and soft tissue and wound dehiscence. Other
compartment syndrome in acute correction for Blount’s causes for skin necrosis are poor skin conditions prior to
disease [70]. Opening wedge osteotomy, large displacements surgery, subcutaneous bones such as tibia and hardware
and multiple osteotomies in the same or different segments prominence especially in subcutaneous areas such as femo-
of the same limb predispose to the development of a post- ral condyles.
operative compartment syndrome.
Iatrogenic Fractures
Vascular Intraoperative fractures can occur, as in osteogenesis
Vascular injury may occur secondary to direct injury, instru- imperfecta, or a longitudinal split, or while trying to stabilise
mentation or deformity correction. Such iatrogenic injuries fragments in the presence of excessive soft-tissue tension.
may be due to drill/osteotome/oscillating saw/inappropriate
retractor placement or implant placements such as screws, Physeal Injury
external fixator pins or excessive stretch following open Physeal damage can occur when correcting close to the joint
wedge osteotomy. The popliteal artery is at specific risk for or due to multiple penetrations by the fixation devices such
osteotomies around the knee joint, e.g. Rab’s osteotomy. The as nails or wide Kirschner wires. Use of intraoperative image
peroneal vessels are at risk when doing an adjuvant fibular intensifier is hence advised when dealing with juxta-physeal
osteotomy at its middle third [16]. deformity corrections.
Late Complications
Joint Stiffness
Soft-tissue adhesions following acute osteotomy correction
are often the cause of joint stiffness more so around the knee
and the ankle. Tethering of the ilio-tibial band and the quad-
riceps may also occur when external fixators are applied to
the thigh. Excessive intra-articular pressure following a
juxta-articular acute opening wedge osteotomy correction
could lead to sequel such as chondrolysis stiffness along
with chondrolysis and avascular necrosis can be associated
with distal transfer of the greater trochanter.
Vascular Fig. 6.29 Radiograph of 7-year-old boy referred for recurrence of

There are reports of delayed presentation of pseudoaneu- flexion deformity of both knees 2 years following corrective femoral
rysms caused by vascular injury related to instruments such osteotomy for the same
as an osteotome, or the penetration of vessels by fixator pins,
screws or wires [71]. Once recognised the treatment options
include ligating the involved vessel if insignificant for the
vascularity of the limb, embolisation and vascular repair. large angular corrections and thermal necrosis due to use of
power instruments such as an oscillating saw. This is also
Recurrent Deformity more likely when combining acute correction with gradual
Recurrence of the deformity following an osteotomy is likely lengthening. Some of this can be avoided by adding transla-
in the presence of metabolic bone disease, especially if the tion and collapse which increases stability and bone contact
underlying metabolic disorder is not corrected or controlled. and decreases length gained at the osteotomy site. Possibility
Other causes are residual soft-tissue contractures requiring of poor healing is high when doing acute corrections of greater
soft-tissue lengthening or muscle balancing such as in neuro- than 30° with associated lengthening [73].
muscular foot deformity (Fig. 6.29). In coxa vara, the pres-
ence of an open physis and failure to appropriately realign Hardware-Related Complications
the capital physis increase the risk of recurrence. In some of In children, the peri-implant complications are high because
the congenital limb deficiency and posteromedial bowing of the greater difference in the stiffness of the implant and
there is a deformity memory and this causes a recurrence by bone. As the underlying bone is smaller and the overlying
bowing of an immature callus. Lack of planning for further soft-tissue envelope less bulky, implant prominence is greater.
disturbed growth in the affected physis, poor bone quality Intramedullary implants being less elastic, if not perfectly
and ongoing disease process such as cystic lesions and aligned with the longitudinal mechanical axis, tends to cut out
fibrous dysplasia are other causes. Recurrence may also be or bone bends at the implant bone junction. The suggested
related to the severity of soft-tissue deformity or magnitude periods for implant removal will vary from 9 months to one
of initial deformity, poor choice of implant or premature and a half years, based on the underlying cause. In some
weight bearing. Correction or optimising the underlying conditions such as osteogenesis imperfecta, these implants
metabolic disorder prior to surgical correction is essential, are not removed, but revised with further growth.
and supplemental external or internal splinting following Even after the healing of osteotomy in deformity correc-
osteotomy correction is required in those with impaired met- tion in osteoporotic bone, fractures or bowing can occur at
abolic state [50]. However, the level of osteotomy, whether the implant bone interphase and cause the hardware to fail.
metaphyseal or diaphyseal, may not be related to the risk of Wrong choice of hardware, especially in those with osteope-
recurrence [50, 72]. In children with cerebral palsy, rota- nia, is another cause of implant failure or recurrence of
tional deformities tend to recur if de-rotation is performed in deformity (Fig. 6.30).
children less than 10 years of age [2].
Cast-Related Complications
Non-union and Delayed Union Patients with anaesthetic limb, impaired cognition and spas-
Non-union and delayed union are rare in children but could ticity have a predisposition to develop skin and soft-tissue
occur in the presence of excessive motion at the osteotomy sores related to overlying casts. To minimise the risk of
site, decreased bone contact and bone gap associated with such cast-related problems, bony prominences should be

10-year-old girl with
osteogenesis imperfecta (OI)
shows fixation failure due to poor
choice of implant and soft bones.
(b) The ideal implant for OI
would be an intramedullary
device
adequately padded and attempts at gaining further correction Acute Versus Gradual Correction
by wedging the cast must be avoided, especially in these
at-risk children. If cast wedging is done, it should include The complication rates with acute correction are generally
removal of cast wedge on the collapsing side to avoid exces- higher than those of gradual correction in the tibia [58, 69].
sive pressure on the underlying skin and soft tissues. Tight Gradual correction of tibia vara has shown to correct angular
plaster casts in the acute post-operative phase should be deformity more accurately than acute correction [58, 69].
avoided to avoid compartment syndrome. The possible cause could be the flexibility to correct minimal
malalignments with the gradual correction techniques, espe-
Muscle Weakness cially with use of computer-assisted technology. However,
When performing a closing wedge osteotomy and substantial the acute correction requires less supervision. Before choos-
shortening, there is a relative lengthening of the overlying ing acute or gradual correction for managing a child with a
muscle-tendon unit. For instance, a closing wedge supra- lower limb deformity, the treating surgeon should consider
condylar extension osteotomy can be associated with quad- factors such as the patient’s growth remaining, magnitude of
riceps insufficiency and weak knee extension. In some current and anticipated deformity and limb length discrep-
cases where the weakness persists despite physical therapy, ancy, availability of resources, familiarity with instrumenta-
one may require surgical measures such as patellar tendon tion and psychosocial issues.
reefing.
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Gradual Deformity Correction
7
David S. Feldman, Adam M. Kurland,
and Abdel Majid Sheikh Taha
Introduction Box 7.1

• Gradual correction is often safer than acute
Deformity correction for traumatic, congenital and develop- correction
mental conditions in the pediatric population may be quite • Gradual correction allows for adjustments
complex and the deformities can be quite severe. In children, postoperatively
the deformities may have their apex (or apices) at the level
of an adjacent joint or growth plate which then requires an
osteotomy away from the site of the deformity and requires
translation at the osteotomy site to restore a normal mechani-
cal axis of the affected limb segment. Additionally, deformi- History
ties may be multi-apical and are almost always in the oblique
plane. The patient may also have a limb with compromised The modern day history of gradual correction takes it origins
overlying soft tissues. from the work of Codivilla in 1905 [3] and based on our
The severity of the deformity and these other confounding research was first utilized clinically in 1934 at the Hospital
factors often make it advantageous to perform a correction for Joint Diseases in New York. De Bastiani and Ilizarov
gradually. The ability to correct a deformity gradually gives expanded the use of this work and explained and elucidated
the surrounding soft tissues sufficient time to accommodate. the biology of distraction osteogenesis. While this method is
Gradual correction may also allow for a more accurate utilized for limb lengthening, a gradual correction to correct
correction and a diminished risk of compartment syndrome, a limb deformity employs the same principles. Current tech-
nerve injury, and non-unions [1, 2]. nology continues to utilize these concepts with both,
Gradual correction provides the opportunity to make computer-assisted management with external fixation and
changes after the procedure, in an office setting, resulting in more recently with intramedullary devices. Distraction
a more accurate overall correction with less morbidity than osteogenesis is the biology required for gradual correction
an acute correction. This chapter reviews the history, biology, through an osteotomy site.
clinical indications and considerations, specific uses, com-
plications, and how to avoid complications during the
gradual correction of the lower limb.
Box 7.2
D.S. Feldman, MD (*) • A.M. Kurland, BA • A.M.S. Taha, MD • There are many devices that allow for gradual
Department of orthopedic Surgery, Division of Pediatric correction, internal and external.
orthopedics, Hospital for Joint Diseases, New York University,
67 Irving Place, 8th Floor, New York, NY 10003, USA • Historically external fixation has been utilized for
e-mail: [email protected]; [email protected]; gradual correction.
[email protected]

106 D.S. Feldman et al.
Biology Many modalities have been described to enhance bone for-

mation including electrical stimulation and low-intensity pulsed
In distraction osteogenesis, whether utilizing internal devices ultrasound [11–13]. Combining these modalities with the grad-
or more traditionally external fixation devices, the intact peri- ual allowance of weight bearing may accelerate the process of
osteum is likely responsible for bone formation [4]. Following remodeling of the regenerate and add to its strength by increas-
an osteotomy, the patient or caretaker is typically instructed to ing its bone density (refer to Chap. 28 for more details).
begin distracting the bone after a period of 5–10 days to allow
the intraosseous blood supply to recover. After this latency
Box 7.3
period with an ideal rate [5–7] (usually 1 mm/day, 1.5 mm/day
• Distraction osteogenesis is the biologic principle
with an intramedullary rod in some cases) and an ideal rhythm
utilized in gradual correction.
(traditionally 4×/day but continuous “auto distraction” is
• The regenerate can be enhanced with different types
likely even better), new bone will form in the distraction gap.
of modalities.
While leaving the intramedullary blood supply undisturbed at
the time of the osteotomy was thought to be crucial for this
regeneration, this has been questioned because the blood
supply likely regenerates 1 week after cutting the bone. It is
known that the intact periosteum is the most important struc- Clinical Uses
ture in distraction osteogenesis. The gradual tension generated
with distraction allows for filling in of the bone as it is angled Almost any degree of deformity can be corrected with
and/or lengthened. gradual correction (Fig. 7.1). Gradual correction, as the
Understanding how the bone is formed during the dis- name implies, allows for the slow and precise correction of
traction has been debated in the literature and it is likely deformity. The deformity may be angular, rotational, transla-
that both intramembranous and enchondral ossification tional, or any combination of these (Figs. 7.1, 7.2, 7.3, and 7.4)
occurs within the regenerate bone. Distraction osteogenesis [14]. Limb length inequality can also be addressed using this
is similar to fracture healing, in which an initial hematoma method (Fig. 7.5). This technique is typically associated with
transforms into fibrocartilaginous tissue and fibrous tissue. less disruption of the soft tissue envelope surrounding the
Within 2–3 weeks this tissue matures to a striated appearance deformity than with acute correction [1]. The soft tissue
of the regenerate [6, 8–10]. envelope includes many vital structures, such as blood vessels,
Fig. 7.1 Severe deformity. A patient with pseudopseudohypoparathy- gradual correction to correct very severe deformity. (a) Preoperative
roidism (pseudoPHP) with complex, multiplanar and multiapical defor- clinical and radiographic view. (b) During correction. (c) After one side
mities of both lower extremities using the Ilizarov technique and the is completed. (d) Final result
Taylor Spatial Frame™. This case demonstrates the possibility through
7 Gradual Deformity Correction 107
Fig. 7.2 In the case of hypophosphatemic rickets with a multiapical tibial and fibular rotational deformities were gradually corrected with
deformity and rotational deformities of the tibia and fibula, a femoral the use of a circular external fixation device. (a) Preoperative clinical
deformity resolved to one location and was corrected acutely while the and radiographic view. (b) During correction. (c) Final result
Fig. 7.3 Translation and angular

deformity—This deformity of
infected non-unions demonstrates
a case in which the translational
deformity cannot be corrected at
the site of deformity due to poor
soft tissue and a single vessel leg.
This translational deformity is
corrected by two angular
osteotomies away from the site of
the deformity. (a) Preoperative
clinical and radiographic view.
(b) During correction. (c) Final
result
Fig. 7.4 In a patient with

Ellis–van Creveld disease with
femoral retroversion and tibial
angular deformity, deformity
correction was performed via an
extra-articular osteotomy of the
femur with derotation and
gradual correction of the tibial
angular deformity with external
fixation. Note the initial patellar
dislocation was reduced once the
rotational deformity was
corrected. (a) Preoperative
clinical and radiographic view.
(b) During correction. (c) Final
result
nerves, muscles, and ligaments, which have different physical or compression or injury to the anterior tibial artery as it
properties from bone and thus may not tolerate the forces of transverses the interosseous membrane. Both of these eti-
acute correction, especially in the setting of a large deformity. ologies, i.e., swelling and injury to the artery, are much less
For instance in the case of a severe valgus deformity around common with gradual correction. Rarely are prophylactic fas-
the knee joint, performing an acute correction can stretch the ciotomies required in gradual correction.
common peroneal nerve, possibly resulting in a postoperative
nerve palsy and subsequent foot drop [14]. Gradual correction
allows for monitoring of the nerve function or dysfunction and Box 7.4
a release of the nerve may be performed during the correction • There are many clinical uses for gradual correction.
process, should symptoms develop. • Protection of nerve function and the soft tissue
Blount disease, a condition ideally suited for gradual envelop are key components to gradual correction.
correction, is an oblique plane (anterolateral) deformity of the
proximal tibia that presents in young children and adolescents
(see Fig. 7.6). The deformity is often at the level of or close to
the proximal tibial physis and may be associated with internal
tibial torsion [15, 16]. Historically, the operative correction of Analysis of Deformity
Blount disease was associated with malunions and delayed
unions as well as compartment syndrome and nerve injury Planning for deformity correction follows the same principles
(most commonly the nerve to the extensor hallucis longus). whether performing a gradual or an acute correction. One
Many of these complications can be minimized with gradual must analyze the axes in the coronal, transverse and in the
correction [17, 22]. Compartment syndrome occurs either sagittal planes [18, 19], identify the apex of each deformity,
due to swelling in the compartment obstructing venous return plan the level of the osteotomy (or osteotomies), and decide
Fig. 7.5 Deformity correction with limb length inequality. This case tion and lengthening of the tibia were achieved using a hexapod exter-
demonstrates tibial varus and femoral rotation concurrent with length nal fixation device. (a) Preoperative radiographic view. (b) Clinical and
inequality of both bones. An acute derotational osteotomy of the femur radiographic view during correction. (c) Final result
and bifocal osteotomy of the tibia were performed. Deformity correc-
on the surgical technique and hardware to be used. In addition potential when compared to normal bone and may heal at a
to analyzing the axes and determining the degree and loca- slower rate (see Fig. 7.7) [22]. Osteogenesis imperfecta is an
tion of the deformity, the patient’s age and remaining growth example in which the bone density and architecture is weak
are also important factors. Patients that are still growing can [23], making it more difficult to maintain alignment without
typically benefit from growth modulation procedures partic- supplemental measures such as preoperative bisphospho-
ularly when the deformity is close to the joint (refer to nates and the use of an intramedullary device instead of an
Chap. 4 for more details) [20]. To ensure accurate correction external fixator.
of a deformity of the lower limb, both the individual features The deformity must be quantified and should be analyzed
of the bone being osteotomized and the limb as a whole must on the anteroposterior (AP) and lateral radiographs. Varus or
be understood. The overall alignment of the extremity, the valgus angulation and the medial or lateral translation are
alignment of the tibia and femur independently, the joint measured on the AP view. Procurvatum or recurvatum and
alignment (laxity), and rotational deformity require evalua- anterior or posterior translation are measured on the lateral
tion (refer to Chaps. 2 and 3 for more details). radiographs. Rotational alignment is measured clinically
Of the many factors that affect bone healing, the quality using the thigh-foot axis with the patient in prone and if
of the underlying bone is perhaps the most important [21] necessary may be supplemented with a CT scan or MRI.
and should be the first step in assessing the deformity. The Length is measured using a standing full-length lower
potential to heal dictates the success of the procedure, the extremity radiograph. To quantify the deformity, mechanical
extent of fixation needed, and the possibility of hardware axis deviation (MAD) and joint laxity, a standing patella
failure. The ability of a bone to heal differs substantially forward full-length AP and lateral radiograph of both lower
between various conditions (Figs. 7.7 and 7.8). For instance, in limbs are used [2]. Femoral and tibial deformities are further
multiple enchondromatosis, the bone has a higher neoplastic quantified on patella forward AP and lateral views of the
Fig. 7.6 Angular deformity. This is a case of untreated Blount disease using a hexapod external fixation device. (a) Preoperative clinical and
in an adult. To elevate and restore the joint line, an intra-articular oste- radiographic view. (b) During correction. (c) Final result
otomy was performed. The deformity was then gradually corrected
individual long bones. In the case of the very young child

with unossified epiphyses, an intraoperative knee arthrogram Planning the Osteotomy
may be performed to outline the femoral condyles and tibial
plateau to more accurately assess the deformity present in Determining the level of the osteotomy is an integral part of
the tibia and/or femur (refer to Chap. 2 for more details). the presurgical plan. Ideally the osteotomy should be located
at the apex of the deformity. At times, the osteotomy cannot
be performed at the apex of the deformity due to the proxim-
Box 7.5 ity of the joint, the physis, or compromised surrounding soft
• Thorough analysis of the deformity including an tissues. These issues necessitate moving the osteotomy away
assessment of the quality of the bone is essential from the apex. For example in Blount disease, the apex is usu-
prior to gradual correction. ally in proximity to the knee joint or the proximal tibial phy-
• A thorough clinical exam and standardization of sis, making it impractical both to osteotomize in these areas
imaging studies allows for accurate deformity and to safely obtain satisfactory fixation at the osteotomy site.
analysis. Additionally, one must avoid performing the osteotomy prox-
imal to the tibial tubercle since this may lead to patella
Fig. 7.7 Poor regenerate secondary to an underlying condition. An which necessitated stabilization of the bone internally with a rush
11-year-old patient with hemangiomatosis and tibial bowing under- rod and locking plate. (a) Preoperative radiographic view. (b) During
went deformity correction with hexapod external fixation. correction. (c) Delayed union. (d) Final result
Hemangiomatosis prevented proper healing of the osteotomized bone,
Fig. 7.8 Delayed healing secondary to underlying condition. This case monolateral frame. The tibial deformity was treated with a hexapod
demonstrates deformity correction and lengthening in the setting of fixation device. The patient underwent simultaneous growth modulation
Ollier’s disease. The patient had limb length inequality and varus defor- on the contralateral side. (a) Preoperative radiographic view. (b) During
mity and underwent lengthening of the femur proximally with a mono- lengthening. (c) Femur after femoral lengthening and deformity
lateral fixator and deformity correction of the distal femur with the same correction. (d) During tibial deformity correction. (e) Final result, tibia
tracking problems and injury to the tibial physis [24].

However, performing the osteotomy away from the apex has Devices
its consequences. Translation of the bone is necessary in such
cases in order to restore the normal mechanical axis and allow Many techniques and various types of fixation devices can be
the joint lines to remain parallel to the floor. The further the utilized once the preoperative planning phase has been com-
osteotomy is from the apex of the deformity, the larger the pleted. External fixation remains the gold standard for
magnitude of the required translation. Additionally, it is nec- gradual deformity correction as it allows easy and accessible
essary to keep in mind that gradual translational correction postoperative manipulation of the deformity. Both circular
always begins with distraction. As long as the surgeon and monolateral options exist. Many circular systems utilize
accounts for these factors preoperatively, they should not the principle of the hexapod fixator in which there are two
prove problematic. rings: a fixed reference ring and a mobile ring (see Figs. 7.1,
The anticipated outcome of deformity correction is a 7.2, 7.3, 7.4, 7.5, 7.6, 7.10, 7.11, and 7.12). This configura-
normal mechanical axis without oblique joint lines (Fig. 7.9). tion allows for a three-dimensional movement of the mobile
In most cases, the need to have a straight bone is less important ring relative to the reference ring. The final configuration is
than having a corrected mechanical axis (see Fig. 7.1). the result of a combination of many uniplanar movements
Exceptions to this are the cases of metabolic bone diseases, namely anterior–posterior translation, medial–lateral trans-
such as hypophosphatemic rickets (see Fig. 7.2) or osteogen- lation, and internal–external rotation. It is simply many vec-
esis imperfecta in which a straight bone is often necessary for tors adding up over time to give the final vector correction,
proper healing and the avoidance of later stress fractures. which is the desired orientation of the distal fragment [2].
Many hexapod systems are available including the Eisenberg
fixator™, the Ortho-SUV™, the Smart Correction™, the
Box 7.6 Spider Frame™, the TL-Hex™ and the Taylor Spatial
• Factors affecting the location of the osteotomy include Frame™. These devices can be utilized to correct deformity
the etiology and the location of the deformity. not only of the tibia [2] or femur [25] but of the foot [26, 27]
• Bone quality may affect the osteotomy location and and upper extremity as well.
the type of fixation utilized. Monolateral devices, which are more typically used in
acute correction, may also be utilized in gradual deformity
correction (Fig. 7.13). The multi-axial correcting (MAC™)
Fig. 7.9 Surgical correction at

the location of the deformity, i.e.,
tibia or femur, is essential. This
patient initially presented with
20° of femoral varus and a near
normal tibia. The patient’s varus
was surgically corrected by 10°
via a distal femoral osteotomy,
leaving 10° of varus unaddressed.
The patient then underwent a
tibial osteotomy with external
hexapod fixation in an attempt to
address the remaining varus
deformity. This resulted in a
corrected mechanical axis but a
valgus deformity in the proximal
tibia and created oblique joints of
the knee and ankle. The patient’s
resultant MPTA was 103° (15° of
valgus) and aLDFA 94° (13° of
varus). This oblique joint line
creates sheer stress in the joints
involved. The patient now has
pain in his ankle and knee that
will require a revision osteotomy.
(a) Preoperative radiographic
view. (b) During deformity
correction. (c) Final result with
oblique knee and ankle joints
with pain. This osteotomy will
require a revision
fixator system is an example of a monolateral fixation that without concern of loss of correction through bending of the
allows for gradual correction in all planes. The MAC™ sys- regenerate [31] (refer to Chap. 8 for more details).
tem allows for two planes of angulation, two planes of trans- With the advent of internal lengthening devices, the use of
lation, compression, distraction and rotation. The internal fixation to achieve correction is gaining popularity.
adjustments are usually done in the outpatient setting. This One such method is the reverse planning technique which
system does not employ the use of computer software to achieves both length and deformity correction. As the name
plan the correction [28–30]. implies, this technique involves planning the correction in
A locking plate, used in concert with external fixation reverse, i.e., starting with the desired deformity correction
(lengthening over a plate), mimics internal fixation creating a and length and determining the appropriate position of the
stable fixed angle construct within the limb. It is inserted per- self-lengthening intramedullary nail (IM) at the time of the
cutaneously, allowing minimal iatrogenic soft tissue injury, osteotomy. Blocking screws are used to obtain optimal nail
and provides stability when locked after distraction osteogen- position and to prevent rod migration and loss of correction
esis. The locking plate allows for early removal of the frame (Fig. 7.14) [32] (refer to Chap. 9 for more details).
Fig. 7.10 Recurvatum. This 14-year-old patient with recurvatum and lengthening with external hexapod fixation. (a) Preoperative clinical
limb length inequality of the tibia secondary to posttraumatic physeal and radiographic view. (b) During correction. (c) Final result
arrest underwent proximal osteotomy, deformity correction, and tibial
Fig. 7.11 Gradual deformity correction of untreated clubfoot in a (b) Intraoperative clinical view. (c) During correction. (d) Final result
young adult with external fixation. (a) Preoperative radiographic view.
follow instructions and adhere to the postoperative regimen,

Box 7.7 which includes the correction schedule, physical therapy,
• Circular fixators, monolateral fixators, intramedul- and weekly office visits. Regular and close follow-ups mini-
lary devices and hybrid techniques can be utilized mize complications and allow for early detection when
for gradual correction. untoward events occur. Some complications, such as joint
• Blocking screws are often needed when intramedul- subluxations and dislocations, if unattended, may be delete-
lary devices are used. rious and life altering for the patient.
Patients can typically be discharged within 2–3 days post-
operatively, but must be instructed on the proper postopera-
tive program prior to their discharge. Protected weight
bearing with the aid of crutches or a walker is typically per-
Postoperative Care mitted immediately following surgery. While an inpatient,
physical therapy should commence to teach the patient exer-
The use of gradual deformity correction demands a compliant cises to maintain and restore range of motion of local joints.
patient (and caretaker) and a devoted surgeon. Applying the Physical therapy should be continued several times per week
fixation device is only the start of the journey. The surgeon as an outpatient, with range of motion exercises and pro-
must be committed and able to accommodate seeing the gressing to strengthening exercises (refer to Chap. 12 for
patient on a frequent basis. The patient and caretaker must more details).
Fig. 7.12 Protecting against joint subluxation and dislocation. This ening with a knee spanning hexapod fixator to prevent knee subluxation
case demonstrates distal femoral deformity and limb length inequality and dislocation. (a) Preoperative radiographic view. (b) Knee stabilized
secondary to a congenital femoral deficiency and associated knee joint by external fixator during correction. (c) Final result
laxity. The patient underwent deformity correction and femoral length-
Fig. 7.13 Deformity correction with monolateral fixation device. correction and limb lengthening was performed using a monolateral
A 15-year-old patient with humeral deformity and limb length inequal- fixation device. (a) Preoperative clinical and radiographic view.
ity secondary to neonatal sepsis-related physeal arrest. Deformity (b) During correction. (c) Final result
Fig. 7.14 Acute deformity correction with gradual lengthening: intramedullary rod with blocking screws. (a) Preoperative radiographic view. (b)
Immediate postoperative radiographic view. (c) During correction
Follow-up visits should typically be scheduled weekly for

the first month and then every 2 weeks until correction of the Box 7.8
deformity has been completed. When tricortical consolida- • A dedicated patient and surgeon with close follow-up
tion is observed on AP and lateral radiographs, the frame is are necessary for successful deformity correction.
often dynamized for 2 weeks prior to removal. Upon • Physical therapy and a home exercise program are
removal, the patient is allowed to foot-flat weight-bear with necessary to protect the adjacent joints.
the use of assistive devices, followed by gradual increase in
weight bearing until the return of full function [33].
The knee is particularly at risk when treating congenital

Potential Complications femoral deficiencies and must be observed postoperatively
for subluxation. To adequately protect the knee joint and
The potential complications of performing gradual correc- ensure maintenance of full extension, a hamstring and/or
tion can primarily be divided into those that are related to iliotibial band release including the lateral intermuscular
surgery more generally and those that are specific to this type septum may be necessary. The knee and hip are further at
of procedure. General complications include those related to risk for dislocation if femoral rotation is not properly cor-
anesthesia, infection whether at the surgical site or at a pin rected (Fig. 7.16).
site [34] and delayed union or non-union, which is a poten- During tibial lengthening the ankle is also at risk for a
tial complication of any surgery that involves an osteotomy. variety of complications. If the ankle becomes tight in equi-
Procedure specific complications include joint stiffness, nus, treatment initially includes physical therapy, but should
joint instability, subluxation, and potential dislocation. tightness persist a percutaneous tendoachilles lengthening or
The surrounding joints remain at risk during the correction gastrocnemius recession may be required. Fibular migration
phase and lengthening process. The ankle, knee, and hip all may occur proximally, especially when performing defor-
may be subjected to significant strain during gradual correc- mity correction expected to result in an increase of length
tion due to incidental limb lengthening. The hip is suscepti- greater than 2.5 cm. To preemptively protect against this, a
ble to dislocation both during and after femoral lengthening, syndesmotic screw is inserted through the distal fibula into
especially with a dysplastic hip. If the patient presents with the distal tibia (Fig. 7.17). Performing a gradual deformity
acetabular dysplasia, this should be treated prior to femoral correction, as opposed to an acute correction, lessens the
lengthening and deformity correction. Pelvic pins can also incidence of nerve damage; however, it remains a concern.
be utilized to stabilize the hip if it is inherently unstable In cases of genu valgum greater than 15–20°, a peroneal
(Fig. 7.15). Furthermore, an adductor or psoas release may nerve release may be required to prevent motor dysfunction,
be required to reduce stresses on the hip. dysesthesias, and pain on the dorsum of the foot.
Box 7.9
• Joints can sublux or dislocate around the area of
correction and must be protected.
• Spanning fixators can be used to protect the joint
during deformity correction.
• Soft tissue releases may be utilized to prevent and/
or treat contractures.
Summary
Gradual deformity correction has grown in popularity from

the early 1900s through Ilizarov and De Bastiani to the present
day. Instrumentation has evolved allowing for a more facile
gradual correction of complex deformities utilizing external
Fig. 7.15 External fixator spanning the hip joint utilizing pelvic pins to fixation and intramedullary lengthening devices. The future
stabilize the pelvis during deformity correction. Radiographic view, entails ever-improving devices and a better understanding of
pelvic pins the use of biologics to enhance the regenerate bone.
Fig. 7.16 Hip and knee complications: adolescent with a congenitally of the dysplastic hip and varus femur. (b) Radiographic view of bifocal
short femur that had previously undergone a successful 4-cm lengthen- femoral deformity correction and lengthening with monolateral fixation.
ing. The patient now had a limb length inequality of 5 cm and varus. (c) Femur fracture. (d) Plated femur fracture. (e) Osteomyelitis in the
Bifocal deformity correction and lengthening was performed with a femur, fixed in 70° of anteversion. (f–h) Progression of the destructive
monolateral external fixator that was placed on the femur and did not arthropathy and depression of the lateral plateau as well as the rotary
span the hip or knee joint. Postoperatively the patient developed a sub- subluxation of the knee joint. (i) Subluxation of the hip. (j) Dislocated hip
luxed hip and a rotary subluxation of the knee followed by a dislocation with a triangular femoral head. (k) Intraoperative supine clinical view of
of the hip, which was subsequently relocated. Additionally the patient the lower extremities prior to surgical relocation of the hip and treatment
developed a femur fracture through the regenerate following removal of of anteversion. (l) Radiographic view of the relocated hip and knee.
the fixator that was plated. The patient’s femur was fixed in 70° of ante- The view of the knee demonstrates the extent of the arthropathy.
version and developed osteomyelitis. (a) Preoperative radiographic view (m) Radiographic view of the total hip and knee replacements

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Hybrid Techniques for Limb Length
and Deformity Correction 8
Mark T. Dahl and Chang-Wug Oh
Despite these extraordinary gains in the field of limb

Introduction lengthening and deformity reconstruction, patients and their
families must endure months of pin site maintenance and
The primary indications for bone lengthening and deformity ever-present external fixation. The resulting soft tissue scar-
correction include limb length discrepancy resulting from ring can lead to further contracture and adjacent joint diffi-
congenital and posttraumatic conditions such as fibular culty. Additionally, Ilizarov’s methods take years and scores
hemimelia, congenital short femur, fracture malunion, and of cases for surgeons to master the techniques [4]. Even
premature growth arrest. Suitable patients include selected after years of experience with bone regeneration, surgeons
children, adolescents, and adults. Distraction osteogenesis commonly confront yet another new complication arising
(DO) has been successfully used over the last half-century to from the treatment.
lengthen bone [1]. Critical to successful DO is an optimal
rate and rhythm of distraction for which external fixation has
been a reliable tool [2, 3]. The disadvantages of external Lengthening Over Nail (LON)
fixation frames are well known to include pin tract infec-
tions, pain, soft-tissue tethering, and joint stiffness. Limb The technique, first proposed by Bost and Larsen, used an
lengthening complications such as joint contracture, stiff- intramedullary pin to prevent development of deformity dur-
ness, subluxation, fracture, residual deformity, and chronic ing lengthening in addition to the external fixator used to
pain are well described [4]. The prolonged treatment of bone gain length [5]. Paley, Herzenberg, and Bhave introduced the
lengthening with external fixation can be shortened by sub- modern method of lengthening over nail (LON) in 1997
stituting internal fixation for the external fixator during the (Figs. 8.1 and 8.2) [6, 7]. These authors described 29 patients
consolidation phase of lengthening, thereby saving the treated with an intramedullary rod inserted and an external
patient prolonged external fixation. fixator applied concomitantly at the time of the osteotomy
for lengthening. They compared this group to 31 previously
operated Ilizarov lengthenings. Lengthening over an intra-
medullary nail reduced the average duration of external fixa-
tion by nearly one-half. Knee range of motion returned to
normal 2.2 times faster in the group that had lengthening
over a nail. There were six fractures of the distraction bone
in the Ilizarov group. In the group that had had lengthening
over an intramedullary nail, one nail and one proximal lock-
ing screw failed. Complications were reduced by 25 % in the
nailing group. The authors described a pattern of over-
M.T. Dahl, M.D. (*) reaming by 2 mm, essentially under-sizing the nail prevent-
Gillete Children’s Specialty Healthcare, University of Minnesota, ing entrapment of the rod. They described placing pins
200 University Ave. E., St. Paul, MN 55101, USA
e-mail: [email protected]
intra-cortically, leaving them out of the marrow space to
avoid deep infection. The advantages of lengthening over an
C.-W. Oh, M.D.
Department of Orthopedic Surgery, Kyungpook National University
intramedullary nail thus include a decrease in the duration of
Hospital, 130, Dongdeok-ro, Jun-gu, Daegu 700-721, South Korea external fixation, protection against lengthening site fracture,
e-mail: [email protected] and earlier rehabilitation.

122 M.T. Dahl and C.-W. Oh
Fig. 8.1 (a) A 12-year-old boy had a femoral shortening of about lateral external fixator. (c, d) The target length was achieved. (e) Two
4.5 cm due to the growth arrest of distal femur. (b) Lengthening over an interlocking screws were fixed at the distal segment and the external
IM nail was performed using a trochanteric entry tibial nail and a mono- fixator was removed. (f) 1 year later, the distraction callus had healed
Fig. 8.2 This soldier was struck by an IED (improvised explosive angulation, rotation, and translation of the femur developed. (d–f) Acute
device), causing open brain trauma and open fractures of the left femur correction of the angulation, translation, and rotation was followed by
and right forearm. (a–c) A 10-cm leg length discrepancy (LLD) with lengthening over nail of 10 cm. (g) Functional gait was restored
8 Hybrid Techniques for Limb Length and Deformity Correction 123
Gordon et al. described their initial experience using

LON in preadolescents in a retrospective series [8]. Nine Plate-Assisted Lengthening (PAL)
children (average age, 9 years and 10 months) with femoral
length discrepancy were treated with femoral lengthening Iobst and Dahl first introduced this technique in 2007 [9].
over a humeral intramedullary nail. After nail insertion, a This study is a description of the surgical technique and a
monolateral external fixator was placed and lengthening retrospective examination of six patients treated using a tech-
was performed through a proximal osteotomy. No avascular nique designed for children with open growth plates as a
necrosis or proximal femoral valgus was noted at a mini- means to remove the fixator sooner than with a fixator alone.
mum of 2 years follow-up. The authors concluded that the A percutaneous locking plate is inserted at the beginning of
“technique is effective but has a high rate of complications, the lengthening, along with a circular external fixator. Axis
including osteomyelitis, which developed in two of the nine deviation was a problem in two cases, requiring adjustment
patients.” The technical challenge of pin placement remote during the locking of the plate. The plate is percutaneously
from the intramedullary device requires practice, but is nec- locked at the completion of lengthening, at which time the
essary to prevent the risk of bacterial contamination of the fixator is removed. The procedure is tedious because of fix-
canal from the external fixator pins. ator hardware interfering with access for locking screws. The
goal of shortening fixator time was accomplished with a
mean external fixation index of 0.42 months/cm. Oh et al.
Box 8.1 also described a technique similar to limb lengthening with a
• When positioning the external fixator pins or wires, submuscular locking plate [10, 11]. Submuscular plating has
contact with the IM nail should be avoided. the advantage of providing angular stability and preserving
• Over-reaming of the medullary canal by 1.5–2 mm the periosteal and endosteal blood supply, which is beneficial
may be needed to under-size the diameter of nail, for distraction osteogenesis. The main indication of this tech-
and thus prevent binding of the nail during nique is when an intramedullary nail is not a viable option, as
lengthening. in patients with a narrow medullary canal, bone deformity,
open physes, or joint contractures (Figs. 8.3, 8.4, and 8.5).
Fig. 8.3 (a, b) Clinical photo and AP X-ray of 6-year-old boy with distraction. (e) AP X-ray of the femur at 7 weeks, after plate locking
5.5-cm LLD secondary to congenital femoral hypoplasia. (c) AP X-ray and frame removal. (f, g) X-ray and clinical photo at completion of
of femur at 1 week of distraction. (d) AP X-ray of the femur at 3 weeks treatment
Fig. 8.4 (a) A 15-year-old boy had a tibial shortening of about 4.5 cm four screws were percutaneously placed in the distal segment (e) and
due to congenital postero-medial bowing. (b, c) A submuscular locking the external fixator was removed (f). Seven months later, the distraction
plate was placed laterally without involving the physeal plate, and a callus had healed without complications (g)
ring external fixator was fixed. After achieving the target length (d),
Fig. 8.5 (a) A 14-year-old boy had a femoral shortening of about were percutaneously placed in the distal segment (e) and the external
3.5 cm due to the Perthe’s disease. (b, c) Submuscular locking plate was fixator was removed (f). (g) 10 months later, the distraction callus had
placed laterally, and a monolateral external fixator was fixed anteriorly healed uneventfully
to lengthen the femur. After achieving the target length (d), four screws
Box 8.2 brace in tibia lengthening) is recommended until

• When placing the external fixator, half-pins or wires sufficient consolidation of the distraction callus is
should not contact plate or screws, to avoid cross- achieved.
contamination, which may evolve into deep infection. • When fixing a screw in skeletally immature patients,
• To give sufficient stability, a longer plate should be the adjacent physis should not be violated.
chosen unless an anatomical contraint is present. • Insertion of screws in the distal segment at the end
Also, enough screws are needed to stabilize each of distraction period should be performed prior to
segment. removal of the external fixator. This will prevent
• Protection from full weight bearing and the use of a loss of correction that was gained at the lengthen-
protective device (such as a patellar tendon bearing ing site.
Lengthening and Then Plating (LAP) Box 8.3

• LAP technique is beneficial in children who have an
This hybrid technique employs an external fixator lengthening, open physeal plate or a small intramedullary canal,
with the external fixator replaced by a percutaneously inserted as the IM nailing may be difficult to perform.
plate at the completion of the bone lengthening (Figs. 8.6 and • Because this technique does not require exposing
8.7) [12]. Rozbruch and Fragomen published a retrospective the distraction area and soft tissue dissection is kept
case-matched comparison between LAP and a more typical to the minimum, the risk of deep infection can be
Ilizarov technique with 27 extremities in each group [13]. The minimized.
time wearing the frame and external fixation index were lower • Caution is still needed not to involve pin tracks of
in the LAP group (4.5 vs. 6.2 months and 1.5 vs. 2 months/cm). the external fixator, which can be a source of
Axis deviation was observed in seven patients in the LAP delayed infection.
group and six in the classic group. Varus malalignment with
plate breakage occurred in two patients in the LAP group. Pin-
tract infection was more common in the classic group (12 vs.
2). No deep infections occurred in the LAP group.
The advantage to plating after lengthening is that the cir- Bone Transport with a Plate
cular fixator allows for a precise correction of alignment
before the plating. Care must be taken to leave open access For the segmental bone defect, trifocal and tetrafocal meth-
for the plate insertion, so that contaminated pin sites do not ods of bone transport with multiple osteotomies and two or
later cause infection of the plating site. Oh et al. noted that three levels of bone regeneration can shorten treatment
this technique is beneficial, especially when an IM nail is not times. However, the prolonged duration of external fixation
appropriate for children [14]. Rozbruch described that resid- required to enable consolidation of distraction callus is a
ual deformity may lead to plate breakage [13]. disadvantage and is frequently associated with complications,
Fig. 8.6 (a) An 11-year-old boy with a fibula hemimelia had a tibial Then the percutaneous plating was performed on the medial aspect of
shortening of about 4 cm. (b) A monolateral external fixator was fixed the leg, and (e) the external fixator was removed. (f) The distraction
anteriorly to lengthen the tibia. (c) The target length was achieved. (d) callus had healed uneventfully
Fig. 8.7 (a) Full-length standing AP X-ray of a 12-year-old boy with lation and length correction. Part (g) shows healing after percutaneous
hypochondroplasia and bilateral symptomatic genu varus. They desired contoured plating of the lengthening sites with simultaneous frame
a knee correction and lengthening to improve stature. (b–f) X-rays removal
showing the initial frame application, followed by simultaneous angu-
such as pin tract infection, joint stiffness, discomfort, and tion at the transported segment add stability to the con-
refracture. Bone transport over an IM nail has been construct, this technique can eliminate the fixator time required
ducted with the aim of reducing the duration of external for union at the distraction area as well as the docking site.
fixation, but the proximity of the pin to the nail introduces The risk of spreading infection can be minimized by plac-
the risk of deep infection and failure of fixation [15]. Oh ing the fixator half-pins sufficiently distant from the plate
et al. introduced “bone transport with a plate,” which or screws. This technique can be used in segmental bone
requires a shorter period of external fixation (Fig. 8.8) defects following trauma or infection, in both adults and
[16]. Because pre-inserted plate and additional screw fixa- children.
Fig. 8.8 (a) An 18-year-old male patient had a segmental tibial defect percutaneous screws were fixed at the transported segment and autog-
filled with an antibiotic-impregnated cement block. (b, c) A laterally enous bone grafting performed at the docking site. (f) Radiographs after
based locking plate and a medially based monolateral external fixator 10 months show satisfactory union achieved at the distraction callus
were applied for bone transport. (d) With a gradual distraction osteo- and docking site
genesis, docking of the transport segment was achieved. (e) Then two
Lengthening and Then Screw Fixation

Box 8.4
• Compared to bone transport over a nail, this tech- Ilizarov taught us that the most extreme foot deformities
nique may further reduce the external fixation time must be corrected gradually to stretch the soft tissues and
because the screw fixation at the transported seg- neurovascular structures into the desired position. Conditions
ment adds stability and can eliminate the time such as severely deformed clubfeet can be corrected in this
required for union at the docking site. way with or without osteotomy. Prolonged subsequent
• In order to minimize the risk of secondary infection, external fixation protects the regenerate bone and corrected
the external fixation pins are inserted sufficiently position, but often results in foot stiffness. Inserting percuta-
remotely from the plate or screws. neous screws, pins, or staples at the time of completion of
• If the remaining segment is too short to insert an IM deformity correction allows for earlier removal of the fix-
nail, such as in the case of juxta-articular bone ator. In unpublished experienced, one of the authors (MD)
defects, the use of transport with a plate can be very has used this technique a few times in recent years
useful. (Fig. 8.10). Several weeks of external fixation time can be
saved. The percutaneous, fluoroscopic-guided insertion of
cannulated screws thus protects the osteotomy sites along
with casts or braces.
Lengthening and Then Nailing (LATN)

Summary
This technique, described by Rozbruch in 2008 [13], employs
a conventional deformity correction and external fixator A variety of hybrid techniques have been established to
lengthening, with the external fixator replaced by a percuta- improve the patient experience during limb length and defor-
neously inserted locked nail at the completion of the bone mity correction, by reducing external fixation time to the
lengthening. Its greatest indication is in those cases where latency and distraction phases. Each of these techniques
acute intraoperative deformity correction is most difficult. shares the requirements of careful preoperative planning,
The circular fixator gradually corrects deformities, at the prevention of cross-contamination of external fixator pins,
completion of which a locked nail is inserted (Fig. 8.9). and skill with internal fixation devices.
Fig. 8.9 (a, b) This young man’s tibia fracture was treated with an gradual length and deformity correction. (e) When length was restored,
IM nail, resulting in a nonunion, malunion with shortening of 2.5 cm, a locked IM nail was placed in the tibia and an IM pin placed in the
varus angulation and lateral translation. (c, d) The existing nail was fibula. (f, g) Completed lengthening and healing with equal leg lengths
removed, fibula osteotomized, 6-axis circular fixator applied with was achieved
Fig. 8.10 (a, b) This 11-year-old girl has bilateral fibular hemimelia, with a circular external fixator is ideal for this deformity. (g) Maintaining
with the right foot in extreme equinovalgus. (c) Prior surgery failed to external fixations until healing is unnecessary, as multiple internal fixa-
create a plantigrade foot. (d) A posterior calcaneal osteotomy is par- tion screws provide the support needed and spare the patient an addi-
tially opened acutely, limited by soft tissue tolerance. (e) Interpositional tional 2–3 months in the frame
grafting maintains separation of the osteotomy. (f) Gradual correction
6. Paley D, Herzenberg JE, Paremain G, Bhave A. Femoral lengthen-

Box 8.5 ing over an intramedullary nail. A match-case comparison with
Ilizarov femoral lengthening. J Bone Joint Surg Am. 1997;79:
• Lengthening over nail reduces external fixation 1464–80.
time and facilitates early rehabilitation. 7. Herzenberg JE, Paley D. Femoral lengthening over nails (LON).
• Plate-assisted lengthening in children reduces fix- Tech Orthop. 1997;12(4):240–9.
ator time. 8. Gordon JE, Goldfarb CA, Luhmann SJ, Lyons D, Schoenecker
PL. Femoral lengthening over a humeral intramedullary nail
• Lengthening and then nailing allows for complex in preadolescent children. J Bone Joint Surg Am. 2002;84-A(6):
deformity correction followed by nailing. 930–7.
• Complex foot correction can be done gradually, but 9. Iobst C, Dahl MT. Plate assisted limb lengthening. J Pediatr Orthop.
secured with screws to hasten fixator removal. 2007;27(5):504–9.
10. Oh CW, Song HR, Kim JW, Choi JW, Min WK, Park BC. Limb
lengthening with a submuscular locking plate. J Bone Joint Surg Br.
2009;91(10):1394–9.
11. Oh CW, Kim JW, Baek SG, Kyung HS, Lee HJ. Limb lengthening
with a submuscular locking plate. J Bone Joint Surg Essent Surg
References Tech. 2013;3(4):e24.1–9.
12. Harbechuski R, Fragomen AT, Rozbruch SR. Does lengthening and
1. Green SA. The rancho technique. Orthop Clin North Am. then plating (LAP) shorten duration of external fixation? Clin
1991;22(4):677–88. Orthop Rel Res. 2012;470:1771–81.
2. Ilizarov GA. The tension-stress effect on the genesis and growth of 13. Rozbruch SR, Kleinman D, Fragomen AT, Ilizarov S. Limb length-
tissues: Part I. The influence of stability of fixation and soft-tissue ening and then insertion of an intramedullary nail: a case-matched
preservation. Clin Orthop Relat Res. 1989;238:249–81. comparison. Clin Orthop Rel Res. 2008;466:2923–32.
3. Ilizarov GA. The tension-stress effect on the genesis and growth of 14. Oh CW, Shetty GM, Song HR, Kyung HS, Oh JK, Min WK, et al.
tissues: Part II. The influence of the rate and frequency of distrac- J Pediatr Orthop B. 2008;17(5):265–9.
tion. Clin Orthop Relat Res. 1989;239:263–85. 15. Oh CW, Song HR, Roh JY, Oh JK, Min WK, Kyung HS, et al. Arch
4. Dahl MT, Gulli B, Berg T. Complications of limb lengthening: a Orthop Trauma Surg. 2008;128(8):801–8.
learning curve. Clin Orthop Relat Res. 1994;466:189–97. 16. Oh CW, Apivatthakakul T, Oh JK, Kim JW, Lee HJ, Kyung HS,
5. Bost FC, Larsen LJ. Experiences with lengthening of the femur over et al. Bone transport with an external fixator and a locking plate for
an intramedullary rod. J Bone Joint Surg Am. 1956;38:567–84. segmental tibial defects. Bone Joint J. 2013;95-B(12):1667–72.
Motorized Intramedullary
Lengthening, an Emerging 9
Technology for Limb Length
and Deformity Correction
Mark T. Dahl
Introduction Indications
The first description of limb lengthening is credited to The primary indications for bone lengthening and deformity
Codivilla in 1905 [1]. Limb lengthening has evolved from an correction with motorized intramedullary lengtheners
open step-cut osteotomy, distracted by bed-confined traction, include limb length discrepancy resulting from congenital
to Ilizarov’s methodology of distraction osteogenesis whose and post-traumatic conditions such as fibular hemimelia,
principals include a blood preserving corticotomy, a latency congenital short femur, fracture malunion, and premature
period, regular rate and rhythm of distraction, stable circular growth arrest. Suitable candidates must have completed
external fixation, and functional use of the extremity during growth at the adjacent physis, have adequate canal diameter
treatment. Three decades ago, a small cadre of surgeons and bone length to accept the available nails, and have a
learned Ilizarov’s methods directly from him, and they are canal suitable for intramedullary nailing. These patients
now passing this information on to the next generation of include selected children, adolescents, and adults. Distraction
limb reconstruction surgeons. With this “passing of the osteogenesis (DO) has been successfully used over the last
torch,” we now have the option of a technical refinement to half-century to lengthen bone. An optimal rate and rhythm of
the process, that is, remotely controlled, motorized, tele- distraction is critical to successful DO, for which external
scopic, intramedullary lengthening nails. The discomfort fixation has been a reliable tool [2, 3]. The disadvantages of
associated with the prolonged use of external fixation of any external fixation frames are well known and include pin tract
kind is often poorly described in the literature, but is well infections, pain, soft-tissue tethering, and joint stiffness [4].
known to patients and surgeons. Although the Ilizarov meth- Limb lengthening complications such as joint contracture,
odology included many innovative biological and mechani- stiffness, subluxation, fracture, residual deformity, and
cal concepts that were previously unknown, external fixation chronic pain are well described [5]. Bone lengthening with a
is not without its problems, which include length of time fully implantable device is desirable in order to avoid exter-
required for the treatment, pin site infection, soft tissue teth- nal fixation, mechanical integrity and accurate control of dis-
ering, and scarring at the pin sites, with patients often traction are mandatory. As with any intramedullary rod,
expressing dissatisfaction during the process. This chapter lengthening must occur along the anatomic axis of the bone,
describes the latest methods of delivering Ilizarov’s princi- which will result in a translation deformity, the effect of
ples to patients by fully implanted, motorized, telescopic, which is determined by the original anatomy and the magni-
intramedullary lengthening nails. tude of the lengthening [6]. The prediction and prevention of
these iatrogenic deformities using a novel preoperative plan-
ning technique known as “reverse planning” is essential in
preventing abnormal mechanical loads in the future [7].
M.T. Dahl, MD (*) Remote-controlled, motorized, internal lengthening devices
Gillete Children’s Specialty Healthcare, University of Minnesota,
200 University Ave. E., St. Paul, MN 55101, USA (MILD) have recently become available for use in the femur
e-mail: [email protected] and tibia [8–10].

132 M.T. Dahl
The “Holy Grail” of limb lengthening could be consid-

History ered an intramedullary nail capable of bone fragment sta-
bilization, gradual deformity correction, and lengthening,
Ilizarov’s remarkable discovery of bone regeneration, com- obviating the need for external fixation altogether. Such a
monly referred to as the Ilizarov method, has opened win- device is not currently available since intramedullary nails
dows into the treatment of many conditions previously are only capable of bone stabilization and gradual length-
considered as hopeless maladies. ening or shortening. The correction of angulation, transla-
It is now possible to combine Ilizarov’s principles with tion, and rotation must be done acutely by the surgeon at
those of intramedullary fixation for limb lengthening by the initial surgery, limiting its applicability due to poten-
using preoperative planning, less invasive surgery, temporary tial neurovascular compromise and damage to potential
intraoperative external fixation, specific reaming techniques, bone regeneration.
and intramedullary fixation. Telescopic nails with miniature
motors are remotely controlled by either radio frequency or
magnetic energy [8, 11]. These techniques require precise A Brief History of Internal Limb Lengtheners
planning and execution because only axial length can be
adjusted after the surgery without an additional operation. Bliskunov described an internal lengthening device that con-
Distraction osteogenesis (DO), largely introduced by sisted of an intramedullary femoral rod bolted to the iliac
Ilizarov, now enables us to lengthen bones in children with wing and lengthened by a ratchet mechanism [18]. Cole
conditions such as fibular hemimelia or a congenitally short introduced a device, the intramedullary skeletal kinetic dis-
femur without the additional need for bone grafting [2, 3]. tractor (ISKD), that is actuated by the inherent rotational
We spare these children additional surgeries by tapping the characteristics of gait [19]. The Albizzia nail, introduced in
body’s capacity to form regenerate bone, but the new bone France by Guichet, also has mechanical actuation [20]. The
requires the prolonged use of external fixators throughout Albizzia and ISKD are still in use, but require the patient to
consolidation. Fractures, stiffness, and residual deformity perform intermittent axial rotation of the limb to effect dis-
are often a result of months in external fixation. With DO, we traction. These modalities perform well, albeit rate control
are also able to achieve union in congenital pseudarthrosis of difficulty is reported with each [21, 22] (Figs. 9.1 and 9.2).
the tibia more predictably, a formerly hopeless condition
which often resulted in amputation after multiple attempts at
union with bone graft, pins, and casting [12]. The correction
of severely deformed limbs from developmental, infectious,
and traumatic conditions such as Blount’s disease by using
simultaneous, gradual angular, rotational, and length correc-
tions can now be more precisely performed with web-based
computer analysis than with the Ilizarov device with six-axis
correction [13]. Massive bone defects resulting from trauma,
infection, and tumor can be filled by spontaneously regener-
ating the patient’s own bone by compressing the defect site
while growing new bone at a remote site [14].
Despite these extraordinary gains in the field of limb
lengthening and deformity reconstruction, patients and their
families must endure months of pin site maintenance and the
ever-present external fixation. The resulting soft tissue scar-
ring can lead to further contracture and difficulty with mobi-
lizing the adjacent joint(s). Additionally, it takes years and
scores of cases for surgeons to learn and then become experts
in Ilizarov’s methods. Even after years of experience with
bone regeneration, surgeons commonly confront yet another
new complication arising from the treatment [5].
Intramedullary fixation to supplement external fixation
during limb lengthening has lead to a new generation of limb
lengthening technology, described in a separate chapter as
“hybrid lengthening”. These techniques include lengthening
over nail and plate assisted lengthening, each of which Fig. 9.1 The ISKD was found to be most useful for posttraumatic fem-
reduces the time spent in external fixation [15–17]. oral length discrepancies
9 Motorized Intramedullary Lengthening, an Emerging Technology for Limb Length and Deformity Correction 133
Fig. 9.3 The FITONE nail has an attached cable and antenna. The
transformer box shown converts electric current to radiofrequency
waves, transmitting energy transcutaneously
Fig. 9.2 The ISKD and Albizzia nails are activated by manual limb
rotation
A new era has begun with the introduction of intramedul-

lary lengthening devices whose distraction is controlled by
an external power source which causes an internal actuator to
effect the desired amount of distraction. Baumgart and Betz
first introduced such a device in 1992 [8]. Baumgart further
developed specific insertion tools for the FITBONE nail
(Wittenstein Intens, Igersheim, Germany), and published a
novel planning method in 2009 [7]. The FITBONE device
employs an electric motor imbedded in the telescopic rod,
which is activated by intermittent transcutaneous transmis-
sion of radiofrequency waves to a subcutaneous receiver that
converts these waves into an electrical impulse discharged
via a connecting cable (Fig. 9.3). Distraction only occurs Fig. 9.4 (a, b) The FITBONE stump lengthener was used to lengthen
when the transducer is placed directly over the receiver, a traumatic above knee amputation for a total of 18 cm, allowing the
allowing precise control of distraction rate and rhythm. This patient to be fitted with prosthesis and walk without assistive devices
device has since undergone multiple improvements, and the
current generation has been used in over 2,000 cases world- The PRECICE nail (Ellipse Technologies, Irvine, CA)
wide. Models suitable for antegrade, trochanteric, and retro- was FDA-approved and introduced in 2012. The telescopic
grade femoral use are available. A tibial model is in use as nail has a magnetic drive mechanism that is activated by a
well. A bone transport device and a model for short amputa- handheld external electromagnetic controller. Similar to
tion stump lengthening are also available (Fig. 9.4a, b). The FITBONE, the surgeon instructs the patient in its use, and
FITBONE device requires specific training under the direc- the device is specifically programmed by the surgeon to pre-
tion of its inventor, Professor Rainer Baumgart or a designee. vent application of an improper rate. The patient or family
The FITBONE device is currently available in the USA only member then performs the distraction by applying the
under an FDA-approved compassionate use exemption. controller directly over the internal magnet in the limb three
134 M.T. Dahl
ferences are considered to be part of the tibial discrepancy.

A standing lateral film of the femur or tibia is necessary,
inspecting for obvious angulation as well as the more subtle
variation in the canal thickness often observed in longstand-
ing deformities. All X-rays are made with a magnification
marker so that the segment length and canal diameter can be
accurately measured.
The Reverse Planning Method
The Reverse Planning Method described by Baumgart

begins with defining the final ideal correction accounting
for length, angulation, and translation [7]. This method can
be used with any approach to the femur or tibia, but is par-
ticularly necessary for femoral lengthening, as intramedul-
lary lengthening along the anatomic axis of the femur will
cause medial translation of the knee and lateralization of the
mechanical load to the knee. This coronal plane translation
has been estimated at 1 mm per centimeter of lengthening,
but depends on the magnitude of the preoperative deformity,
the location of the corticotomy, the neck-shaft angle, and the
length to be achieved [23]. Deformity correction with an
intramedullary nail must then occur acutely intraoperatively,
anticipating the future position of the knee. Intramedullary
Fig. 9.5 The PRECICE mechanism has a spindled magnet attached to lengthening of the tibia does not typically lead to similar
gear boxes which rotate against the distraction rod translation problems since the tibial anatomic axis normally
approximates its mechanical axis.
It is important to execute the following steps with preci-
to four times daily, with the rate and rhythm being adjusted sion in the preoperative planning process (Fig. 9.6a–g).
according to weekly clinic visits and radiographs. This
device can apply either compression or distraction, does not Step 0
require a cable/antenna-receiver, and is fully FDA-approved. Make a full-size tracing of the radiograph of the entire lower
The manufacturer recommends specific advanced instruction limb, marking reference points, angles, and lines. Once the
for its users. Either device requires removal after complete bone to be corrected and lengthened is determined by
corticalization has developed, typically 1–2 years after place- the malalignment test [24], consider its curvature in both the
ment. The PRECICE device has now been used in over 800 coronal and sagittal planes. If a straight track for the nail can-
cases worldwide (Bart Balkman, Ellipse Technologies, per- not be achieved, an alternate corticotomy site or a second
sonal communication) (Fig. 9.5). osteotomy should be considered. If the medial proximal tib-
ial angle (MPTA) is normal (85–90°), continue the tibial
mechanical axis line to a point well above the current femo-
General Guidelines for Motorized ral head location (see Fig. 9.6a).
Intramedullary Limb Lengthening
Step 1
As with all limb lengthening procedures, appropriate sur- Draw and extend the mechanical axis of the tibia proximally
geon education is essential. The properly educated surgeon with sufficient length to account for the lengthening goal
then organizes a dedicated team of nurses, therapists, and (see Fig. 9.6b).
orthotists to ensure the comprehensive care of these patients
who often have complex medical and psychosocial issues. Step 2
Preoperative studies include a standing film of both lower Draw the location of the femoral head in its final position after
extremities with block leveling of the pelvis. Foot height dif- lengthening along the mechanical axis has occurred (see
Fig. 9.6 (a–g) Reverse Planning Method for retrograde nailing of the femur
Fig. 9.6c). Choose a corticotomy level near the deformity apex, sagittal translation and angulation, and should allow the exact
but of sufficient distance from the knee joint to allow for both nail position to be duplicated. If coronal deformity exists, the
locking and blocking screw insertion (generally 7–10 cm from corticomy can be manipulated directly with an osteotome
the knee joint line). Depending on the severity of the deformity and/or indirectly with Shanz pins and sterile bumps.
and the magnitude of the planned lengthening, selection of a
corticotomy site too distant from the knee may result in excess
cortical reaming and undesirable conditions for healing. Visual Aids
Step 3 Creating visual aids for surgery can be helpful in many ways.
A second tracing is made outlining the proximal femur to the The preoperative plan, printed full size, is secured to the surgi-
level of the corticotomy (see Fig. 9.6d). The anatomic axis is cal suite wall, next to the patient radiographs. Skin markings
then defined and drawn, and the nail is outlined to scale. In are then created. The fluoroscope is brought into the field in
this step, the surgeon must consider the effect of reaming in the AP projection. The hip, knee, and ankle reference points
both planes, being certain that cortical integrity is maintained and lines are marked on the overlying skin with a sterile mark-
to accommodate the size of implant and characteristics of the ing pen. The anatomic and mechanical axes are marked on the
bone. bone to be treated. The patella and infrapatellar ligament are
marked since they are used to maintain consistent AP posi-
Step 4 tioning. The proposed corticotomy site is marked (Fig. 9.7).
After overlapping the second tracing on the first, and placing The selected nail is opened and the proximal, distal, and
the femoral head in its future location, the papers are then telescopic portions are indicated with skin clips to identify
swiveled until the anatomic axis lines are matched. The nail the desired reaming depth. Anatomic hazards to be avoided,
tracing should enter the distal fragment in a position of ana- such as growth plates, are also marked. These marks are then
tomic harmony, and the mechanical lateral distal femoral used for visually referencing Shanz pin insertion and ream-
angle (LDFAm) should approximate 90° (see Fig. 9.6e). ing paths, and for assisting the surgeon in reducing X-ray
exposure (Fig. 9.8).
Step 5
Shift the outline of the second drawing distally along the ana-
tomic axis line to the point where contact is made between the Venting the Canal
corticotomy ends (see Fig. 9.6f). This image of the bone seg-
ment and nail position, including angulation and translation, Reaming a closed long bone causes elevation of intramedul-
now becomes the intraoperative goal (see Fig. 9.6g). It will lary pressure and intravasation of marrow contents, with the
replicate the desired intraoperative position with regard to potential for creation of fat embolism. This complication is
136 M.T. Dahl
Fig. 9.8 Reaming depth can be guided by applying skin clips at the
appropriate levels
Entry Points
Fig. 9.7 Sterile markings are made on the surface of the skin to aid Ideal entry points are required for proper intramedullary nail-
canal preparation
ing and are defined by several authors [26, 27] (Fig. 9.11a, b).
The correct starting point for trochanteric entry is actually
on the further-most point medial on the tip of the trochanter
best avoided by venting the canal to decrease the marrow without entering the piriformis fossa.
pressure [25]. The planned corticotomy site is routinely
selected as the venting site. This site is drilled (vented) in
percutaneous or open fashion using a subperiosteal approach Reaming Techniques
prior to instrumenting the canal. A series of 4–5 mm drill
holes allow intramedullary reamings to spill at the corticot- As the telescopic nails are straight and the canal is not, one
omy site, also adding biological support to the lengthening must either over-ream a curved canal to accommodate the
site (Fig. 9.9a, b). straight nail, or ‘fit’ rigid reaming to match the straight nail.
The actual corticotomy will be performed at a later point After the canal has been vented and the Shanz pins placed,
in time after the canal preparation. reaming can proceed with attention to appropriate entry
points suitable to the approach chosen [26, 27].
Shanz Pins
Conventional Reaming
Shanz pins are inserted with c-arm guidance in the lateral
position. The femoral condyles are rotated so as to overlap The canal is first vented. A bulb tipped guide wire is placed.
each other on a lateral view, and the pin is inserted from lat- Flexible reamers prepare a cylindrical path but curve at mul-
eral to medial, just inside the posterior cortex at the junction tiple apices, thus requiring over-reaming of 2 mm to accom-
of the metaphysis and epiphysis, and out of the way of the modate a straight nail. Corticotomy is generally performed
future passage of the nail (Fig. 9.10). after this “over-reaming” with flexible reamers.
Fig. 9.9 (a) Venting the canal at

the level of the future
corticotomy is done with a series
of drill holes, thus creating a
subperiosteal pocket into which
reamings will spill. (b) A 5-mm
drill bit with sleeve serves this
purpose
Fig. 9.10 The knee joint skin

mark assists parallel Shanz pin
insertion without switching c-arm
position. The proximal pin is
placed rotationally parallel to the
distal pin, at the level of the
lesser trochanter, perpendicular
to the shaft, and posterior to the
future nail path
Fig. 9.11 (a) Trochanteric entry

is at the medial-most tip of the
trochanter. (b) Reaming sleeves
protect soft tissue
Rigid Reaming Rigid reamers are inserted within the tubes along the
planned pathway, and are increased in 0.5 mm increments to
The canal is first vented. Canal preparation with rigid ream- 0.5 mm greater than the nail diameter. After canal prepara-
ers creates a well-defined pathway for the straight nail with a tion on the “near side” of the corticotomy, the corticotomy is
future line-to-line fit. Blunt or sharp rigid reamers can shape completed with an osteotome and the deformity is corrected
curved canals with asymmetric wall thickness to accept the to match the plan in step 5. With the plan achieved, reaming
nail (Fig. 9.12a, b). then proceeds on the “far side” of the corticotomy to the
Reaming sleeves passed over insertion dilators protect 0.5 mm greater than the nail diameter.
soft tissues from the reamer blades and prevent spillage of Regardless of reaming technique, the nail is inserted with
marrow contents into the joint or soft tissues. simple hand pressure, reaming slightly more as necessary to
138 M.T. Dahl
avoid mallet use, and potential damage to the micromotor. We have learned from our bone lengthening experiences
Alignment is checked, the corticotomy is compressed, and that deformities developing during lengthening are a common
locking screws are inserted. event. Asymmetric muscle forces imposed by unequal muscle
mass, muscle anatomy, and two-joint muscles create the pre-
dictable deformities of varus-procurvatum during femoral
Blocking Screws lengthening and valgus-procurvatum during tibial lengthen-
ing. The development of such deformities can be corrected by
Blocking screws (Poller) are known to be useful in trauma adjustments of the fixator in the outpatient clinic when length-
and reconstruction applications of intramedullary fixation for ening with circular external fixation. When lengthening with
correcting and preventing deformity [28, 29]. They function intramedullary techniques, these deformities must be antici-
to narrow a canal in epiphyseal and metaphyseal areas, and pated and prevented with the appropriate use of blocking
thus guide nails in juxtaarticular applications (Fig. 9.13 a–c). screws, as later correction requires additional surgery.
Antegrade Femoral Lengthening
Patient Indications
Antegrade femoral lengthening is indicated for patients with

leg length discrepancy originating from the femur. Angular
and rotational deformity of the proximal to mid femur can be
corrected acutely, with the latency period suitably delayed to
account for the correction magnitude. A piriformis entry can
be used for adults. A trochanteric entry has been shown to be
safe for preadolescents as young as age 12 years [30]
(Fig. 9.14a, b).
Lengthening along the anatomic femoral axis will result
in 1 mm of knee medialization for every 10 mm lengthened,
potentially resulting in an undesirable transfer of load later-
ally [18]. Assessment of the weight-bearing line on preoper-
ative full-length AP X-rays will be helpful in determining
whether this amount of medial translation will be harmful to
Fig. 9.12 (a) Carefully inspecting the canal preoperatively determines
if the canal will accept an intramedullary nail. (b) In specific instances the knee in the future. Acute deformity correction can be
rigid reamers may be used to “shape” the inner canal assisted by temporary intraoperative placement of Shanz
Fig. 9.13 (a) Blocking screws are placed against the nail. (b) Blocking screw location is specific to each case, generally on the “concave” side of
potential deformity. (c) A single posterior screw maintained this extension osteotomy
Fig. 9.14 (a) A 16-year-old girl

with a 3.5-cm idiopathic right
femoral shortening with
preoperative weight bearing line
2 mm medial to the medial spine,
suggests an antegrade
lengthening will result in
lateralization of 3.5 mm. (b) A
trochanteric entry best avoids the
hip vasculature, and resulted in
the predicted and acceptable
weight bearing line 1 mm lateral
pins, and progressive deformity during lengthening can be canal. Shanz pins are placed in the proximal and distal seg-
prevented by blocking screws (Fig. 9.15a–c). ments away from the nail tract to mark rotation and control
the bone segments.
Careful preoperative evaluation of individual hip anatomy
Planning for Antegrade Femoral Lengthening allows determination of the ideal piriformis or trochanteric
entry. For example, a proximal femoral flexion deformity
A graphical method of planning has been described for requires a more posterior entry point. Acquired hip deformi-
length and deformity correction [31]. The corticotomy ties such as anterior displacement of the greater trochanter
should be made at the apex of the deformity in the coronal can malign the entry point as well. The hip is adducted and a
and sagittal planes, when possible. wire is inserted into the intramedullary canal through the piri-
formis fossa or medial tip of the greater trochanter. A 2-cm
Antegrade Femoral Technique incision is made over the wire, and a soft-tissue protector is
A radiolucent table and a bump under the ipsilateral buttock inserted. The intramedullary canal is opened with a cannu-
are used. Sterile draping must provide adequate exposure of lated drill, and then reaming is performed by either of the
the proximal buttock for nail entry. The ipsilateral arm is previously described methods. The assembled nail is inserted
supported across the chest and protected during reaming. At upto the corticotomy site. An osteotome is used to finish the
the planned corticotomy level, a 1-cm incision is made and corticotomy, with completeness confirmed by fluoroscopy.
multiple holes are drilled in a transverse fashion, venting the Deformity correction is completed directly by osteotome
140 M.T. Dahl
Fig. 9.15 (a) Post traumatic

knee ankylosis makes an
antegrade approach to this
femoral lengthening necessary.
(b) Childhood Perthes altered
proximal femoral anatomy;
making piriformis entry more
suitable for the first femoral
lengthening of 8 cm. (c) The
second lengthening was similarly
approached through the
piriformis fossa
Box 9.1. General Guidelines Summary

1. Blocking screws should be employed to prevent deformity at insertion or during lengthening. They are less likely
needed if the deformity and corticotomy apex is at the isthmus, and/or the lengthening is small, less than 3 cm.
2. Blocking screws are most commonly needed in the distal femur and proximal tibia, as these locations often have
large canal-to-nail size ratios.
3. Postoperative monitoring is done weekly during distraction and monthly during consolidation.
4. Preoperatively plan each case with life-size drawings or computer graphic simulation. The Reverse Planning
Method is most useful in retrograde femoral applications.
5. Nail length is planned to allow 6–7 cm of the wider portion of the nail in the distracted segment at the completion
of distraction.
6. The canal must be vented before reaming.
7. Isthmic bone diameter must be able to accommodate reaming 2 mm greater than the nail diameter for flexible
reaming, and 0.5 mm for rigid reaming.
8. Shanz pins control segments and prevent rotational deformity, particularly in femoral lengthenings where the cor-
ticotomy is not well visualized through the wound.
9. Periosteum-sparring, non-heat-generating corticotomy should be positioned at or near apex of the deformity.
10. Comminution of the osteotomy should be avoided, as this creates nail instability, may prevent acute correction,
encourages lengthening deformity, and may lead to poor bone regenerate.
11. The nail should be advanced by hand pressure only in order to avoid damage of the distraction motor and gear
boxes.
manipulation of the osteotomy site or indirectly by bumps

and a surgical assistant. The corrected position of the osteot- Retrograde Femoral Lengthening
omy may be secured with a femoral distractor or sterile
bumps. The nail is passed across the osteotomy site correct- Patient Indications
ing any residual deformity. Rotation is optimally set by rotat-
ing the Shanz pins into their pre-osteotomy position, and the Retrograde femoral lengthening is indicated for skeletally
proximal interlocking screws are inserted via the targeting mature patients with the any of the following:
jig. The corticotomy is manually compressed. Proper align- 1. Where antegrade femoral lengthening will result in exces-
ment is checked and adjusted using an alignment grid, long sive axis deviation or translation
alignment rod, or Bovie cord. Distal locking screws are 2. Hip arthrodesis or deformity of the proximal femur pre-
inserted using the “perfect-circle” freehand technique. venting antegrade femoral nailing
Iliotibial band release is performed for lengthening of greater 3. Distal femoral deformity suitable for acute correction and
than 3 cm and in cases of congenital origin. retrograde nailing (Fig. 9.16a, b).
Fig. 9.16 (a) Hip arthrodesis

was performed after proximal
femoral osteosarcoma resection.
(b) A retrograde femoral
lengthening was the necessary
approach for the 6.5-cm limb
length discrepancy
142 M.T. Dahl
A retrograde femoral approach is also indicated in skele- Retrograde Femoral Surgical Technique
tally immature patients with premature arrest of the distal
femoral physis, provided canal size and bone length is suffi- A radiographic grid is placed under the patient, and the hip
cient for straight nail insertion. This technique requires entry position is centered over the grid marker. The orientation of
through and protection of the knee joint as described by the distal joint line, patella, and the anatomic and mechanical
Watson and others [26, 27]. femoral axes are marked with sterile skin markers. The cor-
ticotomy level, future nail position, and junction of the
telescopic portion of the nail are marked with skin clips to
Planning guide reaming depth and direction.
The femoral canal is vented through the future corticot-
Retrograde femoral lengthening is routinely planned with omy site by drilling multiple holes through a 10-mm lateral
the Reverse Planning Method as described by Baumgart [7]. incision. Two Shanz pins are placed, one distal and another
This method of planning can be performed with X-ray trac- proximal to the corticotomy site, in a lateral to medial direc-
ings of the preoperative full-length standing radiographs, or tion and rotationally parallel.
with commercially available software systems. In either The infrapatellar ligament is split longitudinally through
case, it is recommended that the surgeon reproduce the actual a 10 mm infrapatellar incision, and a 3 mm guide wire is
size of the entire limb using preoperative, intraoperative, and inserted to a point in the intercondylar notch anterior to the
completed correction X-rays for intraoperative use. PCL origin, and directed along the path that will allow for
reaming of the distal bone segment as planned (Figs. 9.17
and 9.18a–d).
Biplane fluoroscopy guidance for determining the exact
entry point avoids hyaline cartilage injury. The articular sur-
faces are protected from reamers and reamings with a protec-
tive sleeve so that insertion, removal and exchange of reamers
never touch the articular surfaces, and never actually enter
the joint.
Reaming just to the corticotomy level in 0.5-mm incre-
ments is performed. The corticotomy is then completed with
an osteotome at the venting site. Drilling six to eight 5-mm
holes should be adequate to avoid bone spikes at the corti-
cotomy since they may prevent acute deformity correction.
The osteotomy site is then angulated, translated, and/or
rotated to the preoperatively planned position and held there
by a femoral distractor or an assistant. Reaming of the diaph-
Fig. 9.17 The skin incision for retrograde femoral lengthening is
ysis is performed to 0.5 mm greater than the diameter of the
transverse or longitudinal, with a longitudinal incision through the nail. The nail is inserted with hand pressure, and alignment is
ligament checked with the grid. Additional reaming is performed if
Fig. 9.18 (a) A 3-mm guide wire is inserted to the center of the notch, just anterior to the PCL origin and posterior to the articular surface. (b–d)
Reaming sleeves are inserted, protecting the joint and ligament from reamings and reamer damage
Fig. 9.19 (a) Distal femoral physeal closure after fracture resulted in val- tion, and translational correction determined by Baumgart’s Reverse
gus and shortening. (b) Properative computer-assisted planning defines, Planning Method. (d) Mechanical axis and weight bearing line and length
weight bearing line lateral 12 mm, valgus mechanical distal lateral femoral predicted to be restored to normal after length achieved. (e) Full length
angle 79°, and axial discrepancy of 5 cm. (c) Retrograde nail size, posi- radiograph demonstrates ideal correction and achievement of goals
the nail does not pass easily. Adjustments in alignment are Planning
made if needed and locking and blocking screws are added.
This 14-year-old boy had premature asymmetric distal Tibial anatomic and mechanical axes are collinear in the
femoral physeal arrest of the right side, resulting in 9° of val- undeformed tibia so that planning for translation is unneces-
gus and 5 cm of shortening. Preoperative full-length standing sary. A modest tibial deformity can be acutely corrected to
X-ray with a lift under the short limb (b) computer-assisted allow passage of a telescopic nail, provided that the apex of
planning (c) corticotomy with intraoperative correction (d) the deformity (level of the correction) is amenable to stable
end of lengthening with neutral mechanical axis (e) end result fixation, and the surrounding neurovascular structures can
with ideal alignment and matching length (Fig. 9.19a–e). safely tolerate the acute correction. For example, proximal
tibial valgus deformities are at greater risk for peroneal nerve
palsy than are proximal tibial varus deformities. Blocking
Tibial Lengthening screws are inserted during canal preparation for corrections
in the proximal third of the tibia since valgus and procurva-
Patient Indications tum may develop during lengthening. Corrections in the
lower half of the tibia where the bone is wide may not have
Candidates for tibial lengthening with a telescopic nail are adequate distal purchase at the end of lengthening, and may
skeletally mature patients with a tibial length discrepancy of also require enhanced fixation with blocking screws. These
2–6 cm, modest deformity thought to be safely correctable features limit acute corrections to the region of the junction
acutely, and an open medullary canal with adequate diameter of the proximal and middle thirds of the tibia, and sometimes
and length to accommodate the nail. to the diaphysis.
144 M.T. Dahl
oriented parallel to the adjacent joint. Fixator assisted nailing

Tibial Surgical Technique techniques can be used [23]. Blocking screws should be liber-
ally used to prevent intraoperative deformity and prevent
Position the patient supine on a radiolucent table with the deformity from developing during lengthening (Fig. 9.20a–e).
image intensifier on the opposite side. Sterilely prepare the
entire limb, including the foot, leg, thigh, and hip. Perform a
fibular osteotomy at the junction of the middle and distal Intraoperative Nail Testing for All Limb
thirds through the internervous plane between peroneal and Lengthening Techniques
soleus muscles. Insert a 4.5-mm fully threaded cortical screw
distally from the fibula to the tibia with the foot in dorsiflex- All nails are tested intraoperatively, before closure, to ensure
ion. Proximal fibular attachment to the tibia can be accom- mechanical function and that subsequent distraction at the
plished in selected cases using the Rancho technique [32]. osteotomy site will occur postoperatively.
A prophylactic anterior compartment fasciotomy is per-
formed in patients at risk for developing a compartment syn-
drome, such as those with substantial deformity corrections, Post-operative Management
previous neurovascular compromise, or those anticipating
greater lengthening goals (greater than 3 cm). Perform a gas- Encourage isometric exercises and active range of motion of
trocsoleus recession if there is existing equinus contracture, adjacent joints throughout the lengthening process. Use a
or for lengthenings more than a few centimeters or with con- cam walker boot to support the foot and ankle in neutral
genital etiology. Further protection against equinus can be position in tibial lengthenings. Use a knee immobilizer to
achieved by static or dynamic dorsiflexion bracing or extraar- support full knee extension during femoral lengthenings.
ticular pinning as described by Herzenberg [33]. Restrict weight bearing to 30 lb pressure maximum until
Use an infrapatellar incision, and longitudinally split the three cortices are evident on X-rays (generally 3–4 months).
tendon or approach the tibia medial to the tendon. Obtain a Oral opiates should be adequate for pain control. Avoid non-
starting point in the center of the intraarticular area under steroidal anti-inflammatory drugs (NSAIDs) beyond the first
biplanar image intensifier control. few days postoperatively, as these agents can slow regener-
Choose the tibial corticotomy level at the deformity apex, ate bone formation. Consider 2–6 weeks of thromboembo-
at least 8 cm from the proximal joint line, thereby allowing lism prophylaxis appropriate to the patient’s risk factors.
room for both locking and blocking screws. Plan the nail Begin physical therapy, stressing ankle dorsiflexion and
length such that there will be 6–7 cm of the wider portion of knee extension for tibial lengthenings, and knee and hip
the nail in the distal segment after distraction is complete extension for femoral lengthening. Weekly postoperative
[33]. The corticotomy is performed through a 2–3 cm verti- physical therapy visits are advised to emphasize the need for
cal incision, with a tiny elevator lifting the medial and lateral continued therapy in preventing contractures in all patients,
periosteum. Multiple 5 mm drill holes will vent the canal. but most importantly in those with congenital etiologies
Shanz pins are placed rotationally parallel to each other, (Fig. 9.21).
yet parallel to the adjacent joints in the coronal plane. The Recommend calcium and vitamin D supplements, and a
tourniquet, if used, must be deflated before entering the canal diet high in protein and carbohydrates, and low in fat. The
and reaming. Ream in 0.5-mm increments with either rigid importance of the latency period must be considered [34].
or flexible reamers, and do so with soft tissue protection and Latency is commensurate with the patient’s age, health, bone
a flexed knee supported on a padded triangle. Posterior and being treated, bone quality, location of the corticotomy, and
medial blocking screws are placed at this time, guiding the magnitude of the acute correction performed. Generally, the
reamers along a path to prevent a valgus and procurvatum latency period is slightly longer in intraoperatively per-
deformity. formed corrections than with gradual corrections, 5–7 days
The corticotomy is completed with an osteotome, the in a child’s femur, and 7–10 days in a child’s tibia. Adults
knee is flexed, and the nail inserted by hand pressure. The require latency of 7–10 days in the femur and 10 days in the
proximal interlock jig will require removal to confirm align- tibia. Acute rotational and translational corrections require
ment, as the knee will not fully straighten with the jig in an additional 2 days of latency.
place. When satisfied with alignment, insert the proximal The rate and rhythm of distraction should begin at
interlocking bolts first. Manually compress the corticotomy. 0.25 mm every 6 h, and then adjusted based on weekly
“Perfect circle” free-hand insertion of the distal interlocks is assessments of the X-ray appearance of the bone regenerate
now completed. density, volume, contour, and consistency.
Deformity correction can be achieved with tibial Shanz Physical therapy sessions and principles previously out-
pins placed posterior proximally and distally, with each pin lined must be followed [35]. The weekly follow-up visits
Fig. 9.20 (a) Adolescent

Blount’s with 15° varus, 10°
procurvatum, 10° internal
rotation, and 3 cm of shortening.
(b, c) Acute interoperative varus,
rotation and procurvatum
correction with fixator assisted
nailing, then gradual lengthening.
(d, e) Deformity correction
maintained throughout
lengthening by proximal
posterior and lateral blocking
screws
during the distraction phase can be extended to monthly vis- meeting in 2013 [36]. The authors point out that certain com-
its during the consolidation phase. Patients must be informed plications of limb lengthening are intrinsic to any bone
to prevent contracture, improve motion, and not to exceed lengthening, and can occur regardless of the technique or
the 30 lb weight-bearing restriction during this phase. Full device used.
weight-bearing is not allowed until corticalization is Limb lengthening complications include:
radiographically evident on three of four cortices. Physical 1. Failures of preoperative assessment
therapy can be modified to strength and endurance training 2. Intraoperative complications
once three cortices are evident. Removal of the implant is 3. Incomplete corticotomy
performed 1 year after surgery, provided the regenerate bone 4. Iatrogenic deformity during insertion
is circumferentially corticallized. 5. Premature consolidation
6. Failures of bone formation
7. Device failure
Complications of Motorized Intramedullary 8. Joint contracture
Lengthening 9. Joint subluxation
10. Deformity during lengthening
Little has been written on the subject of complications of 11. Neurological consequences
motorized limb lengthening, as the technology is so new. 12. Muscular consequences
Green and Dahl presented a poster exhibit on the subject as 13. Psychological aspects
it relates to all methods of lengthening at the AAOS annual 14. Social/familial aspects
146 M.T. Dahl
Fig. 9.21 Proper therapy compliance allowed the patient illustrated in

Fig. 9.20 to maintain nearly full knee range during treatment
Fig. 9.23 Atrophic regenerate in a multiply operated femur requires a
delay and/or slower lengthening rate until the regenerate improves
Pin and wire site infection has been eliminated

altogether.
Premature consolidation is most likely to occur in the
young patient with femoral lengthening without acute defor-
mity correction. A clue to this occurrence in fixator length-
enings, extreme wire or pin site deflection on X-ray, is not
evident in intramedullary lengthenings. A failure of the cor-
ticotomy to continue separation at the rate expected signals
the clinician to increase the rate of distraction (Fig. 9.22).
Poor new bone formation seems to occur more commonly
with intramedullary lengthenings than with fixator lengthen-
ings, as reaming damages the marrow source of regenerate
bone, and acute corrections damage the periosteal source of
new bone. Atrophic bone formation should be corrected by
temporarily stopping the lengthening for 3–5 days, and then
resuming the lengthening at a slower rate until the regenerate
improves. Delayed healing, primarily in adults, can be
expected in 4 % and can be minimized by ideal corticotomy
technique, a longer latency period, weekly monitoring of
X-rays, and slowing the rate or temporarily stopping the
Fig. 9.22 Excessive bone formation in a patient with remote head lengthening in the face of poor regenerate. If the regenerate
trauma prevented distraction beyond 2 cm despite increasing the length- does not improve with these maneuvers, a reversal of the
ening rate to 1.5 mm/day lengthening can be done with PRECICE device. The penalty
for continued lengthening in the face of poor regenerate bone
15. Fracture after lengthening is a nonunion, which may require nail exchange and/or bone
16. Deformity after lengthening grafting (Fig. 9.23).
17. Stiffness following lengthening Fractures occurring through regenerate bone should no
18. Surgical site infection longer occur, as the nail protects the regenerate bone. Femur
Fig. 9.24 (a) This 14-year-old

female with congenital short
femur required additional
reaming, with resulting anterior
cortical thinning. (b) After
completing the lengthening a
fracture developed through the
proximal locking bolt at the area
of maximal cortical thinning on
the tension side of the femur
fracture at the tip of a lengthening nail can occur when the

bone is too small for the device (children), and excessive or
eccentric reaming has occurred to accommodate the nail
(Fig. 9.24a, b).
Deformity during distraction, a known complication of
fixator lengthening, is at risk to occur in intramedullary
lengthenings too. This occurs most commonly in the distal
femur or the proximal tibia where the wider metaphyseal
bone may allow angulation around an intramedullary nail
despite adjacent locking screws. Blocking screws should
be placed on the concave side of the anticipated deformity
of varus/procurvatum in the femur and valgus/procurvatum
in the tibia. Surgical site infection is rare, but deep infection
has been reported in a patient previously having under-
gone prolonged external fixation in the past (Figs. 9.25a, b
and 9.26).
Contracture or subluxation of the hip, knee, or ankle in
congenital cases can occur, just as in external fixator length-
enings. While doing intramedullary lengthenings in congeni-
tal etiology, protection of the knee joint by spanning is not
possible, requiring more strict maintenance of the ability to
fully extend the knee. The joint dislocation cascade of knee
flexion contracture, followed by knee translation, progress-
ing to posterior subluxation, and then to frank dislocation
must be prevented at the beginning. Prevention methods
require awareness, vigilance, and modest lengthening goals,
as these soft tissue complications are intrinsically related to
the underlying diagnosis and host condition. Physical ther-
apy directed at stretching, and joint specific static and
dynamic bracing are of value as in any lengthening [35]. The
ankle joint is protected from equinus deformity by static
splinting, stretching, and in certain congenital cases, an
Fig. 9.25 (a) Congenital short tibia of 3.5 cm with normal axial align- extraarticular screw, removed after completion of the
ment. (b) Valgus deformity developed during lengthening. Note the lengthening.
large space in the proximal metaphysis allowed the nail to migrate later-
ally during lengthening
148 M.T. Dahl
Summary
Critical to the success of a motorized internal lengthening

nail are combining proper surgical training, accurate preop-
erative planning, minimally invasive surgery, mechanical
integrity of the construct, and ideal control of the rate and
rhythm of distraction. Early designs that were mechanically
actuated had problems with rate control resulting in bone
formation complications. The two current designs use either
magnetic or electrical control, and have reliable use while
eliminating pin and wire complications and fixator-associated
pain. While internal lengthening has obvious advantages,
there are specific patient indications. Children with open
growth plates or small bones are not suitable candidates.
External fixation and associated hybrid techniques such as
lengthening over nail and plate-assisted lengthening con-
tinue to be necessary for certain cases. The correction of
severe foot and joint deformities will still require external
fixation for the foreseeable future. Improvements in instru-
mentation and expansion to other conditions will broaden
the safety and efficacy of these devices. The motorized intra-
Fig. 9.26 Blocking screws serve to narrow a capacious canal, in this medullary lengthening nail is an important new tool for the
example, correcting proximal tibial valgus limb length and deformity correction surgeon.
References
Outcomes
1. Codivilla A. On the means of lengthening, in the lower limbs, the
muscles and tissues which are shortened through deformity. J Bone
Early clinical case series of the FITBONE and PRECICE
Joint Surg. 1905;2(4):353–69.
devices report favorable outcomes and patient satisfaction, 2. Ilizarov GA. The tension-stress effect on the genesis and growth of
with relatively few implant problems [10, 11, 37]. High tissues. Part I. The influence of stability of fixation and soft-tissue
accuracy of distraction, maintenance of joint motion, favor- preservation. Clin Orthop Relat Res. 1989;238:249–81.
3. Ilizarov GA. The tension-stress effect on the genesis and growth of
able alignment, and fewer complications have been noted,
tissues: Part II. The influence of the rate and frequency of distrac-
compared with external fixation lengthenings [9]. tion. Clin Orthop Relat Res. 1989;239:263–85.
Patients report significantly lower levels of pain with 4. Antoci V, Ono CM, Antoci Jr V, Raney EM. Pin-tract infection dur-
motorized intramedullary lengtheners, compared with exter- ing limb lengthening using external fixation. Am J Orthop (Belle
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nal fixators [38]. Reports of mechanical device failure such
5. Dahl MT, Gulli B, Berg T. Complications of limb lengthening.
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(Roman Stauch, Intens, personal communication). Nail 6. Burghardt RD, Paley D, Specht SC, Herzenberg JE. The effect on
breakage of the PRECICE is reported to be 11/600 and is mechanical axis deviation of femoral lengthening with an intra-
medullary telescopic nail. J Bone Joint Surg Br.
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bearing compliance. Failure to distract is reported in 12/600 lower limb using a straight intramedullary nail with or without
with the PRECICE device. Transmitter, receiver, cable, or deformity correction. A new method. Oper Orthop Traumatol.
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motor failure of FITBONE is reported to be 16/2,000
8. Baumgart R, Betz A, Schweiberer L. A fully implantable motorized
(Personal communication, Bart Balkman 2014). intramedullary nail for limb lengthening and bone transport. Clin
Just as in Ilizarov methodology, proper surgeon educa- Orthop Relat Res. 1997;343:135–43.
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overemphasized.
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Part II
Related Concepts and Management Options
Biomechanically Based Clinical
Decision Making in Pediatric Foot 10
and Ankle Surgery
Jon R. Davids
and arthrodeses) utilized to correct these segmental malalign-

Introduction ments. Finally, a standardized approach for the preoperative,
intraoperative, postoperative, and surveillance of ankle and
The understanding and classification of functional deviations foot alignment and function will be presented.
and deficits at the ankle and foot are based upon an apprecia-
tion of normal function during the gait cycle [1–3]. The
interaction between the ankle, foot and the floor is a critical Ankle and Foot Function During Normal Gait
element of normal gait. Function of the ankle and foot is
determined by a complex interaction of anatomy, physiol- The understanding of ankle and foot function during normal
ogy, and physics. Proper ankle and foot alignment is required gait is facilitated by considering the lower leg to consist of
for optimal function of the knee and hip during gait. four segments: the tibial or shank segment, the hindfoot
Disruption of normal function of the ankle and foot may dis- (talus and calcaneus), the midfoot (navicular, cuneiforms,
rupt knee and hip function, compromising the energy effi- and cuboid), and the forefoot (metatarsals and phalanges)
ciency of gait and in extreme cases precluding the ability to [1–3, 5, 6] (Fig. 10.1a, b). It is also helpful to consider the
ambulate. foot to consist of two columns: the medial column (talus,
Clinical decision making for the management of ankle navicular, cuneiforms, great toe metatarsal, and phalanges),
and foot deformities in children can be standardized by the and the lateral column (calcaneus, cuboid, lesser toe metatar-
use of a diagnostic matrix (Table 10.1) [4]. This paradigm is sals, and phalanges) [7] (Fig. 10.2a–c). Standardized, consis-
based upon the collection and integration of data from five tent terminology is required to describe the alignment of the
sources: the clinical history, physical examination, plain separate segments of the ankle and foot [8]. Movement of the
radiographs, observational gait analysis, and in complex plantar aspect of the segment in question during the gait
cases associated with certain disease processes (e.g., cerebral cycle is described as inversion (towards the midline) or ever-
palsy, myelodysplasia, and hereditary sensorimotor neuropa- sion (away from the midline). Movement of the distal aspect
thies), quantitative gait analysis (which may include kine- of the segment in question during the gait cycle is described
matic/kinetic analyses, dynamic electromyography (EMG), as adduction (towards the midline) or abduction (away from
and dynamic pedobarography). the midline). Supination is a combination of inversion and
This chapter will begin with an overview of normal ankle adduction. Pronation is a combination of eversion and
and foot function during the gait cycle. This will provide a abduction. Rotation of the segment about its longitudinal
framework for the identification of common (or coupled), axis towards the midline is described as internal rotation.
and uncommon (or uncoupled), segmental malalignments of Rotation of the segment about its longitudinal axis away
the ankle and foot. This will be followed by an overview from the midline is described as external rotation.
(principles and indications) of the most common interven- The gait cycle is a period of time beginning with the ini-
tions (i.e., soft tissue surgery, guided growth, osteotomies, tial contact of the reference foot with the ground, continuing
through ipsilateral stance and swing phases until the subse-
quent ipsilateral initial contact. Stance phase occurs when
the reference limb is in contact with the ground. Swing phase
J.R. Davids, MD (*)
occurs when the reference limb is not in contact with the
Shriners Hospital for Children, 2425 Stockton Boulevard,
Sacramento, CA 95817, USA ground. The interaction of the ankle and foot with the ground
e-mail: [email protected] during the stance phase of the gait cycle is described by the

154 J.R. Davids
Table 10.1 The diagnostic matrix for the assessment of the ankle and foot deformities
Source Information
Clinical history Pain
Tripping
In-/Out-toeing
Physical examination Gross foot shape weight-bearing/non weight-bearing
Flexible/rigid
Plantar callous pattern
Radiographic examination Segmental alignment weight-bearing, AP and LAT views
Observational gait analysis Foot contact with floor (3 rockers)
Foot progression angle
Foot clearance in swing phase
Quantitative gait analysis Kinematics
Kinetics
Dynamic EMG
Pedobarography
Fig. 10.1 Three segments of the

foot. (a) Diagram of the
anteroposterior view of the right
foot. The hindfoot segment is
blue, the midfoot segment is red,
and the forefoot segment is
white. (b) Diagram of the lateral
view of the right foot. The
hindfoot segment is blue, the
midfoot segment is red, and the
forefoot segment is white
Fig. 10.2 Two columns of the

foot. (a) Diagram of the
anteroposterior view of the right
foot. The medial column is blue,
and the lateral column is red. (b)
Diagram of the lateral view
(medial side) of the right foot.
The medial column is blue. (c)
Diagram of the lateral view
(lateral side) of the right foot.
The lateral column is red
10 Biomechanically Based Clinical Decision Making in Pediatric Foot and Ankle Surgery 155
Fig. 10.3 Skeletal alignment of the lower extremity during the first during the first rocker of stance phase. (e) This “unlocks” the primary
rocker of stance phase. (a) The tibia is rotating internally (red arrow) joints of the midfoot (talo-navicular in solid circle, calcaneocuboid in
and the ankle is plantarflexing (blue arrow). (b) Hindfoot alignment dashed circle). (f) Coronal view of the articulation between the hind-
during the first rocker of stance phase consists of calcaneal eversion foot and midfoot in first rocker of stance phase. Lateral is to the left,
(red arrow). (c) Hindfoot alignment during the first rocker of stance medial is to the right. TNJ talo-navicular joint, CCJ calcaneocuboid
phase consists of calcaneal abduction (red arrow). (d) Hindfoot prona- joint, Calc calcaneus. The main axes of the TNJ and CCJ are parallel
tion (eversion and abduction) forces the talus to plantarflex (red arrow) (red lines), which allows motion and “unlocks” the midfoot
concept of three rockers [2, 3]. In normal gait, the heel is the rocker), and a rigid lever during terminal stance (third or
first part of the foot to contact the ground at initial contact. forefoot rocker) [2, 3]. During loading response, the tibial or
The ankle subsequently plantar flexes until the foot is flat on shank segment rotates internally, and the ankle is plantarflex-
the floor. This motion is controlled by the eccentric activity ing (Fig. 10.3a). This results in eversion and abduction of the
of the ankle dorsiflexor muscle group. The first, or heel hindfoot, primarily through the subtalar joint (see Fig. 10.3b,
rocker, occurs from heel strike to foot flat during the loading c). Pronation of the hindfoot forces the talus to plantarflex,
response subphase of stance. As the body progresses for- which “unlocks” the joints of the midfoot, which follows
ward, the tibia advances forward over the foot, which is into pronation (see Fig. 10.3d, e) This coupled movement of
achieved by ankle dorsiflexion. This motion is controlled by the hindfoot and midfoot results in maximum flexibility of
eccentric activity of the ankle plantar flexor muscle group. the foot, which allows the joints to contribute to shock
The second, or ankle rocker, occurs as the tibia advances absorption. During midstance, the tibial or shank segment is
over the foot during the midstance subphase of stance. rotating externally, and the ankle is dorsiflexing (Fig. 10.4a).
Immediately prior to the initial contact of the opposite foot, This results in inversion and adduction of the hindfoot, pri-
the heel of the reference foot rises off the ground and dorsi- marily through the subtalar joint (see Fig. 10.4b, c).
flexion occurs through the metatarsophalangeal joints of the Supination of the hindfoot forces the talus to dorsiflex, which
forefoot. This motion is controlled by concentric activity of “locks” the joints of the midfoot, which follows into
the ankle plantar flexor muscle group. The third, or forefoot supination (see Fig. 10.4d, e) This coupled movement of the
rocker, occurs as the ankle begins to plantar flex during the hindfoot and the midfoot results in restoration of the longitu-
terminal stance subphase of stance. This is an essential event dinal arch of the foot and maximum rigidity of the foot,
during normal gait, as the largest moment generated by any which enhances stability. During terminal stance, the tibial
single muscle group during the gait cycle is the internal plan- or shank segment continues to rotate externally, and the
tar flexion moment generated by the ankle plantar flexor ankle continues to dorsiflex. As the body progresses forward,
muscle group during third rocker in terminal stance [2, 3, 9]. the center of pressure beneath the foot advances distally into
In the stance phase of the normal gait cycle, the ankle and the forefoot. Because the segments of the foot are aligned to
foot provide shock absorption during loading response (first promote maximum rigidity, the forefoot is stable as it is
or heel rocker), stability during midstance (second or ankle loaded. The rigidity of the foot segments provides an optimal
156 J.R. Davids
Fig. 10.4 Skeletal alignment of the lower extremity during the second during the second rocker of stance phase. (e) This “locks” the primary
rocker of stance phase. (a) The tibia is rotating externally (red arrow) joints of the midfoot (talo-navicular in solid circle, calcaneo-cuboid in
and the ankle is dorsiflexing (blue arrow). (b) Hindfoot alignment dur- dashed circle). (f) Coronal view of the articulation between the hind-
ing the second rocker of stance phase consists of calcaneal inversion foot and midfoot in second rocker of stance phase. Lateral is to the left,
(red arrow). (c) Hindfoot alignment during the second rocker of stance medial is to the right. TNJ talo-navicular joint, CCJ calcaneocuboid
phase consists of calcaneal adduction (red arrow). (d) Hindfoot supina- joint, Calc calcaneus. The main axes of the TNJ and CCJ are no longer
tion (inversion and adduction) forces the talus to dorsiflex (red arrow) parallel (red lines), which restricts motion and “locks” the midfoot
lever arm to the ankle plantar flexor muscles during terminal

stance. Normal, expected segmental alignment patterns of Box 10.1
the ankle and foot during the stance phase of the gait cycle, • The lower leg consists of four segments: the tibial or
as described above, are the consequence of coupled move- shank segment, the hindfoot (talus and calcaneus),
ments between the anatomical segments. the midfoot (navicular, cuneiforms and cuboid), and
In the swing phase of the normal gait cycle, the foot and the forefoot (metatarsals and phalanges).
ankle contribute to clearance and pre-positioning for the sub- • The interaction of the ankle and foot with the
sequent stance phase [2, 3, 6]. During pre- and initial swing ground during the stance phase of the gait cycle is
the tibia or shank segment is rotating externally and the ankle described by the concept of three rockers; first or
is plantar flexing. The segments of the foot are “unlocked” as heel rocker, second or ankle rocker, and third or
the limb is unloaded. During mid swing the tibia or shank forefoot rocker.
segment is rotating internally and the ankle is dorsiflexing. • In the stance phase of the normal gait cycle, the
These coupled motions serve to functionally shorten the limb ankle and foot provide shock absorption during
and promote clearance. During terminal swing these coupled loading response (first or heel rocker), stability
motions continue and the foot is maintained in a plantigrade during midstance (second or ankle rocker), and a
alignment, perpendicular to the anatomical axis of the tibia rigid lever during terminal stance (third or forefoot
or shank segment. This pre-positioning of the foot during rocker).
terminal swing will result in a heel strike at the initial con- • In the swing phase of the normal gait cycle, the foot
tact, which is the optimal alignment for the ankle and foot as and ankle contribute to clearance and pre-position-
the extremity enters the subsequent stance phase in loading ing for the subsequent stance phase.
response.
of the midfoot and variable malalignment of the forefoot

Segmental Malalignment Patterns (Fig. 10.7a, b) The lateral column is functionally and/or
of the Ankle and Foot structurally longer than the medial column. Compensatory
ankle valgus deformity may be seen in association with equi-
Segmental malalignments of the ankle and foot may be cat- nocavovarus foot segmental malalignment.
egorized as coupled or uncoupled. Coupled segmental In all three coupled segmental malalignment patterns,
malalignments represent exaggerations of normal segmental heel strike at initial contact does not occur, disrupting the
alignments that occur during the gait cycle (as described first or hindfoot rocker and shock absorption function in
above). The three most common coupled segmental malalign- loading response. Equinus and equinocavovarus malalign-
ments are equinus, equinoplanovalgus, and equinocavovarus. ment patterns disrupt the second or ankle rocker by blocking
Equinus is characterized by excessive plantar flexion of the ankle dorsiflexion, compromising stability function in mid-
hindfoot relative to the ankle, with normal midfoot and fore- stance. Equinoplanovalgus malalignment maintains the mid-
foot alignment (Fig. 10.5a, b) Equinoplanovalgus is charac- and forefoot segments in an “unlocked” alignment,
terized by equinus deformity of the hindfoot, coupled with compromising stability function in midstance, which may
pronation deformities of the midfoot and forefoot (Fig. 10.6a, result in excessive loading of the plantar, medial portion of
b). The lateral column of the foot is functionally and/or struc- the midfoot. All three coupled segmental malalignments
turally shorter than the medial column. Ankle valgus and hal- may compromise the ability of the ankle plantar flexor mus-
lux valgus deformities are frequently seen in association with cles to generate an adequate internal plantar flexion moment
equinoplanovalgus foot segmental malalignment (see during third or forefoot rocker. The hindfoot malalignment
Fig. 10.6c, d) Equinocavovarus is characterized by equinus associated with equinus and equinocavovarus malalignment
deformity of the hindfoot, coupled with supination deformity patterns shortens the length of the plantar flexor muscles,
compromising their ability to generate tension, as described
by the length-tension curve for skeletal muscle [10, 11].
With equinoplanovalgus, the moment generating capacity of
the ankle plantarflexor muscles is further compromised by
the malalignment of mid-and forefoot segments, which
effectively shortens the lever arm available to this muscle
group during the third or forefoot rocker. All three segmental
malalignment patterns of the ankle and foot may inhibit
ankle dorsiflexion in swing phase, compromising clearance
in midswing and proper positioning of the foot and ankle in
terminal swing.
Uncoupled segmental malalignments are alignment pat-
terns between the hind-, mid-, and forefoot that never occur
during the gait cycle. Equinocavovalgus is an example of an
Fig. 10.5 Plain radiographs of the foot in a child with equinus defor-
mity. (a) Anteroposterior view shows normal segmental alignment. (b) uncoupled segmental malalignment pattern (Fig. 10.8a, b).
Lateral view shows hindfoot plantarflexion (diminished calcaneal pitch, Uncoupled segmental malalignments of the ankle and foot
indicated by solid arrow towards angle formed by solid lines, normal is are relatively uncommon, and are frequently the conse-
approximately 20°), with otherwise normal segmental alignment quence of deformity following previous surgery.
Fig. 10.6 Plain radiographs of the foot in a child with equinoplanovalgus deformity (increased tibiotalar angle, lateral wedging of the distal
gus deformity. (a) Anteroposterior view shows hindfoot pronation, talo- tibial epiphysis, and a high fibular station). (d) Clinical photograph of
navicular uncoverage, forefoot abduction and hallux valgus. (b) Lateral the left hindfoot in weight-bearing for this child. There is significant
view shows hindfoot plantarflextion, midfoot pronation (excessive hindfoot valgus deformity, which the radiographs show to be a conse-
naviculocuboid overlap), and forefoot pronation (excessive overlap of quence of both tibiotalar (ankle) and talo-calcaneal (subtalar) valgus
the metatarsals). (c) Anteroposterior view of the ankle shows ankle val- malalignments
158 J.R. Davids
Surgical Interventions
Interventions to correct foot deformities in children may be

selected to improve function and/or cosmesis. Both of these
goals may be achieved by surgeries designed to improve foot
shape. It is presumed that improved foot shape following soft
tissue and skeletal surgery can restore both the stability func-
tion of the foot during the second or ankle rocker in mid-
stance and the skeletal lever arm function of the foot during
Fig. 10.7 Plain radiographs of the foot in a child with equinocavovarus
deformity. (a) Anteroposterior view shows hindfoot supination (exces- the third or forefoot rocker in terminal stance [12–15].
sive overlap of the talus and calcaneus), and forefoot adduction (medial However, it is important to recognize that increased foot stiff-
deviation of the great toe metatarsal relative to the talus). (b) Lateral ness associated with many skeletal surgical procedures (e.g.,
view shows hindfoot varus (parallelism of the talus and calcaneus), arthrodesis) utilized to improve foot shape may compromise
midfoot supination (diminished naviculocuboid overlap), and forefoot
supination (excessive stacking of the metatarsals) shock absorption function of the foot during the first or ankle
rocker in loading response [16]. Cosmetic improvements fol-
lowing foot surgery are related to improved visual assess-
ment of static standing foot alignment (particularly restoration
of the medial longitudinal arch and toe alignment) and
improved foot progression angle during stance phase.
Soft tissue surgeries include release, lengthening, or
shortening of muscles, tendons, ligaments, and joint cap-
sules; or transfer of the muscle tendon unit. Release of soft
tissue structures is generally reserved for fixed ankle and
foot deformities associated with progressive disease pro-
cesses in subjects who have significant impairment and
whose goals are to improve static alignment in order to pro-
mote brace wear, shoe wear, or foot position in a wheelchair,
and to facilitate transfer level motor activities. Lengthening
of soft tissue structures is appropriate for fixed ankle and foot
deformities associated with static or stable disease processes
Fig. 10.8 Plain radiographs of the foot in a child with equinocavoval-
gus deformity, which is an example of an uncoupled malalignment. (a) in subjects whose goals are to improve alignment to facilitate
Anteroposterior view shows hindfoot pronation, talonavicular uncover- dynamic functional motor activities. It is important to recog-
age, forefoot abduction and hallux valgus interphalangeus. (b) Lateral nize that in most cases there is preexisting weakness of the
view shows hindfoot varus (parallelism of the talus and calcaneus),
muscle tendon unit that is being lengthened, and that all
midfoot supination (diminished naviculocuboid overlap), and forefoot
supination (excessive stacking of the metatarsals). The normal coupling lengthening surgical procedures result in additional weaken-
between the three segments of the foot is disrupted, as shown by the ing. When operating on muscles and tendons, selective surgi-
dissonance between anteroposterior and lateral radiographic views cal lengthening techniques that minimize the subsequent
weakness of the muscle tendon unit are therefore favored
[10, 11]. Surgical procedures that partially (also called
“split”) or completely transfer the muscle tendon unit are
Box 10.2 reserved for completely dynamic ankle and foot deformities
• Segmental malalignments of the ankle and foot may associated with static or stable disease processes in subjects
be categorized as coupled or uncoupled. Coupled who have relatively lower levels of motor impairment [17–
segmental malalignments represent exaggerations 19]. Partial and complete transfers are performed to address
of normal segmental alignments that occur during a dynamic muscle imbalance. Achieving perfect dynamic
the gait cycle. Uncoupled segmental malalignments balance with all types of tendon transfer can be challenging.
are alignment patterns between the three segments Over correction may occur with either partial or complete
of the foot that never occur during the gait cycle. transfers, and under correction may be seen following partial
• The three most common coupled segmental transfers. Proper patient selection is essential.
malalignments are equinus, equinoplanovalgus, Skeletal surgeries include guided growth, osteotomy, and
and equinocavovarus. arthrodesis. Guided growth can be utilized to correct ankle
valgus deformity and metatarsus/phalangeal deformity
Table 10.2 Levels of ankle and foot deformity and treatment options
Treatment options
Levels of deformity Pharmacologic/neurosurgery Muscle tendon surgeries Skeletal surgeries
I: Dynamic soft tissue imbalance, Neuromuscular junction blockade Partial or complete tendon transfers Usually not necessary
no skeletal deformities Selective dorsal rhizotomy
Intrathecal baclofen
II: Fixed soft tissue imbalance, no Not appropriate as isolated intervention Sequential lengthening Usually not necessary
fixed skeletal deformities (Myotendinous Junction Recession,
Tendon Lengthening)
III: Fixed soft tissue imbalance, Not appropriate as isolated intervention Sequential lengthening Osteotomy or arthrodesis
with structural skeletal deformities Appropriate in conjunction with (Lengthening, shortening,
skeletal surgery angular, rotational)
associated with juvenile hallux valgus [20–22]. Typically

two or more years of growth remaining is required to achieve
correction by guided growth strategies. Osteotomy and
arthrodesis techniques may correct deformity by addition
(i.e., lengthening), subtraction (i.e., shortening), angulation,
or rotation. Acute skeletal lengthening techniques are pre-
ferred as they utilize coupled segmental relations between
the segments of the foot to achieve correction [12, 23, 24].
These procedures require a bone graft, and in most cases
internal fixation. The use of allograft is favored over auto-
graft, though late allograft collapse during the re-ossification
phase of graft incorporation has been reported [25]. Acute
skeletal shortening procedures are used for the correction of
the most rigid foot deformities, which are usually associated
with congenital conditions (e.g., arthrogryposis) or periph-
eral neuropathies and myopathies (e.g., dystrophin deficient
muscular dystrophies).
Clinical decision-making for surgery is guided by the clas-
sification of foot deformities into three levels (Table 10.2).
Fig. 10.9 Intraoperative clinical photograph of the use of a “foot
Level I deformities are characterized by dynamic soft tissue pusher” to achieve simulated weight bearing lateral radiographs
imbalance. Skeletal anatomy is normal. Level II deformities
are characterized by fixed or myostatic soft tissue imbalance.
However, the underlying skeletal segmental malalignments
are flexible and correctable on manipulation. Level III defor-
mities are characterized by structural skeletal deformities that
are usually associated with fixed or myostatic soft tissue imbal- Box 10.3
ance. For foot deformities associated central nervous system • Soft tissue surgeries include release, lengthening, or
conditions (e.g., cerebral palsy) it is not always possible to shortening of muscles, tendons, ligaments, and joint
determine preoperatively if the deformity is level II or III. In capsules; or transfer of the muscle tendon unit.
such cases, sequential soft tissue lengthening is performed • Skeletal surgeries include guided growth, osteot-
first, followed by intraoperative assessment of segmental foot omy, and arthrodesis.
alignment with stress radiographs under fluoroscopy • Clinical decision-making for surgery is guided by
(Fig. 10.9). If correction of alignment is determined to be the classification of foot deformities into three
insufficient, then sequential skeletal surgery, focused on the levels
segment(s) that remain malaligned is performed.
160 J.R. Davids
required to confirm the diagnosis and for adequate planning

Assessment Tools and Indications prior to surgical management. True or simulated weight
bearing is essential; foot segmental alignment may be dra-
Clinical decision making for the management of ankle and matically different in loaded versus unloaded conditions, and
foot deformities in children integrates a range of data by the non-weight bearing views are of little value. Qualitative and
use of a diagnostic matrix (see Table 10.1) [4]. The integra- quantitative assessment of plain radiographs should be done
tion of data from multiple sources results in a degree of redun- is a systematic fashion, referring to normative data to objec-
dancy that improves decision making and quality of outcomes. tively describe the ankle and foot segmental alignment
When the data is consistent across the fields, and the problem (Table 10.4) [7, 26]. Accurate assessment of the causes of
is common, the confidence in decision making should be hindfoot alignment in the coronal plane (i.e., determining the
high. When the data is apparently inconsistent across the relative contributions of deformity at the tibiotalar and talo-
fields, or the problem is unusual, then the confidence in deci- calcaneal joints) requires analysis of the anteroposterior
sion making should be lowered. Surgical treatment paradigms radiograph of the ankle in addition to the views of the foot
for coupled segmental malalignments of the ankle and foot [27] (see Fig. 10.6) For the preoperative assessment of the
are more advanced and generally more effective than those valgus and varus foot, kinematics is limited by the reliance
for uncoupled segmental malalignments. The latter deformi- on a single segment foot model that can notaccount for
ties are individually unique, and therefore require careful, abnormal midfoot alignments [28, 29]. Dynamic electromy-
case-by-case surgical planning and treatment. ography (EMG) is only necessary for feet with dynamic
Different types of ankle and foot deformities are best varus deviations, to sort out the relative contributions of the
evaluated with different combinations of assessment tools at gastrocsoleus complex, the tibialis posterior, and tibialis
different points in the course of management (Table 10.3). anterior muscles [30, 31]. Pedobarography is indicated for
Plain radiographic views at all points include standing valgus and varus malalignments [32]. The relation between
anteroposterior and lateral views of the foot, and a standing static standing foot alignment (as indicated by plain radio-
anteroposterior view of the ankle. Additional views of the graphs) and dynamic foot loading (as indicated by pedo-
hindfoot (Cobey view) and subtalar joint (Harris heel view), barography) is complex [33, 34]. The former is not always a
while not part of the routine radiographic assessment para- good predictor of the latter, and when there is apparent dis-
digm, may be used to further assess for overall hindfoot crepancy between the two modalities, priority should be
alignment and the presence of a talocalcaneal coalition. given to the pedobarograph data as it is a closer measure
In the case of the latter, computed tomography scan (CT) is of actual function [32, 34]. Intraoperative assessment relies
Table 10.3 Assessment tools for the ankle/foot

Assessment tools
Physical
examination Standing/stress
History (ROM, OGA) radiographs Kinematics Kinetics EMG Pedobarography
Deformity Preoperative
Preoperative
Equinus X X X X
Valgus X X X Limited X X
Varus X X X Limited X X X
Intraoperative
Equinus X
Valgus X
Varus X
Postoperative
Equinus X X X
Valgus X X X X X
Varus X X X X X
Surveillance
Equinus X X
Valgus X X
Varus X X
ROM range of motion, OGA observational gait analysis, EMG electromyography, X appropriate tool to use
Table 10.4 Normal radiographic angle measurements [7, 26]

Ankle and foot segments and columns* Normal mean ±1 SD* Increased value (>Mean + 1 SD) Decreased value (<Mean − 1 SD)
Hindfoot
Tibiotalar Angle (anteroposterior view, degrees) 1.1 ± 3.75 Eversion Inversion
Calcaneal Pitch (lateral view, degrees) 17 ± 6.0 Calcaneus Equinus
Tibiocalcaneal Angle (lateral view, degrees) 69 ± 8.4 Equinus Calcaneus
Talocalcaneal Angle (lateral view, degrees) 49 ± 6.9 Eversion Inversion
Midfoot
Naviculocuboid Overlap 47 ± 13.8 Pronation Supination
(anteroposterior view, percentage)
Talonavicular Coverage Angle (anteroposterior 20 ± 9.8 Abduction Adduction
view, degrees)
Lateral Talo-First Metatarsal Angle 13 ± 7.5 Pronation Supination
(lateral view, degrees)
Forefoot
Anteroposterior Talo-First Metatarsal Angle 10 ± 7.0 Abduction Adduction
(anteroposterior view, degrees)
Metatarsal Stacking Angle (lateral view, degrees) 8.2 ± 9 Supination Pronation
Columns
Medial-Lateral Column Ratio (lateral view) 0.9 ± 0.1 Abduction Adduction
For direction on how to measure each angle or distance ratio on plain radiographs, see reference [7]
*
See figure 10.2
primarily on stress radiographs, which requires the use of a 3. Perry J. Gait analysis: normal and pathological function. Thorofare,
NJ: Slack Inc.; 1992.
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5. Inman VT. The human foot. Manit Med Rev. 1966;46(8):513–5.
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lance is guided by the history and physical examination, with 7. Davids JR, Gibson TW, Pugh LI. Quantitative segmental analysis
additional assessment tools utilized only if a problem has of weight-bearing radiographs of the foot and ankle for children:
normal alignment. J Pediatr Orthop. 2005;25(6):769–76.
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graphic study of skeletal deformities in treated clubfeet. Clin
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9. Gage JR. The clinical use of kinetics for evaluation of pathologic
• Clinical decision making for the management of gait in cerebral palsy. Instr Course Lect. 1995;44:507–15.
ankle and foot deformities in children integrates a 10. Delp SL, Statler K, Carroll NC. Preserving plantar flexion strength
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11. Firth GB, McMullan M, Chin T, Ma F, Selber P, Eizenberg N, et al.
• Coupled segmental malalignments are easier to treat Lengthening of the gastrocnemius-soleus complex: an anatomical
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Pediatric Joint Contractures
11
Christopher Iobst
Pathoanatomy
Introduction
Equinus contracture can be an unrecognized underlying
Joint contractures can be a debilitating cause of lower factor in many of the pathologies associated with the foot
extremity deformity. They can occur in isolation or in com- and ankle. In a normal gait pattern, the center of pressure on
bination with bony deformity. Mild joint contractures can the foot is approximately the second metatarsal-cuneiform
often be managed nonoperatively, while severe contractures joint [3]. The pull of the ankle plantarflexors, the gastrocne-
may require surgical interventions. All contractures can be mius and soleus muscles, counteracts the body’s anteriorly
frustrating to deal with, both for the patient and the physi- displaced center of pressure and prevents the body from fall-
cian, as they often recur in the growing child despite best ing forward in stance. In an equinus deformity, however, the
efforts. It is important to learn how to identify, evaluate, and foot’s center of pressure moves about 3 cm distally and 3 mm
manage joint contractures in pediatric patients. This chapter laterally to its normal location [3]. This abnormal loading of
reviews the current methods for evaluating and treating equi- the forefoot and midfoot in stance can lead to chronic
nus contractures, knee flexion contractures, and knee exten- mechanical overload and structural breakdown of the foot
sion contractures in children. and ankle. It has been demonstrated that patients with
metatarsalgia or related forefoot/midfoot symptoms had less
average maximal ankle dorsiflexion with the knee in exten-
Equinus Contracture sion than a control population without foot symptoms [2].
Equinus also interferes with gait by preventing the tibia’s
Equinus contracture is described as the inability to fully forward progression over the foot during stance phase, which
dorsiflex the ankle through the tibiotalar joint. Equinus shortens the stride length. Another abnormal gait finding
contracture interferes with gait by decreasing stability in may be hyperextension of the knees, which develop as a
stance phase, by removing the smooth transition of the compensatory mechanism to allow an equinus ankle to main-
body over the foot, and by causing inadequate clearance of tain a plantigrade foot position.
the foot in the swing phase [1]. Although the term equinus Equinus positioning of the foot has frequently been called
is frequently used, a working definition remains lacking. an Achilles contracture. However, this is a misnomer because
Digiovanni et al. have offered the following standardization the majority of the perceived stiffness or stretch occurs within
of terms to describe the inability to dorsiflex through the the muscle bellies themselves, not in the tendon [2]. The ten-
tibiotalar joint: gastrocnemius equinus represents maximal don is responsible for only about 3–5 % of this change in
ankle dorsiflexion of less than or equal to 5° with the knee position [2]. Therefore, although physicians often describe
in full extension, and Achilles tightness represents maximal “Achilles” tightness and address the Achilles tendon directly
ankle dorsiflexion of less than or equal to 10° with the knee to treat this disorder, the origin of the problem is often at the
in 90° of flexion [2]. level of the gastrocnemius and soleus muscles [2].
Equinus deformity can be divided into two main types,
muscular and osseous. Muscular equinus can be further
subdivided by identifying the primary cause, gastrocne-
C. Iobst, MD (*)
mius equinus or gastrocsoleus equinus. It is important not to
Department of Orthopedic Surgery, Nemours Children’s Hospital,
13535 Nemours Parkway, Orlando, FL 32827, USA overlook osseous deformity that can mimic soft tissue
e-mail: [email protected] contractures. For example, distal tibial procurvatum, forefoot

164 C. Iobst
equinus, and flattop talus can each mimic an ankle equinus with the knee extended, then there is gastrocnemius equinus.
contracture [4]. It is also possible to have a combination of On the other hand, if the ankle dorsiflexion is less than
muscular and osseous equinus. neutral with both the knee flexed and extended, then it can
either be gastroc-soleus equinus or osseous equinus. Osseous
equinus can be determined by identifying bony deformity
Clinical Evaluation such as distal tibial procurvatum or intra-articular exostoses
on a lateral radiograph of the ankle. Some foot deformities,
While obtaining the history, the physician should try to such as a cavus foot with forefoot plantarflexion, can also be
ascertain the underlying cause of the contracture (neuro- mistaken for an equinus deformity.
muscular disease, iatrogenic, congenital, etc.) as this can
influence the natural history and help the surgeon make
appropriate treatment recommendations. It is also important Imaging
to find out the extent to which the contracture affects the
child’s function and whether or not any previous treatment Radiographically, equinus can be evaluated on a weight-
has been attempted. bearing lateral radiograph in which the patient stands with
Proper examination of the foot and ankle for equinus the knee straight. The magnitude of the equinus can then be
requires a thorough understanding of the relevant anatomy. determined from the standing lateral radiograph by measur-
Equinus deformity may be caused by contracture of the gas- ing the tibial-sole angle [6]. This is calculated by drawing a
trocnemius alone or a contracture of both the gastrocnemius line along the sole of the foot from the plantar aspect of head
and the soleus. Because the gastrocnemius originates above of first metatarsal to plantar aspect of calcaneus and joining
the knee joint and the soleus does not, it is possible to dif- it with a line drawn along the long axis of the tibia (Fig. 11.2).
ferentiate the contribution of each muscle to the equinus The degree of equinus deformity can be categorized as mild
contracture by physical examination. The Silfverskiold test (less than 20° plantarflexion), moderate (20°–40°) and severe
evaluates maximal ankle dorsiflexion with the knee in full (greater than 40°) [7].
extension and then again with the knee in 90° of flexion which
relaxes the gastrocnemius muscle (Fig. 11.1) [5]. It is critical
for both components of this test, that the examiner inverts the Management Strategy and Decision Making
subtalar joint and supinates the forefoot, prior to dorsiflexing
the ankle. This locks the talonavicular joint and neutralizes Nonoperative
the hindfoot, preventing any motion through the midfoot, Most mild and some moderate equinus will respond to
which could be misconstrued as ankle dorsiflexion. If the physical therapy with stretching, casting and bracing.
ankle joint dorsiflexes past neutral equally well with both Because early age at first surgery in children with cerebral
the knee extended and flexed, there is usually no equinus palsy is a major risk factor for recurrent equinus deformity,
contracture present. However, if the ankle joint dorsiflexes temporizing measures to postpone the first surgery should be
with the knee flexed but does not reach the same dorsiflexion considered in younger children [8, 9]. Options include the
Fig. 11.1 The Silfverskiold test is used to determine the contribution flexed than with the knee extended, a gastrocnemius contracture is pres-
of the gastrocnemius to the overall equinus contracture. The foot is dor- ent. The test should be performed with the forefoot supinated and the
siflexed with the knee extended and then again with the knee flexed to sub-talar joint inverted to lock the midfoot joints
90°. If there is more dorsiflexion of the ankle possible with the knee
11 Pediatric Joint Contractures 165
Fig. 11.2 The magnitude of the equinus can be measured on a lateral

radiograph of the ankle by measuring the tibial-sole angle. Equinus
deformity will have a measurement of greater than 90°
use of botulinum toxin A, casting and orthoses. Serial casting

should be performed with the patient in a prone position to Fig. 11.3 Zone 1 level surgeries include the Baumann procedure and
obtain the most effective correction [10]. Botulinum toxin A the Strayer procedure. Illustration created by and reprinted with permis-
has had successful short-term outcomes and may allow sion of Daniel Biggers
surgery to be delayed until after the age of 6 years in chil-
dren with cerebral palsy [9]. Planning of surgical proce- stance phase, but also preserve adequate strength and
dures can be based on the results of selective botulinum moment generation to control advancement of the tibia over
toxin A injections. Potential problems include the develop- the foot [11]. The most devastating complication of surgery
ment of antibodies to botulinum toxin A and the cost of the for equinus deformity is excessive weakness of plantarflex-
treatment. Botulinum toxin A should only be used in mus- ion with a calcaneus gait. Surgery for recurrent equinus is
cles that produce dynamic deformities in the absence of possible but there are currently no reliable methods with
fixed contracture. which to shorten an over-lengthened gastrocnemius–soleus
complex [11]. Therefore it is better to err on the side of
Operative undercorrection especially in patients with cerebral palsy
Lengthening of Muscles and Tendons and follow the philosophy “a little equinus is better than cal-
If the patient demonstrates an equinus deformity with gait caneus” [11]. Overcorrection may result in a severe crouch
but the ankle has sufficient dorsiflexion past neutral on pas- gait and the loss of walking ability [11].
sive range of motion examination, the equinus is dynamic, Operations designed to lengthen the triceps surae can be
and surgical treatment may not be needed. However, if the assigned to one of three discrete anatomical zones. Zone 1
child has failed conservative management or demonstrates starts at the gastrocnemius origin and ends at the most distal
tripping, falling, or forefoot pain with functional impair- fibers of the medial belly of the gastrocnemius [9]. The oper-
ment, then surgery should be considered [11]. The presence ative procedures in Zone 1 include the Baumann and the
of a fixed equinus contracture at the ankle, with dorsiflexion Strayer distal gastrocnemius recession (Fig. 11.3). The pro-
to less than neutral is also an indication to operate. An impor- cedure described by Baumann involves intramuscular length-
tant principle governing surgery for equinus is to use the ening of the gastrocnemius and soleus performed in the
most conservative gastrocnemius lengthening procedure interval between the gastrocnemius and soleus fascia [13].
possible to achieve approximately 5° of dorsiflexion with the The gastrocnemius may be lengthened selectively, or differ-
knee extended [11]. The amount of lengthening required for ential lengthening of the gastrocnemius and soleus may be
a specific degree of fixed equinus is often much less than performed by varying the number of cuts in the fascia
surgeons estimate. The appropriate surgical treatment for overlying the two muscles. Herzenberg et al. found it more
equinus deformity must not only achieve adequate length of effective to make a second more distal incision in the gastroc-
the gastrocnemius–soleus complex to allow dorsiflexion in nemius muscle rather than adding an incision in the soleus [14].
166 C. Iobst
Fig. 11.4 Zone 2 level surgeries include the Vulpius and the Baker Fig. 11.5 Zone 3 level surgeries include the White and Hoke proce-
procedures. Illustration created by and reprinted with permission of dures. Illustration created by and reprinted with permission of Daniel
Daniel Biggers Biggers
At the junction of Zones 1 and 2 it is possible to perform Zone 3 extends from the end of the soleus muscle to the
selective lengthening of the gastrocnemius alone, as insertion of the Achilles tendon on the calcaneus [9]. The
described by Strayer [15]. It is also possible to perform dif- gastrocnemius aponeurosis and the soleus fascia pro-
ferential lengthening of the gastrocnemius and soleus by vide two groups of fibers that are distinct at first, then
separating the gastrocnemius fascia from the soleus fascia rotate, and finally blend together within the substance of
and dividing the gastrocnemius aponeurosis and soleus fas- the Achilles tendon [12]. Zone 3 procedures include all
cia separately in a technique known as the modified Strayer forms of lengthening of the Achilles tendon, as well as heel
procedure [11]. cord advancement (Fig. 11.5). Zone 3 lengthening of the
Zone 2 extends from the distal end of the medial belly of Achilles tendon should be used when there is a contracture
the gastrocnemius muscle to the end of the soleus muscle [9]. of both the gastrocnemius and the soleus of greater than 30°
This zone is characterized by condensation of the gastrocne- and no improvement in ankle dorsiflexion is seen with knee
mius fascia into a broad aponeurosis, which lies superficial flexion [11].
to the soleus fascia. The layers are most accurately referred to Achilles tendon lengthening may be performed by open
as the conjoined gastrocnemius aponeurosis–soleus fascia [12]. or percutaneous techniques. In the open technique, a longitu-
The two Zone 2 layers can very rarely be separated and then dinal incision is made just lateral to the Achilles tendon. The
only partially and by sharp rather than blunt dissection [12]. tendon is lengthened in Z-fashion and repaired with heavy
Zone 2 procedures include those of Vulpius and Baker and non-absorbable suture. Percutaneous lengthening can be per-
involve performing a gastrocnemius–soleus complex reces- formed by either two or three transverse partial tenotomies.
sion with the use of a single transverse division of both the The first tenotomy is made proximal to the calcaneal inser-
gastrocnemius aponeurosis and the soleus fascia (Fig. 11.4). tion and a little more than half of the tendon is divided by
Neither selective nor differential lengthening of the two inserting a scalpel longitudinally into the center of the ten-
layers that comprise the conjoined tendon is possible in don, turning the blade out toward the edge of the tendon, and
Zone 2 with these procedures [12]. The Zone 2 procedures pushing the tendon down onto the blade. The opposite half of
are neither anatomically nor biomechanically equivalent to the tendon is then divided by using a similar maneuver
the Zone 1 procedures [12]. The Vulpius procedure is often 3–4 cm more proximally. In a patient with a valgus hindfoot,
grouped with the Strayer procedure and referred to as a gas- the distal cut is through the lateral half of the tendon. In a
trocnemius recession, when it is in fact a gastrocnemius– varus hindfoot, the distal incision is through the medial half.
soleus complex recession [12]. After completing the incisions, the ankle should be dorsiflexed
gradually with the heel inverted. The foot should be dorsiflexed The stability of the gastrocnemius–soleus musculotendinous
to neutral. It is important to verify the integrity of the ten- unit after surgical lengthening may also be relevant to the
don after a percutaneous lengthening is performed. The sur- decisions about postoperative care and rehabilitation proto-
geon should squeeze the calf and simultaneously watch the cols [12]. The intrinsic stability after Zone 1 or 2 lengthening
ankle. If the ankle plantarflexes when the calf is squeezed, may permit very early weight-bearing and relatively short
then the tendon can be presumed to be intact within the periods of cast immobilization. The relative instability after
tendon sheath. If there is no plantar flexion, the tendon may Zone 3 lengthening and the documented tendency for contin-
have been ruptured by the lengthening procedure and an ued lengthening under small loads may require longer immo-
open repair will be needed. bilization and delayed weight-bearing [12].
The stability of the musculotendinous unit after a particu- A typical postoperative regimen after equinus surgery
lar lengthening procedure as well as the amount of lengthen- consists of a below-the-knee cast with the foot in a neutral
ing achieved are directly related to the residual cross-sectional dorsiflexion for 3 weeks. Unrestricted weight bearing begin-
area of the soft tissues remaining intact at the site of the ning the first postoperative day is prescribed if a Zone 1 or
lengthening [12]. For example, after a Baumann intramuscu- Zone 2 procedure was performed. Casts are removed after 3
lar tenotomy, all of the soleus and gastrocnemius muscle weeks to check healing and to cast molds for an ankle–foot
fibers, plus the intact fascia on the posterior aspect of the orthosis. The standard postoperative ankle–foot orthosis is
gastrocnemius and the anterior aspect of the soleus remain solid with hinges inserted but not activated [11]. Casts are
intact. This confers excellent stability and resists further reapplied and removed at 6 weeks after surgery where they
lengthening of the musculotendinous unit [12]. The Baumann are replaced by the ankle–foot orthoses.
procedure appears to yield good results in children with The greatest risk after equinus surgery is recurrence and it
spastic diplegia and mild equinus deformities with no correlates strongly with the age of the patient at surgery.
reported overcorrections [16]. Zone 2 procedures achieve Children with cerebral palsy who undergo heel cord length-
greater lengthening than Zone 1 procedures [12]. This may ening at 7 years or younger are particularly at risk for recur-
be related to the decreasing cross-sectional area of muscle at rence [8]. Recurrence of equinus is also possible in cases of
the site of the fascial division. In Zone 3, the stability of the congenital disease, such as arthrogryposis, or with the loss of
musculotendinous unit after a percutaneous triple hemi- function of the anterior compartment muscle(s), as in severe
section depends entirely on side-to-side links between the burn cases, poliomyelitis, or nerve injury [6]. Recurrence in
largely parallel collagen fibers of the tendon, which have posttraumatic equinus is rare as long as the anterior tibial
been divided at three contiguous levels [12]. This may con- muscles are functional. Recurrent equinus can be corrected
tribute to overlengthening and poor outcomes, especially in with a Vulpius gastrocnemius–soleus complex recession in
ambulatory children with spastic diplegia [12]. Zone 2 [12]. In some cases of repeated lengthening the
Therefore, there is no single surgical procedure that Achilles tendon becomes scarred and adherent, so the subse-
should be considered a panacea for all patients with equinus quent lengthenings must be done using the open technique.
deformity. Rather, the history and physical examination will When performing a lengthening the surgeon must be
help guide the surgeon to decide which procedure may be careful not to position the foot in excessive dorsiflexion.
more appropriate for each clinical situation. For example, in Overstretching of the tendon can lead to excessive length and
children with spastic hemiplegia and equinus deformity, the calcaneus gait. Calcaneus can also occur after lengthening if
Silfverskiold test usually reveals a substantial fixed contrac- there is an unrecognized flexion contracture of the knee. If the
ture of both the gastrocnemius and the soleus [12]. A Zone knee remains crouched and the Achilles tendon becomes
2 lengthening (Vulpius) or a Zone 3 procedure (percutane- longer, the ankle will drop into excessive dorsiflexion during
ous hemi-section) might be selected, depending on the stance phase and create an inefficient gait pattern.
severity [12]. In contrast, a Zone 3 procedure in patients
with spastic diplegia is associated with a very high preva- External Fixation
lence of calcaneus deformity and crouch gait at long-term While mild to moderately severe equinus contracture can be
follow-up [12]. In these patients, the Silfverskiold test usu- addressed with soft tissue lengthening, severe equinus defor-
ally reveals either an isolated contracture of the gastrocne- mities often cannot be corrected with a single open surgical
mius or a contracture of the gastrocnemius that is greater procedure because the skin and neurovascular structures
than that of the soleus [12]. Therefore, a Zone 1 procedure may not tolerate such an acute stretch. The Ilizarov method of
such as the Baumann or Strayer would be recommended. gradual correction can be used in these situations. The risk
The type of cerebral palsy should be carefully considered of a neurovascular stretch injury during treatment can be
when deciding on the type of surgical procedure and overall further reduced by decompressing the posterior tibial neuro-
management of an equinus deformity [9]. vascular bundle at the tarsal tunnel during frame application,
168 C. Iobst
especially in the presence of any associated varus deformity

[4]. If any neurologic compromise occurs during the gradual
distraction, the rate of the distraction can be slowed or tem-
porarily stopped to allow for neurological recovery. Nerve
decompression should be considered when nerve function
does not improve or if any new motor deficit is noted [4].
In addition to providing a method for correction of
severe contractures, external fixation has several other
advantages. During external fixation treatment there will be
access to the soft tissues for wound care, especially in the
presence of abnormal soft tissues such as scars, burns, and
compromised skin [4]. External fixation also allows for
adjusting the rate of deformity correction during the post-
operative period and can allow for joint range of motion
and early weight bearing [4].
All ankle external fixator constructs need to allow for dis-
traction of the ankle joint space prior to beginning the defor-
mity correction. This should be performed at the time of
frame placement. In a simple equinus correction, the ankle
should be distracted 2–5 mm compared with preoperative
radiographs [6]. This limits cartilage compression of the Fig. 11.6 An example of a constrained external fixator with hinges
ankle joint and any rockerbottom deformity that may occur placed at the center of rotation of the ankle. Illustration created by and
as the ankle is brought into dorsiflexion [6]. The distraction reprinted with permission of Daniel Biggers
of the hindfoot, however, must be done in a posterior-inclined
direction. If distraction is performed in a purely axial direc-
tion, parallel to the tibia, the talus will tend to sublux anteri-
orly [6]. Fixation should be applied to both the talus and the
calcaneus to prevent overdistraction of the subtalar joint dur-
ing the correction of the tibiotalar joint contracture [4].
Types of External Fixation Constructs

There are three different types of external fixator constructs
that may be used to correct equinus contractures depending
on the hinge system used: constrained, unconstrained, and
virtual. The constrained fixator has a single hinge centered
on the axis of rotation of the ankle (Fig. 11.6). The normal
trans-malleolar axis is 80° in the coronal plane and 84° in the
sagittal plane [6]. Therefore, the axis created by the hinges is
positioned on a slightly oblique plane with respect to the
horizontal from the tip of the lateral malleolus to just distal
to the tip of the medial malleolus [6]. Anterior subluxation of
the talus can be avoided by carefully positioning the hinges
slightly inferior to the axis of the ankle by 4–5 mm [6]. This
produces a counter-push on the foot during correction and
directly opposes anterior subluxation of the talus [6]. The
constrained fixator allows for range of motion of the joint Fig. 11.7 An example of an unconstrained external fixator for correction
of an equinus deformity
during contracture correction.
An unconstrained or flexible frame can be used for simple,
unidirectional deformities with no associated bony deformities ring [6]. The use of a footplate or connecting bars between the
[6] (Fig. 11.7). Frames for the correction of a simple equinus calcaneal and metatarsal half rings is usually not needed for
contracture require much less rigidity than those for bony simple equinus correction. A single olive wire is placed in the
instability. Consequently, this construct consists of only a calcaneus and in the metatarsals and attached to the respec-
tibial ring block, a calcaneal half ring, and a metatarsal half tive half ring. The calcaneal wire should be placed relatively
be maintained with a cast for approximately 4 weeks after

frame removal and with functional bracing thereafter.
However, once the Ilizarov fixator is removed the defor-
mity may recur, especially as the child grows [17].
Increasing the duration of time in the fixator does not
seem to prevent recurrence [17]. Any motor imbalance
should be identified and addressed with tendon transfer or
bracing to try to prevent recurrence. The parents and
patients should be made aware that additional procedures
are most likely going to be necessary to maintain the foot
in a plantigrade position as the child gets older.
Box 11.1. Equinus Deformity

• Recurrence is common after equinus surgery espe-
cially in children under age 7 years.
• In an external fixation construct for equinus correc-
Fig. 11.8 An example of a Taylor Spatial Frame™ used for correction tion, distraction must be performed first to prevent
of an equinus deformity. The lateral process of the talus is chosen as the impaction of the tibia and talus.
origin. Illustration created by and reprinted with permission of Daniel
• Watch for anterior subluxation of the talus during
Biggers
correction of equinus with an external fixator.
• The surgeon should choose the appropriate zone for
proximally and posteriorly to prevent wire cutout and to soft tissue lengthening based on the physical exami-
increase its biomechanical advantage [6]. Instead of building nation, the underlying diagnosis, and the amount of
a hinge at the center of rotation like the constrained frame, equinus contracture present.
the unconstrained frame allows rotation to occur naturally
at the tibiotalar joint as the correction progresses.
The Taylor Spatial Frame™ (Memphis, TN) allows the
surgeon to correct the equinus deformity around a virtual
hinge (Fig. 11.8). The lateral process of the talus is generally Knee Flexion Contracture
chosen as the “origin.” In severe contractures, the tibial ring
can be mounted obliquely to create a larger space posteriorly Etiology
for the struts to fit. The tibiotalar joint can be distracted
acutely with the frame in the operating room or the distrac- Knee flexion contractures commonly develop in children
tion can be built in to the deformity program. It is also pos- with neuromuscular disease such as cerebral palsy. They are
sible to prevent anterior subluxation of the talus by accounting also associated with congenital deformities of the lower
for it during data input in the deformity software program. extremities such as congenital femoral deficiency, tibial
A typical comprehensive spatial frame deformity program hemimelia, congenital dislocation of the patella, arthrogry-
will define the deformity as plantar flexed (amount of equi- posis, popliteal pterygium syndrome, sacral agenesis, and
nus to correct), short 5–10 mm (to distract the tibiotalar certain skeletal dysplasias [18]. Knee flexion contractures
joint), anteriorly translated (to prevent anterior talar sublux- are considered to be more disabling than knee extension con-
ation), and slightly internally rotated (the ankle naturally tractures [19]. As little as 5° of extension loss can create a
externally rotates as it dorsiflexes). noticeable limp, strain the quadriceps muscle and lead to
Postoperatively, deformity correction with the external patellofemoral pain [20]. Murray and Fixsen found that only
fixator is started as soon as the patient can tolerate it com- 46 % of their patients with arthrogryposis and knee flexion
fortably. Weekly or biweekly postoperative radiographs contractures became community ambulators whereas 89 %
should be taken to follow the deformity correction and to of the children with extension contractures did [21]. Unlike
ensure that the ankle remains distracted 2–5 mm without hip and ankle contractures, the other lower extremity joints
any subluxation [6]. The equinus deformity should be or the lumbar spine cannot compensate for knee flexion con-
overcorrected by 10°–15° if possible and held with the tractures [22]. Hoffer et al. found that a knee flexion contrac-
fixator for a minimum of 4–6 weeks further to allow for ture of greater than 20° was incompatible with functional
soft tissue adaptation [6]. The corrected position should ambulation [23].
170 C. Iobst
Fig. 11.9 Examination of the knee should evaluate maximum knee extension and maximum knee flexion
Biomechanics contracture of the knee with a soft endpoint will likely have
an improvement from soft tissue procedures [22]. The stabil-
In children the hinge point of the knee is defined as the ity of the knee should be tested and the function of the quad-
intersection of the posterior femoral cortex and the physis or riceps evaluated. One relative contraindication to surgical
physeal scar [24]. Normally, the mechanical axis of the limb treatment is the absence of active quadriceps function
will pass slightly anterior to the hinge point of the knee because of the high likelihood of recurrence of the flexion
allowing the knee to lock when the lower limb is fully deformity [18, 26]. The flexibility of the hamstring muscles
extended [24]. This permits the quadriceps muscle to rest must be assessed and can be measured via the popliteal
during prolonged standing [24]. In a knee flexion contrac- angle. This angle is created first by positioning the patient
ture, however, the center of gravity falls posterior to the knee. supine on the examining table, flexing the hip to 90° and then
This arrangement places increasing demand on the quadriceps maximally extending the ipsilateral flexed knee. The acute
to resist the progressive crouch and energy expenditure angle between the vertical and maximal extension of the
increases during a crouched gait. This constant activation of tibia is the popliteal angle. There is variability in measured
the quadriceps muscle will result in fatigue and increases the popliteal angles with a mean value of 26° in normal children
oxygen cost during such pathologic gait [25]. Knee flexion 4 years and older [18]. The examining physician must care-
contracture also functionally shortens the limb creating a gait fully assess all of the lower extremity joints for strength,
disturbance. Consequently, a fixed knee flexion deformity spasticity and contracture. While tight hamstrings are gener-
exceeding 10° can lead to anterior knee pain, decreased ally the cause of a crouch knee gait, quadriceps and gastroc-
endurance, patella alta, and progressive crouch gait in soleus weakness may also lead to excessive knee flexion in
ambulatory patients [25]. In wheelchair users, knee flexion stance phase [18]. A bent-knee gait may also be a compensa-
contracture may impair standing, transfers, and activities of tion for equinus and toe-walking if the popliteal angle is nor-
daily living. mal [18]. The hip must also be examined because correction
of hamstring contractures without addressing concomitant
hip flexion contractures will lead to increased hip flexion and
Clinical Evaluation forward lean during gait [18]. Furthermore, distal femoral or
proximal tibial deformities may create or exaggerate a knee
The physical examination for knee flexion contracture should flexion deformity. In patients with skeletal dysplasias, for
focus on observation of the gait pattern from both the frontal example, the knee flexion deformity may be the result of
and sagittal plane. A typical knee flexion contracture gait bony or articular cartilage abnormalities rather than any soft
pattern will include greater than normal knee flexion during tissue tightness [18]. Patients with congenital knee flexion
midstance and an inability to extend the knee fully at the contractures that have never reached full extension position-
end of swing phase continuing into initial contact [18]. ing may develop compensatory deformities of the distal
Walking will also exhibit decreasing cadence and step length. femur and proximal tibia [19]. Syndromic and congenital
A focused examination of the knee should include an assess- contractures such as arthrogryposis are also occasionally
ment of the maximal knee extension, maximal knee flexion associated with lateral displacement of the extensor mecha-
and the total arc of motion present (Fig. 11.9). As the range nism and the mechanical axis [26]. Each particular problem
of motion is evaluated, it is important to determine if there is needs to be recognized and addressed at the time of surgical
firm or soft endpoint to maximal knee extension. A flexion correction in order to prevent recurrence.
defined as a line connecting the center of the femoral head to

the center of the ankle. In a normal situation the mechanical
axis should pass slightly anterior to the hinge point of the
knee. If the lower extremity has fixed flexion deformity at
the knee the mechanical axis will pass posterior to the hinge
point of the knee. The anterior cortical lines of the distal
femur and proximal tibia should also be measured [24]
(Fig. 11.11). These lines represent the overall position of the
knee joint and will determine if the knee joint is in a neutral
position or a fixed flexion position. In a normal limb the
anterior cortical lines of the femur and tibia should be col-
linear [24]. If these lines are not collinear, then the angula-
tion between the anterior cortical lines of the distal femur
and proximal tibia should be measured to quantify the total
amount of flexion deformity. On the lateral radiographs, the
distal femoral and proximal tibial joint orientation lines as
measured by the posterior distal femoral and posterior
proximal tibial angles should be drawn [24]. This analysis
helps determine if a deformity is entirely the result of a con-
tracture or if it has a bony component. The comparison of the
amount of deformity determined by the joint analysis and
the amount of deformity denoted by the anterior cortical line
measurement allows the soft tissue component of the defor-
mity to be determined. If the degree of deformity measured
Fig. 11.10 Lateral radiograph of the lower extremity demonstrating a by the anterior cortical lines is not equal to the degree of
knee flexion contracture. The image was taken with the knee in maxi- deformity determined by the joint analysis, then a soft tissue
mum extension
component is present [24]. Multiple combinations of bone
deformity and soft tissue contribution can result in the same
Imaging clinical deformity.
The radiographic evaluation of a knee flexion contracture

requires modification of the usual anteroposterior radio- Decision Making and Management Strategy
graphs of the femur, tibia, or knee. Because of the flexed
position of the knee these views can be difficult to obtain. Decision Making
Similarly, long leg anteroposterior films are rendered mean- Knee flexion contractures can occur in combination with
ingless by the flexed knee [22]. Obtaining individual tibia other lower extremity deformities. In limbs with a knee con-
and femur posteroanterior radiographs with the child in the tracture and a coronal plane deformity of the femur or tibia it
prone position is another method to overcome such obstacles can be difficult to differentiate the contribution of each to the
in positioning the extremity for radiography [22]. Lateral total limb malalignment [22]. As noted earlier, flexion con-
radiographs with the knee in maximum flexion and extension tracture of the knee prevents proper positioning of the limb
may help define the knee’s range of motion and demonstrate for radiographic analysis making it challenging to accurately
any tendency towards tibial hinging or subluxation with measure the magnitude and location of the bone deformity.
changes in the arc of motion [22]. In long-standing knee flex- In these cases, it is easier to correct the knee flexion contrac-
ion contractures with reduced pressure and motion at the ture first and then, once the knee is straight, corrective oste-
joint, the lateral radiograph will help identify any compensa- otomies can be accurately planned [22]. Hip deformities and
tory changes of the proximal tibia. The resulting dome contractures create additional obstacles to correcting knee
shaped epiphysis can cause posterior tibial subluxation dur- contractures, maintaining correction, sitting, and ultimately
ing deformity correction [19]. ambulating after the correction. Hip flexion contractures
The most meaningful way to evaluate the lower extremity greater than 30°, extension contractures, and rotational
sagittal plane alignment is with a full-length standing lateral abnormalities that prohibit positioning the knee directly
view radiograph of the lower limb with the knee joint maxi- anteriorly need to be addressed prior to knee contracture cor-
mally extended [24] (Fig. 11.10). This view allows evalua- rection [22]. If the knee is treated first, it will be difficult to
tion of the mechanical axis of the lower extremity, which is maintain the knee correction while treating the hip.
172 C. Iobst
Fig. 11.11 In normal alignment,

the anterior cortical lines of the
distal femur and the proximal
tibia should be collinear. If the
lines are not collinear, then the
angle created represents the
magnitude of the extension or
flexion contracture present.
Illustration created by and
reprinted with permission of
Daniel Biggers
Nonoperative The major disadvantage with these methods, however, is

In most cases of knee flexion contracture, an initial attempt the amount of corrective force that may be applied with
at nonoperative management should be considered. In gen- casts, splints, and braces is limited by the low tolerance of
eral, patients with relatively normal hip and ankle function the skin to direct pressure [29]. Additionally, the knees
can tolerate a knee flexion contracture of less than 20° [22]. must be carefully monitored for the development of poste-
The quadriceps’ function is bolstered when the body’s cen- rior tibial subluxation and recurrence rates are high. Two
ter of gravity is brought anterior to the knee by leaning for- recent reviews of the literature reached similar conclusions
ward with the hip locked in extension and using the plantar that stretching does not have clinically important effects in
flexors to stabilize the ankle. Nonoperative methods include joint mobility, pain, spasticity, or activity limitation in peo-
stretching by physical therapy, serial casting, hinged cast ple with, or at risk of, contractures [30, 31].
bracing, adjustable splinting, skin traction, dynamic spring-
loaded splinting, botulinum toxin A, and bracing. These Operative
methods are generally successful in patients with mild flex- Few patients with clinically significant knee flexion contrac-
ion contractures, or are used to complement posterior soft- ture can be managed definitively with nonoperative tech-
tissue knee release to gradually finish the correction. niques. If conservative treatment does not prevent the
Bracing with knee–ankle–foot orthoses or floor reaction progression of fixed knee flexion deformity, then surgical
ankle–foot orthoses may improve gait efficiency if there is options should be considered. The type of surgical interven-
quadriceps or ankle weakness [19]. The posterior push on tion chosen should be based on the cause of the contracture,
the knee from the brace in stance phase can improve mild its magnitude, its duration, the presence of tibiofemoral sub-
dynamic knee flexion contractures [18]. Mild contractures luxation, neurologic involvement, and the patient’s ability to
can also be managed by serial casting. A serial casting pro- participate in treatment [29]. The most common surgical
tocol of weekly sequential wedging demonstrated that options include soft tissue releases, anterior distal femoral
patients who were less than 12 years old and had an initial epiphysiodesis, extension osteotomy of the distal femur, and
flexion contracture of less than 15° were likely to maintain arthrodiastasis using an external fixator. Prerequisites for any
correction 1 year postoperatively [27]. Dynamic splinting, knee flexion contracture surgery include the absence of a
which delivers low torque, prolonged duration stretching concomitant hip flexion deformity of more than 15° and the
was found to improve active range of motion by an average presence of adequate quadriceps and gross neurologic func-
of 23° in a review of eight studies in the literature [28]. tion of the affected limb.
Soft Tissue Releases In most cases, the femur can be safely shortened acutely by
Soft tissue release procedures described in the literature 3 or 4 cm. In these large, long-standing contractures, the
may take different forms: distal hamstring tenotomy or surgeon should be aware that there might also be a fibrous
lengthening, proximal hamstring release, or hamstring ten- adhesion in the anterior part of the knee that blocks full
don lengthening combined with posterior knee capsulot- extension [18].
omy. Hamstring lengthenings are indicated when the After hamstring lengthening, a knee immobilizer can pro-
popliteal angle is greater than the flexion contracture [22]. vide sufficient immobilization for 3 or 4 weeks. If there is
In children, knee flexion contractures of less than 20° are any residual contracture, a long-leg cast is applied. Early
often responsive to hamstring lengthening alone [22]. weight bearing and ambulation are encouraged. The greatest
However, these soft tissue releases may not be sufficient to improvements in the knee flexion contracture are seen within
gain full correction in a more severely contracted knee. In the first year after surgery [18]. However, in a growing child
this situation, the long-standing altered resting length of the recurrence over time is common, with the return of the flex-
popliteal vessels and sciatic nerve may prevent full correc- ion deformity estimated at around 3° per year [32].
tion safely [29]. Neurovascular complications are more
common if full extension is performed immediately at sur- Bony Correction Options
gery in order to immobilize the patient in a straight cast. An alternative concept to the soft tissue releases for knee
The surgeon should be careful not to aggressively stretch flexion contracture is to alter the alignment of the distal
the posterior structures of the knee or a nerve palsy of all or femur to create knee extension. Two methods for achieving
part of the sciatic nerve can occur. The sciatic nerve does this correction have been described: distal femoral extension
not appear to tolerate elongations, especially when abruptly osteotomy and guided growth with anterior distal femoral
applied, of more than 5–12 % of its original length without hemiepiphysiodesis. Extension osteotomies of the distal
injury [32]. Hip flexion with the knees extended in casts femur are indicated for children with contractures no greater
can further stretch the sciatic nerve [18]. If a nerve palsy is than 45° [22] (Fig. 11.12). Although larger contractures have
recognized, the cast should be immediately removed and the been treated with extension osteotomies, there is increasing
knee allowed to flex. Postoperative serial casting requires risk of a neurovascular stretch injury. While shortening of
vigilance. The surgeon must also be wary of any developing the femur helps to protect the neurovascular structures, it
skin necrosis, joint cartilage compression, or joint subluxation exacerbates the limb shortening already caused by the angu-
that may occur while serial casting [29]. lar osteotomy. While the extension osteotomy can produce a
For children with flexion contractures of the knee, proxi- straight knee there are several concerns with this technique.
mal hamstring release is usually not recommended as the Distal femoral extension osteotomies does create a second-
primary soft tissue procedure as it can cause anterior pelvic ary deformity (angulation and shortening) instead of correct-
tilt and hyperlordosis of the lumbar spine [18]. Most suring the primary soft tissue deformity and may lead to
geons prefer to perform distal hamstring releases instead. abnormal joint-loading forces on the posterior femoral con-
A typical release pattern includes an intramuscular aponeu- dyles in the ambulatory patient [22, 29, 32]. Because of rapid
rotic lengthening of the semimembranosus, a Z-lengthening bone remodeling in the younger child, extension osteotomies
of the semitendinosus, and either a tenotomy or a have a high recurrence rate and should probably be delayed
Z-lengthening of the gracilis. When lateral hamstrings are until after 12 years of age [19].
included in the procedure, an intramuscular aponeurotic Guided growth treatment using an anterior distal femoral
lengthening of the biceps femoris is recommended. Usually hemiepiphysiodesis is another option for fixed knee flexion
two separate transverse cuts are needed in the fascia of the deformity exceeding 10°. Younger patients (<12 years old)
semimembranosus and biceps femoris to obtain adequate have demonstrated the best results with this technique and
lengthening [18]. Medial hamstring lengthening alone is correction of fixed knee flexion deformity up to 40° has
usually sufficient in patients with mild crouch and moder- been accomplished [25]. Guided growth using tension-band
ately increased popliteal angles. The addition of lateral ham- plates appears to have a faster correction rate and fewer
string lengthening will improve maximum knee extension complications than staples [25, 33]. Contraindications for
during stance phase. This is potentially helpful in patients guided growth treatment include less than 12 months of pre-
with more severe crouch but it also increases the risk of dicted growth remaining and flexion deformities that are
developing anterior pelvic tilt [18]. If there is a hard end caused by a dynamic etiology [25]. This technique does not
point to the range of motion in extension then the addition of seem to impair knee flexion or knee stability, which is a pos-
a posterior capsulotomy may be indicated. sible advantage over the soft tissue releases. However,
Correction of knee flexion contractures greater than 60° because the hemiepiphysiodesis correction is gradual, it
usually involves additional complete posterior capsular release may take a few years to obtain a meaningful correction in
with or without femoral shortening [18]. The femur must be large contractures, which may not be a suitable time frame
shortened to decompress the posterior neurovascular structures. for some patients.
174 C. Iobst
Fig. 11.12 Distal femoral

extension osteotomy stabilized
with a locking plate to correct a
knee flexion contracture (case
provided by Steven Frick, MD)
External Fixation with Gradual Soft Tissue Correction

Gradual extension of knee flexion contracture with circular
or monolateral external fixation can be used on essentially
any magnitude of contracture severity. This option has
many potential advantages especially in managing patients
with severe flexion contractures. Acute correction of long-
standing severe contractures risks stretching the sciatic
nerve and its branches and can create large skin defects
posteriorly. Hypertension caused by the sudden stretching
of the sympathetic nerves located around the blood vessels
can also occur [32]. In these situations, the sciatic nerve
may be more tolerant of gradual correction and skin prob-
lems may be more easily managed with external fixation.
With external fixation, tibial subluxation can be addressed
during contracture correction and no femoral shortening is
required, even with large deformities [22]. In flexion defor-
mities secondary to popliteal pterygium, the contracted
skin and fascia can be gradually stretched. The rate of con-
tracture distraction can be decreased as necessary to protect
soft tissue and neurovascular viability.
Fig. 11.13 The center of rotation of the knee can be identified at the
In external fixation of knee flexion contractures, the fixator intersection of the posterior femoral cortex line and the physis (or phy-
should be applied with its hinge carefully positioned on the seal scar)
knee joint center. The true center of knee rotation can be
approximated running through the origins of the medial and
lateral collateral ligaments [22]. Under image intensification When using a Taylor Spatial Frame™, this point can be cho-
in the operating room, the knee is positioned with the posterior sen as the “origin” (Fig. 11.14). It is important to avoid placing
and distal femoral condyles overlapping in the lateral projec- the hinge too posterior or too proximal to the center of rotation
tion. When the knee is too immature to visualize the femoral [22]. Posterior placement will aggravate the tendency for the
condyles well, an arthrogram may help better visualize the tibia to sublux posteriorly and proximal placement will lead to
femoral condyles [19]. The knee joint center of rotation can be anterior tibiofemoral impaction in full extension.
identified at the intersection of a line along the posterior femo- Patients with known preoperative subluxation of the tibia
ral cortex and the physis or physeal scar [24] (Fig. 11.13). on the femur should first have reduction of the subluxation
The extremity is then placed in a long leg cast with the

knee in full extension, with a mold applied to prevent poste-
rior tibial subluxation. The cast is removed in approximately
2 weeks and full-time knee–ankle–foot orthosis use is
begun immediately. Physical therapy to regain knee motion
is generally started at this time. After gradual correction with
an external fixator, there is usually a loss of knee range of
motion compared to the preoperative total arc of motion [29].
This range, however, has been redirected to a more useful and
functional arc compared to preoperatively. Fractures are a
potential complication when using external fixation, espe-
cially in the proximal femur. The surgeon should be aware of
any limitation of hip range of motion that could lead to exces-
sive stress on the femur with positioning. Fracture risk can be
minimized by using half pins no larger than 33 % of the diam-
eter of the bone, by avoiding unicortical pins, and by avoiding
tight clusters of metaphyseal pins [19, 22].
Recurrence of deformity is still a frequent clinical prob-
lem after any surgical treatment of knee flexion contractures
in children. Such procedures are best at redirecting the arc of
motion but rarely increase the total arc of motion [18, 29].
Fig. 11.14 External fixation used to gradually correct a knee flexion Weak or absent quadriceps power has been identified as an
contracture (same patient as in Fig. 11.10)
important factor leading to recurrence [26]. Pediatric con-
tractures can recur due to growth or from remodeling of
with the external fixator followed by distraction and gradual extension osteotomies [22, 32]. In very stiff contractures,
extension of the knee [22]. To help prevent tibial subluxation damage to the epiphysis can occur from excessive pressure
from occurring during the correction process, an arch wire between the femur and tibia when the knee is extended [18].
technique can be used in the proximal tibial ring so that as In some cases of recurrent severe knee flexion contracture
the wire is tensioned it pulls the tibia slightly anterior on the despite multiple surgeries, amputation with prosthetic fitting
femur [22]. In children with severe flexion contractures, it may be appropriate. If there is severe limb length inequality,
may not be possible to use full rings on the femur and the knee arthrodesis and a Symes amputation may provide a
tibia, as they will impinge on one another posteriorly. A half more satisfactory result than multiple failed attempts at cor-
or two-thirds ring can be used as a drop ring either in addi- rection [18].
tion to or as substitution for the periarticular full rings [22].
There is some debate as to whether soft tissue release is
necessary before attempting correction with an external fix-
ator but most surgeons agree that distraction of the knee joint
with the external fixator is mandatory prior to correcting the Box 11.2. Knee Flexion Contracture
deformity. This initial distraction of 5–10 mm is important to • The other lower extremity joints and the lumbar
avoid impingement and articular cartilage damage during spine cannot compensate for a knee flexion
correction [18, 19, 22, 26, 32]. contracture.
The usual rate of correction with the external fixator is • Prone radiographs of the femur and tibia allow eval-
1°–2° per day although this can be adjusted based on the uation of the coronal plane in patients with knee
patient’s tolerance [22, 32]. Stiff knees require a slower rate flexion deformity.
of correction to reduce the chances of a periarticular frac- • The standing full-length lateral view radiograph is
ture [22]. The external fixator construct should have a remov- critical to evaluate the sagittal plane alignment of a
able distractor to allow the patient to move the knee during patient with a knee flexion contracture.
correction treatment although such mobilization of the knee • Over-aggressive stretching of the knee into exten-
is not always well tolerated by the patients [19, 29]. Once the sion can cause a neurovascular injury to the limb
correction is completed, it is recommended that the frame after soft tissue releases.
be maintained at full extension for an additional few weeks to • Correction of the knee flexion contracture with an
allow the soft tissues to adapt to the new position. The frame external fixator should begin with distraction of the
is removed in the operating room and the leg is measured for knee joint.
a knee–ankle–foot orthosis with locking knee hinges [22].
176 C. Iobst
Decision Making and Management Strategy

Knee Extension Contractures
Nonoperative
While some joints such as the wrist or ankle can tolerate sig- Knee stiffness is expected to occur to a certain degree during
nificant loss of motion and still be compatible with reason- femoral lengthening. Physical therapy focused on maintain-
able function, the knee is much less forgiving [34]. Knee ing motion during the distraction and the consolidation phase
stiffness can become so disabling that it limits the patient’s is standard practice. However, certain patients do not respond
ability to perform activities of daily living. Knee extension to physical therapy [36]. The minimum acceptable amount
contractures are far less common than flexion contractures. of knee flexion for walking is 70° but this is not sufficient for
During gait, extension contractures of the knee cause swing- normal daily activities and a minimum of 110° is preferable
through dysfunction as well as dampen the small flexion in young patients [34]. Patients may be considered candi-
moment in mid-stance and thus make it difficult to walk effi- dates for surgery when they reach a plateau in gaining flex-
ciently [22]. Knee extension contractures also create prob- ion during their physical therapy and when flexion remains
lems with sitting comfortably. less than 90° [34]. Patients who fail to improve knee range
after at least one manipulation under general anesthesia
should also be candidates for surgery [34].
Etiology Manipulation under anesthesia has been shown to improve
the range of motion in patients with knee stiffness [37–39]. In
Common causes for knee extension deformity include scar- patients with less than 90° of knee flexion, this technique can
ring or fibrosis of all or part of the quadriceps after trauma, be used. It is designed to forcefully overcome the adhesions
adhesions from intra-articular knee surgery and quadriceps in the knee with the patient under general anesthesia and ade-
tightness after femoral lengthening. The anatomic condi- quate muscle relaxation. The physician should flex the
tions that cause a loss of knee flexion can be both intra- patient’s ipsilateral hip to 90° and apply progressive force to
articular (fibrosis and shortening of the medial and lateral the knee joint while holding the leg close to the knee. This
para-patellar retinaculum, adhesions from the deep surface should be done gradually and without excessive force.
of the patella to the femoral condyles) and extra-articular Audible and palpable lysis of the adhesions is often noted and
(fibrosis of the vastus intermedius with adherence to the rec- the knee should develop an appreciable increase in motion.
tus femoris muscle and to the front of the femur, shortening As more adhesive tissue accumulates in the knee, the chance
of the rectus femoris, adherence of the quadriceps to frac- of a successful manipulation decreases over time. Therefore,
ture callus or regenerate bone, adherence of the skin to the most authors recommend performing the manipulation within
underlying muscle, and pin site tethering on the lateral side 3–4 months from the index surgery. Complications from the
of the femur) [34, 35]. manipulation are related to the force needed to manipulate the
knee and include fracture, hematoma, rupture of the extensor
mechanism and wound dehiscence. The physician should be
Clinical Evaluation cognizant that skeletally immature patients are especially at
risk for distal femoral physeal fractures while undergoing
The physical examination is similar to that already presented manipulation of the knee [38].
for knee flexion contractures. The physician needs to evalu-
ate the arc of motion present and the stability of the knee, Operative
including the patella. Quadricepsplasty is the term applied to describe surgical
procedures to the quadriceps muscle designed to improve
knee flexion [34]. Numerous techniques have been devel-
Imaging oped to treat knee extension contractures including the
Thompson quadricepsplasty, the Judet quadricepsplasty, and
Radiographs of the knee are recommended to search for any their modifications [34–36, 40–45]. The literature suggests
distal femoral or proximal tibial deformities that may create that the range of motion achieved intra-operatively may be
or exaggerate the knee extension deformity. The physician the most important determining factor for the final gain of
should search for any underlying lateral femoral condylar knee flexion [35]. Despite the improvement in knee range of
hypoplasia and genu valgum in patients with congenital motion that these procedures achieve, quadricepsplasty can
femoral deficiency [36]. This finding can put the patient at create new problems such extensor lag, infection and iatro-
risk for developing an obligate patellar dislocation with genic injuries. Careful patient selection is paramount because
knee flexion. these procedures require intensive postoperative therapy [34].
Patient and family compliance and motivation are essential origin from the greater trochanter [34]. The literature reflects
for a satisfactory outcome. that the Judet quadricepsplasty seems to have a much lower
Many extension contractures of the knee have both intra- risk of extensor lag than the Thompson quadricepsplasty and
and extra-capsular components that need to be addressed. avoids the skin issues of the midline anterior incision [34,
Para-patellar incisions provide access to the scarred areas, 44]. Another promising modification of quadricepsplasty,
patella, and distal quadriceps mechanism. Intra-capsular called extra-articular mini-invasive quadricepsplasty, com-
releases involve incising the lateral and medial retinaculum, bines open release of the extra-articular contracture with
the supra-patellar pouch, and any intra-articular adhesions. arthroscopic release of the intra-articular contractures [45].
Extra-capsular contracture releases require extra-periosteal Several quadricepsplasty techniques have been developed
release of the quadriceps off the femur and the lateral inter- specifically for patients with knee extension contracture after
muscular septum. Tethering between the skin and quadriceps femoral lengthening. These patients usually do not have the
muscle should also be released. intra-articular contractures often seen in posttraumatic or iat-
The Thompson technique, described in 1944, is based rogenic knee extension contractures [42]. Hosalkar et al.
on isolating the rectus femoris completely from the vasti [40]. describe a technique using a lateral incision that combines a
This involves stripping the rectus femoris from the other primarily extra-articular release with a V-Y lengthening of
vastus muscles and releasing the vastus lateralis and vastus the quadriceps tendon [43]. They recommend repairing the
medialis from either side of the patella through a longitu- quadriceps tendon with the knee in 60° of flexion. A second
dinal midline incision. If the rectus femoris remains tight technique, the distal quadricepsplasty, is advocated by
and is limiting flexion, then lengthening of the rectus fem- Martin et al. [36]. The authors recommend this procedure for
oris is done. patients with stable knees and symptomatic restriction of
The combination of releases performed in the flexion after at least 3 months of unrestricted activities and
Thompson quadricepsplasty weakens the extensor mecha- intensive physical therapy, or those who develop obligate lat-
nism and can cause an extensor lag. Up to 80 % of patients eral patellar instability with knee flexion. In patients with
can develop an extensor lag after rectus femoris lengthen- patellar instability, residual external rotation or distal femo-
ing using the Thompson quadricepsplasty [34]. Extensor ral valgus is corrected before or as part of the surgical proce-
lag, if permanent, may be significant enough to affect the dure. Complete transection of the quadriceps tendon provides
stability of the knee and require some patients to use more potential length than the classic V-Y lengthening and
orthotics long term [34]. The anterior midline incision allows for transposition to treat obligate patellar dislocation.
used in this technique is another potential concern. The They describe making a long oblique incision of the quadri-
anterior location of the incision can create ischemia of the ceps tendon starting on the medial side as far distally as pos-
skin over the patella and delayed wound healing when the sible, and extending proximally and laterally as vertically as
knee is flexed [35]. Finally, extensor mechanism rupture possible to exit in the lateral patellar capsule incision. In
could occur intra-operatively if forceful manipulation of cases of patellar instability the distal or patellar portion in
the knee is attempted as a way to circumvent lengthening transposed medially relative to the proximal or quadriceps
of the rectus femoris [35]. portion. To determine the amount of lengthening required,
A modification of Thompson’s technique has shown they recommend establishing a firm repair of the tendon at
more optimistic results [35]. In this technique, the connec- 80°–90° of flexion.
tion of the vastus medialis to the superomedial corner of the Khakharia et al. describe a more limited quadricepsplasty
patella is preserved and anterolateral or lateral incisions are for femoral lengthening patients who are still in the external
used to avoid the problems with anterior incisions. These fixator [42]. At an average of 35 days from the end of distrac-
modifications also allowed early and intensive postoperative tion, patients with less than 60° of knee flexion were treated
rehabilitation. by a transverse fasciotomy to release the iliotibial band and
In 1956, Judet described his quadricepsplasty technique the anterior fascia of the thigh. The anterior fascia of the vas-
using a controlled staged correction of the knee stiffness [41]. tus intermedius was also released with care not to incise any
It permits sequential release of the intrinsic and extrinsic of the muscle tissue. Of the 16 patients treated with this
components limiting knee flexion and affords the opportu- method, one patient had a 10° extensor lag and one had clini-
nity to stop as soon as adequate flexion is obtained, thereby cal weakness. The authors do not recommend the limited
minimizing disturbance to the quadriceps muscle [34]. The quadricepsplasty for chronic knee extension contracture
procedure is done through two incisions. The first is a short from non-lengthening related etiologies.
medial para-patellar incision to release intra-articular adhe- The postoperative management is an important compo-
sions. The second is a long lateral incision allowing release nent to the overall outcome of all quadricepsplasties. Most
of extra-articular adhesions. The final step, which is necessary authors use a continuous passive motion machine that is
in only 20 % of patients, is a release of the vastus lateralis applied in the recovery room and set to a 0°–60° arc of
178 C. Iobst
motion and then advanced as tolerated. Weight bearing is 4. Lamm BM, Standard SC, Galley IJ, Herzenberg JE, Palay D.
External fixation for the foot and ankle in children. Clin Podiatr
allowed with crutches or a walker and a knee immobilizer to
Med Surg. 2006;23:137–66.
protect against inadvertent excessive knee flexion. Active- 5. Silfverskiold N. Reduction of the uncrossed two-joint muscles of
assisted knee flexion, passive flexion, active and passive the leg to one-joint muscles in spastic conditions. Acta Chir Scand.
extension, and isometric quadriceps exercises are encour- 1924;56:315–30.
6. Kirienko A, Villa A, Calhoun JH. Ilizarov technique for complex
aged postoperatively.
foot and ankle deformities. The equinus foot. New York: Marcel
Dekker; 2004. p. 25–57.
7. Calhoun JH, Evans EB, Herndon DN. Techniques for the manage-
ment of burn contractures with the Ilizarov fixator. Clin Orthop.
Box 11.3. Knee Extension Contractures 1992;80:117–24.
• Knee extension contractures are less common than 8. Koman LA, Smith BP, Barron R. Recurrence of equinus foot defor-
mity in cerebral palsy patients following surgery: a review. J South
knee flexion contractures.
Orthop Assoc. 2003;12:125–33.
• Patients should be considered for surgery if the 9. Shore BJ, White N, Graham HK. Surgical correction of equinus
knee flexion remains less than 90°. deformity in children with cerebral palsy: a systematic review.
• Because intensive postoperative therapy is required J Child Orthop. 2010;4:277–90.
10. Johnson J, Silverberg R. Serial casting of the lower extremity to
after quadricepsplasty, patient selection is critical.
correct contractures during the acute phase of burn care. Phys Ther.
1995;75:262–6.
11. Firth GB, Passmore E, Sangeux M, Thomasen P, Rodda J, Donath
S, et al. Multilevel surgery for equinus gait in children with spastic
diplegic cerebral palsy. J Bone Joint Surg. 2013;95:931–8.
Summary 12. Firth GB, McMullan M, Chin T, Ma F, Selber P, Eizenberg N, et al.
Lengthening of the gastrocnemius-soleus complex. J Bone Joint
Surg. 2013;95:1489–96.
A lower extremity joint contracture can be a frustrating con-
13. Baumann JU, Koch HG. Ventrale aponeurotische Verla ̈ngerung des
dition to deal with for the patient, the family and the sur- Musculus gastrocnemius. Oper Orthop Traumatol. 1989;1:254–8.
geon. Many treatment options can be effective initially but 14. Herzenberg JE, Lamm BM, Corwin C, Sekel J. Isolated recession
slowly recur over time as the child continues to grow. of the gastrocnemius muscle: the Baumann procedure. Foot Ankle
Int. 2007;28:1154–9.
Equinus contractures do not always require an Achilles ten-
15. Strayer LM. Recession of the gastrocnemius: an operation to relieve
don lengthening. A careful physical examination and a thor- spastic contracture of the calf muscles. J Bone Joint Surg. 1950;32:
ough knowledge of the gastroc-soleus anatomy will help the 671–6.
physician choose the correct site for addressing an equinus 16. Saraph V, Zwick EB, Uitz C, Linhart W, Steinwender G. The
Baumann procedure for fixed contracture of the gastrocsoleus in
contracture that will provide the optimal clinical outcome.
cerebral palsy. J Bone Joint Surg Br. 2000;82:535–40.
Knee flexion contractures have multiple options for treat- 17. Carmichael KD, Maxwell SC, Calhoun JH. Recurrence rates of
ment that can be divided into two basic categories: acute burn contracture ankle equinus and other foot deformities in chil-
correction or gradual correction. The options for acute cor- dren treated with Ilizarov fixation. J Pediatr Orthop. 2005;25:
523–8.
rection include soft tissue releases and extension osteoto-
18. Herring JA. Tachdjian’s pediatric orthopaedics. In: Eastwood DM,
mies. Gradual correction can be done with nonoperative editor. Congenital knee flexion contracture. 4th ed. Philadelphia:
techniques (physical therapy, bracing, botulinum toxin) or Saunders Elsevier; 2008. p. 926–9.
operative techniques (guided growth, external fixation). 19. Van Bosse HJP, Feldman DS, Anavian J, Sala DA. Treatment of
knee flexion contractures in patients with arthrogryposis. J Pediatr
Knee extension contractures are less common than flexion
Orthop. 2007;27:930–7.
contractures. Most treatment regimens recommend pro- 20. Laubenthal KN, Smidt GL, Kettelkamp DB. A quantitative analysis
gressing from physical therapy, to manipulation under anes- of knee motion during activities of daily living. Phys Ther. 1972;52:
thesia to quadricepsplasty as necessary to achieve correction. 34–43.
21. Murray C, Fixsen JA. Management of knee deformity in classical
New techniques for quadricepsplasty have been developed
arthrogryposis multiplex congenita. J Pediatr Orthop B. 1997;6:
that appear promising. 186–91.
22. van Bosse HJP. Contractures of the knee. In: Rozbruch SR, Ilizarov
S, editors. Limb lengthening and reconstruction surgery. New York:
Informa Healthcare USA, Inc.; 2007. p. 345–55.
References 23. Hoffer MM, Knoebel RT, Roberts R. Contractures in cerebral palsy.
Clin Orthop Relat Res. 1987;219:70–7.
1. Perry J. Gait analysis: normal and pathological function. 1st ed. 24. Herzenberg JE, Standard SC, Conway JD, Lamm BM, Siddiqui
Thorofare, NJ: Slack; 1992. NA. The art of limb alignment. 2nd ed. Baltimore: Rubin Institute
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J, et al. Isolated gastrocnemius tightness. J Bone Joint Surg. 25. Spiro AS, Babin K, Lipovac S, Rupprecht M, Meenen NM, Rueger
2002;84:962–70. JM, et al. Anterior femoral epiphysiodesis for the treatment of fixed
3. Deheer PA. Understanding equinus. Podiatr Management 2012; knee flexion deformity in spina bifida patients. J Pediatr Orthop.
157–164. 2010;30:858–62.
26. Devalia KL, Fernandes JA, Moras P, Pagdin J, Jones S, Bell cepsplasty for severely stiff knees. J Bone Joint Surg. 2010;92-B:
MJ. Joint distraction and reconstruction in complex knee contrac- 217–21.
tures. J Pediatr Orthop. 2007;27:402–7. 36. Martin BD, Cherkashin AM, Tulchin K, Samchukov M, Birch
27. Westberry DE, Davids JR, Jacobs JM, Pugh LI, Tanner JG. Treatment of femoral lengthening-related knee stiffness with a
SL. Effectiveness of serial stretch casting for resistant or recurrent novel quadricepsplasty. J Pediatr Orthop. 2013;33:446–52.
knee flexion contractures following hamstring lengthening in chil- 37. Fitzsimmons SE, Vazquez EA, Bronson MJ. How to treat a stiff total
dren with cerebral palsy. J Pediatr Orthop. 2006;26:109–14. knee arthroplasty? Clin Orthop Relat Res. 2010;468:1096–106.
28. Furia JP, Willis FB, Shanmugam R, Curran SA. Systematic review 38. Vander Have KL, Ganley TJ, Kocher MS, Price CT, Herrera-Soto
of contracture reduction in the lower extremity with dynamic JA. Arthrofibrosis after surgical fixation of tibial eminence frac-
splinting. Adv Ther. 2013;30:763–70. tures in children and adolescents. Am J Sports Med.
29. Herzenberg JE, Davis JR, Paley D, Bhave A. Mechanical distrac- 2010;38:298–301.
tion for treatment of severe knee flexion contractures. Clin Orthop 39. Evans KN, Lewandowski L, Pickett A, Strauss JE, Gordon WT.
Relat Res. 1994;301:80–8. Outcomes of manipulation under anesthesia versus surgical man-
30. Katalinic OM, Harvey LA, Herbert RD, Moseley AM, Lannin NA, agement of combat related arthrofibrosis of the knee. J Surg Orthop
Schurr K. Stretch for the treatment and prevention of contractures. Adv. 2013;22:36–41.
Cochrane Database Syst Rev. 2010;9, CD007455. 40. Thompson TC. Quadricepsplasty to improve knee function. J Bone
31. Katalinic OM, Harvey LA, Herbert RD. Effectiveness of stretch for Joint Surg. 1944;26:366–79.
the treatment and prevention of contractures in people with neuro- 41. Judet R, Judet J, Lagrange J. Une technique de liberation de
logical conditions: a systematic review. Phys Ther. 2011;91:11–24. l’appariel extenseur dans les raiduers du genou. Mem Acad Chir.
32. Carbonell PG, et al. Monolateral external fixation for the progres- 1956;82:944–7.
sive correction of neurological spastic knee flexion contracture in 42. Khakharia S, et al. Limited quadricepsplasty for contracture during
children. Strategies Trauma Limb Reconstr. 2007;2:91–7. femoral lengthening. Clin Orthop Relat Res. 2009;467:2911–7.
33. Klatt J, Stevens PM. Guided growth for fixed knee flexion defor- 43. Hosalkar HS, Jones S, Chowdhury M, Harley J, Hill RA.
mity. J Pediatr Orthop. 2008;28:626–31. Quadricepsplasty for knee stiffness after femoral lengthening in
34. Ali AM, Villafuerte J, Hashmi M, Saleh M. Judet’s quadriceps- congenital short femur. J Bone Joint Surg. 2003;85-B:261–4.
plasty, surgical technique, and results in limb reconstruction. Clin 44. Daoud H, O’Farrell T, Cruess RL. Quadricepsplasty: the Judet tech-
Orthop Relat Res. 2003;415:214–20. nique and review of six cases. J Bone Joint Surg Br. 1982;64:
35. Hahn SB, Choi YR, Kang HJ, Lee SH. Prognostic factors and 194–7.
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arthrofibrosis of the knee. J Bone Joint Surg. 2006;88:1245–50.
Physical Therapy During Limb
Lengthening and Deformity 12
Correction: Principles and Techniques
Anil Bhave, Erin Baker, and Mary Campbell
Introduction Rehabilitation During Distraction

Osteogenesis
Treating children who have lower limb deformities including
shortening can present challenges for the physical therapist. Rehabilitation for limb lengthening and reconstruction surgery
Whether the deformity is related to a congenital, develop- can be divided into four distinct phases: (1) inpatient phase
mental, or acquired etiology, these patients often present (postoperative days 1–3), (2) lengthening or correction phase,
with biomechanical compensations in their movement strate- (3) consolidation phase, and (4) post frame removal phase [3].
gies in order to be as independent as possible while perform-
ing daily activities. When planning treatment of their lower
limb deformities, besides assessing the child’s psychosocial Inpatient Phase
support system one must critically evaluate the mobility,
strength, and stability across the weight-bearing joints to Physical therapy begins on the first day following surgery.
understand how mobility may be impacted during surgical During this phase the therapist teaches the patient and fam-
correction. The goal of treatment for these children is pri- ily correct positioning in bed. Resting splints are made to
marily to improve their function and gait. In addition to maintain neutral positioning of the adjacent joints (Fig. 12.1).
physical abnormalities, these patients do suffer from body The patient and caretakers are instructed regarding range of
image issues [1]. Many patients report improved body image motion and supine exercises for a home program. The
after limb lengthening [2]. physical therapist is responsible for teaching the patient
In this chapter we discuss the salient principles and how to ambulate with assistive devices, to maintain weight-
techniques employed by the physical therapist while bearing precautions, and maneuver stairs. Equipment such
treating children undergoing limb lengthening and defor- as walker, commode, wheelchair, and crutches are also
mity correction with conventional techniques using exter- ordered in preparation for discharge from the hospital. For
nal fixation as well as more recently introduced patients undergoing limb lengthening, it is preferable to
intramedullary lengthening nails. We also briefly discuss order wheelchairs with reclining back rests in order to
specific rehabilitation issues amongst patients with con- avoid sciatic nerve stretch by not having patients sit upright
genital shortening of the lower limb as well as certain with their hips flexed up to 90°. In addition, elevated leg
other clinical conditions such as achondroplasia and rests are used to afford appropriate leg elevation and remov-
Perthes disease. able armrests are recommended for easy transfers to and
from the wheelchair.
Lengthening or Correction Phase
A. Bhave, MS (PT) (*) • E. Baker, MPT Based on variables such as the underlying diagnosis, need
M. Campbell, DPT for multi-segment lengthening, amount of deformity and
Rubin Institute for Advanced Orthopedics, Sinai Hospital, social considerations, there are three different rehabilitation
2401 West Belvedere Avenue, Baltimore, MD 21215, USA
e-mail: [email protected]; [email protected]; pathways that most patients take after surgery: outpatient
[email protected] physical therapy with one to two sessions per day for 3–5

182 A. Bhave et al.
days per week, a day program with two to three sessions of Consolidation Phase
therapy a day for 3–5 days a week, and inpatient rehabilita-
tion with extensive physical therapy in a rehabilitation hospi- In this phase, the goal of therapy is to maximize range of
tal [4]. The goals of physical therapy during the lengthening/ motion and continue to improve strength. Weight bearing
correction phase are to maximize joint range of motion and and exercises that promote axial loading of the bone are
maintain muscle tone and flexibility. Early joint mobilization encouraged. If patients have lost substantial knee range of
is imperative to prevent contractures. Barker et al. [5] found motion, such as development of flexion contracture after
that greatest loss of motion across joints occurred between tibial or femoral lengthening, then aggressive splinting
surgery and commencement of distraction indicating pain as across the knee joint with customized bracing or casting is
the primary cause. Aquatic therapy is a good adjunct to land done (Fig. 12.3). It is best to treat these contractures before
therapy for patients with multi-segment lengthening and the removal of external fixator since treatment of contracture
those undergoing bilateral lower extremity lengthening when after removal of fixator may lead to insufficiency fractures
full weight bearing is not allowed (Fig. 12.2). due to excessive stress and unresolved soft tissue tension.
Post Frame Removal
Gradual recovery of range of motion and functional strength

through progressive exercises is the focus of physical ther-
apy after removal of the fixator [4]. At this stage, there is a
concern for fracture through the pin sites and at the newly
formed bone of the lengthening regenerate [6]. It is recom-
mended to be cautious in the first 4–6 weeks after fixator
removal to allow for bone healing at these areas and therapy
may be delayed for a month [7]. Casts and braces may be
utilized during this phase for providing additional stability
to the lengthened bone [4]. Once physical therapy is
resumed, it is recommended that closed chain exercises be
started before open chain exercises. Closed chain exercises
are weight-bearing exercises, providing axial loading to fur-
ther strengthen the lengthened bone segment. Open chain
Fig. 12.1 Orthoplast custom foot splints are fabricated prior to dis-
exercises are exercises where the distal limb segment moves
charge from the hospital. In case of monolateral fixators a ring is
attached to the fixator for easy application of the foot splint using around the proximal axis, this leads to increase torque and
Velcro straps stress on the weaker newly lengthened bone segment.
Fig. 12.2 Chlorinated, heated,

hydrotherapy pool for promoting
increased range of motion and
muscle strength
12 Physical Therapy During Limb Lengthening and Deformity Correction: Principles and Techniques 183
Fig. 12.3 Use of a Taylor

Spatial Frame™ strut to apply an
extension force in a patient who
developed severe knee flexion
contracture during tibial
lengthening for fibular hemimelia
limb deficiency. Thigh cuff using
fiberglass casting is linked to the
external fixator with hinges
removal [2]. During this period, home exercise program and

participation in activities play a big part in the final journey
of return to full functionality.
Box 12.1. Complications During Limb Lengthening

• Muscle contractures
• Muscle weakness
• Joint stiffness
• Nerve injury
• Joint subluxation
Role of Physical Therapy in Management

of Complications
After the index surgery, patients can experience bone and/

Fig. 12.4 Careful stabilization of the femur and tibia are critical during or soft tissue complications, which can compromise func-
therapy after fixator removal. Patient receiving hamstring stretch with tional outcomes. Temporary soft tissue dysfunction occurs
posteroanterior mobilization. Therapist hands are placed as close to the in the majority of patients who undergo limb lengthening
joint line as possible
and deformity correction procedures. The severity of
these complications and obstacles to lengthening are
dependent upon the diagnosis, amount of lengthening or
Careful stabilization of the bone segment as close to the correction, soft tissue quality, level of osteotomy, tech-
joint line as possible is necessary to avoid bending stress on nique and device used for surgical correction, and nerve
the bone during mobilization with physical therapy complications [1]. In addition to physical causes of com-
(Fig. 12.4). Manual mobilization with adequate stabilization plications there are social reasons such as poor pain toler-
of the lengthened bone is a safe and effective way to regain ance and inadequate family support that can lead to
motion. Gait training and return to normative function are suboptimal results.
other additional goals during this phase. Recovery is slowest Examples of soft tissue dysfunctions include muscle and
immediately following fixator removal and speeds up tendon contractures, muscle weakness, joint stiffness, nerve
between 6 months to 1 year postoperatively. No further injury, and joint subluxation. Bone complications include
recovery is typically seen after 2 years following fixator poor bone formation, angulations of the regenerate bone, and
184 A. Bhave et al.
Fig. 12.5 Hand placement is

critical for joint mobilization.
Interior mobilization of the
patella during knee flexion
mobilization and correct
posterior stabilization of the
proximal tibia are important
insufficiency fractures. Therapeutic intervention is crucial to

avoid permanent dysfunctions. The physical therapist needs
to have adequate handling techniques and the skill to apply
appropriate forces and stabilization in order to focus treat-
ment targeted at the appropriate tissues (Fig. 12.5). It is a fine
balancing act since excessive forces can cause bone to bend
or fracture. The therapist must keep in mind that the stress
strain curve for tissues in the human body alters with age [8].
Pediatric patients deform their soft tissue with much less
force than adults. Bone and soft tissue complications can
occur during lengthening and consolidation phases, as well
as after the removal of the external fixator.
Muscle Contractures
Contractures occur when elastic tissues and contractile ele-

ments are unable to accommodate change in their length
Fig. 12.6 ASTYM™ treatment for improving muscle flexibility and to
following limb lengthening and deformity correction.
promote remodeling of the fibrous tissue
Myofascial tissues resist such elongation more than most
other tissues and are therefore more prone to contractures.
A relative shortening of diarthrodial or biarticular muscles
(crossing two joints) and the fascial component is often the ion, ankle plantarflexion, and toe flexion contractures.
cause of such contractures [1]. For example, the gastrocne- Likewise, during femoral lengthening, the quadriceps (espe-
mius develops passive tension much more rapidly in response cially the rectus femoris) and hamstring muscles are predis-
to bony lengthening than does the soleus. posed to develop contractures resulting in deficits of flexion
When muscles are kept in a shortened position, often due and potential for fixed flexion deformity across the knee
to postoperative pain early in the lengthening process the joint, respectively.
muscle adaptively shortens by absorbing sarcomeres to Soft tissue restriction can be further complicated by the
maintain efficiency. Prolonged stretch through a muscle has presence of scars from previous surgery, as is often seen in
been proven to add sarcomeres. The therapist must devise a patients with long-standing limb deformities. Scar tissue
way to provide prolonged stretch of such muscles across lacks the elasticity of the native tissue and scar fibers are
adjacent joints. For such stretch to be effective it must have often laid down in a much more disorganized, rigid pattern,
four critical components: (1) adequate intensity, (2) long especially when the tissue is immobilized due to pain or lack
duration, (3) adequate frequency with multiple repetitions, of motion. Utilizing soft tissue mobilization with implemen-
and (4) an active stretch component [8]. tation, such as ASTYM™ (Fig. 12.6) and Graston™, can
Problematic muscles should be identified early in order break down deficient, mature scar tissue and establish a more
to treat contractures effectively. In tibial lengthening, for organized, pliable scar [8].
example, problem muscles are the gastrocnemius and toe Managing muscle contractures effectively is vital for a
flexors. As a result, patients are prone to develop knee flex- satisfactory clinical outcome following limb lengthening and
Fig. 12.7 Pin site around the hip

joint can be painful due to
movement of excess tissue during
therapy. Massage around the pin
site and use of tight wraps help
reduce pain during therapy
at one joint is necessary during mobilization at the other joint

to obtain maximum stretch. Ideally, a patient should have
such treatments twice a day.
Positioning and splinting strategies should also be used to
manage soft tissue contractures. Optimal positions vary by
affected body part. For example, patients who have under-
gone tibial lengthening should be positioned in knee exten-
sion and ankle dorsiflexion (Fig. 12.9). Knee extension,
along with hip abduction, is a desirable position for patients
undergoing femoral lengthening. Commercially made static
splints (Fig. 12.10) augment therapeutic passive stretching
by counteracting the connective tissues’ elastic response to
passive stretching. Using a splint to place the muscle under
tension for as many hours as possible, helps prevent contrac-
Fig. 12.8 A compression wrap around proximal femoral pins to avoid tures by obtaining plastic response in the connective tissue.
tissue motion during therapy. This technique reduces pain as well as
risk of infection
Dynamic splints produce optimal plastic elongation (perma-
nent elongation) of the connective tissue through a low load
prolonged duration stretch (Fig. 12.11). Such a stretch also
deformity correction [1]. Passive stretching is an effective causes physiological remodeling of connective tissue through
way to manage soft tissue contractures. Per our protocol, biochemical response. Dynamic splints work most effec-
moist heat is applied prior to stretching to aid in collagen tively in treating knee contractures as they are a hinge joint.
breakdown and improve stretch of connective tissue [8]. Be aware that splints work only in optimal positions and ten-
Analgesic medications administered 30 min prior to therapy sion should always be increased gradually.
help patients tolerate the stretch better. Reciprocal inhibition, Commercially made dynamic splints do not always pro-
activating the antagonist muscle before stretching the ago- vide the customization needed for an effective stretch.
nist, relaxes the muscle being stretched. Pin sites near the Therefore, the authors developed and utilize a custom knee
fascial and muscle planes are susceptible to inflammation. device (CKD) for contracture management during or after
Thus gentle soft tissue mobilization and massage helps to external fixation (Fig. 12.12). This is a semirigid device
manage tightness caused by adherent soft tissues around the made of a polyester and fiberglass cast of the thigh and lower
pin. Another strategy to reduce pain around the pins during leg with an aluminum hinge across the knee to allow for pro-
joint mobilization and stretching is to use tight pin wraps gressive stretching into the restricted range of motion. Elastic
especially at sites near the knee or hip joint (Fig. 12.7). The bands are utilized to provide the needed tension and the
tight pin wraps reduce skin motion during therapy and sig- rigidity of the casting materials allows for minimal loss of
nificantly reduce pain (Fig. 12.8). Stretching of biarticular the stretch energy to the desired tissues. Knee joint sublux-
muscles 10 times per session and uniarticular muscles five ation can also be managed utilizing the CKD with added
times a session with 20–30-s holds for each stretch is recom- straps at the knee joint to help approximate the joint and
mended. When stretching a biarticular muscle, stabilization reduce the subluxation. The more the patient wears this
186 A. Bhave et al.
Fig. 12.9 Patient undergoing

tibial lengthening and
procurvatum deformity
correction. Gastrocnemius and
soleus contracture with lack of
ankle dorsiflexion occurs
commonly in these patients
Fig. 12.10 Commercially

available walking shoe
modification with Velcro straps to
keep ankle in neutral dorsiflexion
position
Joint Stiffness
Muscle contractures, if not treated aggressively, lead to

capsular and intra-articular adhesions. Contracture of biar-
ticular muscles also introduces compressive force on the
articular cartilage, which leads to a stiffness across adjacent
joints [9]. Characteristics of a stiff joint include loss of
motion and ease of movement within the joint’s available
excursion. Joint stiffness is best prevented with active and
passive motion. Manual traction with joint mobilization is
beneficial and recommended when feasible. However, such
mobilization can only be performed on joints that are not
included in the external fixation construct.
Fig.12.11 Use of commercially available dynamic brace (Dynasplint™)

attached to the proximal tibial ring to improve knee extension
Muscle Weakness
Patients may experience muscle weakness due to disuse atro-

device, the sooner the muscle truly lengthens and range of phy and neurogenic inhibition with associated pain.
motion is restored. This concept can be utilized with tibial Electrical stimulation and hydrotherapy are two modalities
frames, as a thigh cast can be made with a half ring casted that can be used to manage muscle weakness. Electrical
in and then attached to the tibial frame with a hinged joint. stimulation is used as an adjunct to a strengthening program
A telescoping strut can be added and the knee can be pro- and to augment voluntary muscle contraction. The most
gressively straightened if a flexion contracture develops common area for electrical stimulation is the quadriceps
(Fig. 12.13). It is recommended to use these splints all night muscle (Fig. 12.14). In our experience, as long as there is no
or at least 3–4 h per day. direct contact of electrodes to the half-pins, it has been safely
Fig. 12.12 Use of custom knee

device (CKD) in a child with
congenital femoral deficiency
(CFD) undergoing femoral
lengthening using monolateral
fixator. Elastic bands offer
continuous dynamic stretch in to
extension. Patients are
encouraged to use the device
8–14 h per 24-h period daily
Fig. 12.13 Use of removable

thigh cuff attached to tibial frame
with hinges in a patient
undergoing tibial lengthening.
Taylor Spatial strut between ring
incorporated in thigh cuff and
proximal tibia ring allows for
applying an extension stretch
Fig. 12.14 Neuromuscular

electrical stimulation of the
quadriceps muscle with heel
propped position. This position
allows for active stretch as well
as augmented strengthening of
the quadriceps
tolerated by children undergoing conventional distraction intensity settings with these devices as compared to older
osteogenesis treatment with external fixation. devices. Family education for setup is recommended for
At our center, typical electrical stimulation parameters are incorporation of electrical stimulation into a home program.
a biphasic pulse at a frequency of 35–70 Hz and pulse dura- Hydrotherapy helps avoid significant muscle weakness,
tion of 300–400 μs. In addition, an on-to-off ratio of 1:3 is especially in patients with bilateral external fixators or
recommended. Stimulation intensity should be adequate to unilateral femur plus tibial fixators. Hydrotherapy also pro-
cause strong contraction of the muscle. Patients tolerate motes increased active ROM (see Fig. 12.2). Buoyancy
newer electrical stimulation devices better using advanced offsets fixator weight and facilitates muscle strengthening.
frequency modulation software. Children tolerate higher Limb lengthening patients experience aquatic therapy in a
188 A. Bhave et al.
heated, clinically chlorinated pool. Hydrotherapy allows for

weight bearing early in the lengthening process, even if
there are weight-bearing restrictions on land. With the water
up to the nipple line, patients can utilize the buoyancy of the
water to provide their bones with protection from gravity’s
compressive forces to walk for continued cardiovascular
health and muscle memory. The heated water brings muscle
relaxation and freedom of motion, which allows for exercise
and standing mobility. The chlorine levels at our center are
kept at level 5 since patients get in with open pin sites.
Purulent drainage from a pin site is a contraindication
for getting into the pool. Daily assessment of pin sites are
done by the treating land therapist as well as the aquatics
therapist.
Fig. 12.15 Use of knee extension rod in a patient undergoing femoral

Joint Subluxation lengthening for congenital femoral deficiency. Patients are recom-
mended to use the extension rod all night long and 2 h on and 2 h off
during day
Joint subluxation is a dreaded complication of limb length-
ening surgery. Subluxations are caused by lack of opposition
of the joint surfaces, muscle and tendon contracture, and
ligamentous laxity. If a subluxation is not recognized and
treated early, it can result in permanent sequelae. This can
lead to significant reduction in range of motion, pain, and
early onset of arthritis due to articular cartilage erosion.
Knee subluxation in patients who have undergone femur
lengthening for congenital femoral deficiency usually
involves posterolateral translation of the proximal tibia. Such
patients may have cruciate deficient knee joints, hamstring
contracture, quadriceps inhibition, tight iliotibial bands, and
flexed knee posturing. The combined effect of these condi-
tions leads to unopposed posterolateral pull on the proximal
tibia and in some cases also lateral subluxation of the patella
[1]. Knee joint subluxation can occur during femoral length-
ening if the joint is not protected in the frame or during
lengthening with an intramedullary lengthening device. It
can also occur due to unresolved residual tension in the soft Fig. 12.16 Posterior subluxation of the knee in a patient undergoing
tissues post fixator removal. Due to the high frequency of femoral lengthening. The posterior subluxation of the tibia is demon-
subluxations in the congenital femoral lengthening most sur- strated by the arrow. Due to prevalence of this complication, most sur-
geons use tibial frame linked to the femoral frame with hinges to
geons prefer to extend the frame to the tibia with a hinge at
prevent knee subluxation
the knee joint (Fig. 12.15) [1].
Treatment for knee subluxation starts with early detec-
tion. Usual signs include posterior slope of the tibia (com- moderate subluxations respond to conservative regimens,
monly called the ski slope sign) (Fig. 12.16), anterior pain on while more severe ones may need surgery that includes distal
knee extension, and overall sudden increase in stiffness and iliotibial band and biceps femoris releases.
pain. Physical therapy treatment consists of vigorous knee Hip subluxation is a rare complication of femoral length-
extension with proper proximal tibia anterior tracking via ening. It presents as posterolateral migration of the femoral
slings and manual mobilization techniques. Soft tissue mobi- head and usually occurs in patients with acetabular dysplasia
lization techniques and augmented tissue stimulation to the in the face of an adductor contracture during proximal femur
lower kinetic chain using ASTYM™ is particularly useful. If lengthening. Adductor stretching and an abduction pillow
the patient has an external fixator with no extension to the can help prevent hip subluxation in proximal femoral length-
tibia, then manual therapy with joint distraction can also be ening. When hip subluxation occurs, patients usually need
employed. At night, the patient may also use tibial traction additional surgery including adductor release, followed by
and dynamic splints. In the author’s experience, mild to tibial skin traction and intensive therapy.
has the capability to store data for later comparison. One

drawback of such a device or any sensation testing is that the
patient needs to be of an age or maturity to participate reli-
ably in the test. Usually, this is reserved for patient’s aged
6 years and older. When sensory signs and symptoms of
nerve compromise are present, the medical team will either
slow down the lengthening or prescribe medications like
Gabapentin (Neurontin™) or Lyrica™ to manage the pain
associated with nerve problems. In some cases, especially
those with motor weakness, urgent surgical release of the
offending nerve is required. Considerations need to be made
when providing therapy to patients who have nerve compro-
mise. Bi-articular stretches, such as straight leg raises, should
be avoided in favor of mobilization at each individual joint of
the lower leg. This allows for soft tissues to be lax on one end
while the opposite end is treated.
Weight-Bearing Considerations
Functional mobility training will depend greatly on the level

of weight bearing allowed. During the lengthening and con-
solidation phases, weight bearing may be permitted, depen-
dent on the type of fixator or device used and the bone being
Fig.12.17 Pressure specified sensory device (PSSD) (Sensory Management, lengthened [4]. A ring fixator (such as the Ilizarov and
Baltimore, MD). This device allows for on invasive monitoring of sensory Taylor-Spatial™) generally provides the underlying bone
function during lengthening
with substantial support to allow for 50–100 % weight bear-
ing. In the classic Ilizarov frame where each segment has
Nerve Injury four rings and four wires are placed on each ring with spe-
cific tensile strength, all of the force that normally would go
During the lengthening process, as the bony segments slowly through the bone is transferred to the metal frame, protecting
distract, the soft tissue often becomes taut and restricted. It the osteotomy site and allowing for full weight bearing. The
was thought that bony distraction over-stretches the nerve, more recent versions of the circular frame utilize rings simi-
but Nogueira et al. [10] found this is not always the case as lar to that of the Ilizarov but favors half-pins instead of tran-
no correlation was noted between amount lengthened and the sosseous wires as bony anchors. These pins are generally
onset of nerve signs. Patients often present to therapy with better tolerated by the patients as they offer less tearing of
numbness and tingling peripherally with burning pain along the skin. If the foot is incorporated into the frame then a
nerve distributions. The most commonly compromised nerve walking ring is added, fitted with a rubber tread to allow for
is the peroneal nerve, more prevalent in tibial lengthenings safe ambulation (Fig. 12.18).
and corrections. Classic signs are numbness and burning Monolateral rail fixators handle weight-bearing forces
pain on the dorsum of the foot, especially between the first very differently. Typically, majority of the forces on the bone
and second toes. In more severe cases, the extensor hallucis are lateral for the femurs and medial for the tibia. Without
longus (EHL) becomes weak or worse, the tibialis anterior any counter to distribute forces in an even manner, weight
muscle weakness leads to a foot drop. The sciatic nerve can bearing is restricted in such patients during lengthening to
also be over-stretched, especially in large femoral lengthen- prevent loosening of the pins or worse, fracture at the pin
ings or simultaneous femoral and tibial lengthenings. In foot site. Many monolateral fixators for femoral lengthening will
corrections, as in clubfoot, the medial plantar nerve is at have a hinge at the knee and an extension to the tibia, with
greatest risk for compromise, with burning and tingling on a partial ring attached to pins in the tibia (see Fig. 12.15).
the plantar surface of the hallux. If nerve compromise is sus- This offers a great deal of additional stability at the osteot-
pected, the authors utilize the pressure specified sensory omy site, allowing for weight-bearing as tolerated during the
device, or PSSD (Fig. 12.17). This device can reliably test lengthening process.
the light touch sensation of various nerve distributions with Even in cases where monolateral rails are used without a
100 % sensitivity and 87 % specificity [10]. The device also counter as in stature lengthening, weight bearing with a walker
190 A. Bhave et al.
Fig. 12.19 Use of hip extension rod to maintain maximal hip exten-
sion in patient undergoing hip distraction for treatment of Perthes
Diseases. Patients wear the rod all night long
rotation. A partial ring over the pelvis provides stabilization

to a hinged joint at the axis of the sagittal plane for hip
motion and a distractor bar is attached to the monolateral
Fig. 12.18 Application of walking ring to the tibial frame allows for rail. The femur is gently distracted over a period of 10–14
pain free weight bearing and also one can adjust the length of the walk- days and held in position for approximately 4 months to
ing ring to accommodate leg length difference
allow for re-ossification and reshaping of the femoral epiph-
yses. Utilizing this frame can lead to improved joint mobility
is often allowed for transfers only. Repetitive forces through and decreased pain [13].
these devices weaken the bony interface, as would happen The focus of rehabilitation immediately after surgery is
with unrestricted weigh-bearing. Restricted, controlled weight on standing mobility training, family education, and estab-
bearing for very short durations allows for stretching of the lishing a home program for the patient. Since the legs are
gastrocnemius muscles and the work induced is good for car- being held in abduction and weight bearing through the
diovascular health. effected limb is limited, a wide rolling walker is often needed
These considerations around weight bearing will shape initially to aid in ambulation. Patients can progress to axil-
the plan of care in physical therapy. Ambulation during lary crutches eventually to allow for ambulation on stairs, but
lengthening helps to maintain muscular and bony strength selection of crutches is a very individual choice, based on the
and may shorten the rehabilitation process after fixator patient’s safety awareness and balance control. For long dis-
removal [11]. tances, these children still need a wheelchair, with consider-
ation of the size of the patient and the extra width added by
the frame laterally.
Specific Musculoskeletal Disorders Only sagittal plane motion is allowed by the hip distrac-
tor. Therefore, only hip flexion and extension mobilization
Legg-Calve Perthes Disease can be done while in the frame. The family needs to be
educated in a home stretching program to maintain hip
Legg-Calve-Perthes disease (commonly referred to as motion and prevent contractures from immobility and pain.
Perthes disease) is usually seen in children ages 4–10 years A removable anterior telescoping bar is added to the frame
and is more common in boys [12]. Perthes disease has been to provide a progressive, sustained stretch across the hip
treated using the principle of “containment” by long periods into extension (see Fig. 12.19). After 4 months the frame is
of immobilization, historically using hip spica casting to removed and the role of physical therapy increases expo-
hold the femoral head in a most congruous position with the nentially. For the first 6 weeks post removal, the patient is
acetabulum to allow the best reshaping of the femoral head. wearing a hip abduction brace nearly at all times, giving the
Hip spica casts often bring difficulty with mobility of the patient a very wide stance in gait. Weight bearing is allowed
child as well as hygiene issues. Utilizing a hip distractor at this point and gait training is needed to learn to ambulate
external fixator alleviates these issues as well as assures a without a device.
congruous position of the hip (Fig. 12.19). They hold the hip Without frontal and transverse planes of motion across
in 15°–20° of abduction, 10° of flexion and 10° of external the hip during external fixation, abduction/adduction and
internal/external rotation motions are very limited after

frame removal. Therapy focuses on regaining the hip motion,
both passively through joint mobilization and actively
through exercise and mobility training. Control of active hip
motion is challenging in this population since the lumbar
spine so readily provides foot placement in gait and move-
ment in exercise—a compensatory strategy developed dur-
ing the 4 months of non-use in the frame and potentially even
longer, depending on how long the disease progressed prior
to treatment. With an abduction contracture on the effected
limb, the leg feels longer to the patient, and a compensatory
trunk lean to the contralateral side is apparent. Furthermore,
due to the young patient population, it becomes imperative
that the family is involved in treatment, learning to cue the
patient to aid in retraining and carry over the exercise pro-
gram at home. Normalization of gait can take several months.
In our experience, integrating yoga and other treatment
modalities that provide postural awareness help in the recov-
ery of hip motion and strength, and ultimately allow a return
to independent activity.
Congenital Femoral Deficiency Fig. 12.20 Careful hand placement for knee flexion mobilization in a
patient with femoral lengthening for CFD. The therapist’s left hand is
stabilizing the hinge and right hand applies flexion force as well as sta-
Lengthening the femur of a child with congenital femoral bilizes posterior tibia
deficiency (CFD) can be one of the most difficult lengthen-
ings to manage. Up to 95 % of these children have deficient
or absent cruciate ligaments in the knee and such instability After removal of the fixator, therapy can begin to focus on
can cause problems during lengthening [14]. As the femur mobility training as well as regaining motion to the preop-
gets longer, the pull of the muscles increases, especially that erative level. Often children with CFD have acetabular dys-
of the hamstrings. With underdeveloped quadriceps and pain plasia and weakness around the hip, especially involving the
limiting motion, the pull of the hamstrings can cause a pos- abductors. Gait training and exercise that focuses on closed
terior subluxation of the tibia on the femur. Having a soft chain control of the hip and trunk are needed, but often dif-
tissue reconstruction to address the cruciate deficiency prior ficult for the patient. Gaining normal strength of the abduc-
to femoral lengthening in a patient with CFD associated with tors may not be a realistic goal, given the number of prior
ligamentous laxity lowers the risk of postoperative sublux- surgeries including placement of intramedullary devices that
ation [15]. If subluxation occurs, lengthening is slowed or may have been placed and removed from the femur in their
stopped and knee extension mobilization needs to be done lifetimes causing damage to the abductors if a trochanteric
with posterior support to the proximal tibia. approach was used as well as the presence of hip dysplasia.
The half pins used to anchor the fixator across the knee Treating a child or adolescent with an internal femoral
joint can be problematic with knee flexion mobilization. lengthening device presents a similar set of potential prob-
Therapists’ hand placement is important for the safety of the lems, although the external fixation can provide stability to
patient. The knee cannot be mobilized into flexion from the the knee and prevent subluxation. Often CKDs are made
distal tibia because that can cause loosening of the tibial with the use of subluxation straps early in the process to
pins. Anterior to posterior tibial mobilization must be done encourage gentle knee extension and prevent posterior sub-
proximally, with a posterior counter force to prevent pin luxation of the knee during such lengthening (Fig. 12.21).
loosening and fracture of the bone (Fig. 12.20). The loss of
leverage and inefficient grip can make this mobilization dif-
ficult for the therapist. Knee flexion is often where the great- Fibular Hemimelia
est loss of motion occurs during CFD lengthening. If the
flexion measures 40° or less, it is often recommended that Fibular hemimelia is the most common lower extremity
lengthening be stopped, at least temporarily, till further joint deficiency affecting males slightly more than females [16].
mobility is gained [9]. Tibial lengthening and deformity correction for the treatment
192 A. Bhave et al.
Fig. 12.21 Use of CKD bracing

with special straps. The straps
allow for an inferior mobilization
of the distal femur and superior
mobilization of the proximal
tibia. The combination of CKD
with elastic band and special
straps allow for simultaneous
correction of knee flexion
contracture and subluxation
of fibular hemimelia is commonly done with a circular fixator. with excessive anterior pelvic tilt, a more prominent sacrum,
Knee extension and ankle dorsiflexion are the motions most and a smaller spinal canal. These skeletal characteristics
compromised. The foot can be in or out of the frame. In our put the patient at an increase risk of symptomatic spinal ste-
experience, the patient is more apt to lose range of motion at nosis during bilateral lower extremity lengthening. Besides
the knee and ankle if the foot is not included in the frame. neurologic deficits in the lower extremity, loss of bowel
The peroneal nerve is also at risk of being compressed or and bladder function can also occur due to tethering of the
overstretched during tibial lengthening [1]. Neurogenic spinal cord and nerve roots. During treatment, the physical
symptoms can worsen with knee extension. Patients with the therapist should position the patient to compensate for these
foot included in the frame are at a higher risk of peroneal skeletal abnormalities while performing lower extremity
nerve palsy with tibial lengthening as they are unable to mobilizations. This can be done by utilizing pillows and
plantarflex the ankle to provide relief to the nerve at the fibu- wedges to allow for hips to be in a flexed position and the
lar head. Surgeons will often prophylactically release the spine to have decreased lordosis (Fig. 12.22). A prominent
peroneal nerve in such cases. sacrum also puts patients at a higher risk of sacral wounds.
Patients with fibular hemimelia often have altered It is important for patients to have time set during the day
mechanics of the ankle joint. The mobility of the ankle is that they are lying prone to allow for decreased pressure on
generally limited in these patients with some compensation the sacrum to decrease risk of pressure wounds. Wheelchair
at the midfoot and forefoot due to some compensatory hyper cushions are also used to alleviate pressure on the sacrum in
flexibility at these distal joints. It is important for the physi- sitting.
cal therapist to consider these compensatory mechanisms as Individuals with achondroplasia also present with unique
gait training advances following removal of the fixator. soft tissue characteristics including ligamentous and joint
Rocker bottom shoes may help the patient achieve a more laxity, and relatively redundant muscle tissue [17]. This rela-
normal gait pattern despite lack of ankle mobility. tive laxity of the muscles decreases strength and leads to a
waddle type gait pattern. Often, there is a noticeable trunk
sway for compensation as well as a larger base of support
Achondroplasia during the gait cycle. These gait abnormalities provide the
individual with increased stability without the use of core
When approaching a patient undergoing limb deformity cor- control and proper hip abductor mechanism function.
rection due to underlying achondroplasia, it is important to Redundant muscle tissue along with ligamentous and joint
take into consideration the unique morphologic features of laxity allows for increased flexibility early in the limb length-
this patient population. Skeletal characteristics in achondro- ening process, limiting the amount of motion lost in the early
plasia include but are not limited to increased lumbar lordosis phase of lengthening.
Fig. 12.22 Prone positioning in

a patient with achondroplasia
undergoing four-segment
lengthening. Use of a wedge
allows for reduction of the
hyperlordosis in patients
While undergoing bilateral lower limb deformity correction/

lengthening, patients are allowed weight bearing for transfers
only. In addition to muscular and cardiovascular benefits
mentioned earlier, transfer training gives the patient and
family increased independence at home. Patients can use push
up blocks to help provide upper extremity leverage during
lateral and anterior/posterior transfers from wheelchair to bed.
Assistance should be given to the lower extremities, lifting
from the full tibia rings if available. Core stabilization exer-
cises can help the patient control the spine for transfers, par-
ticularly important in head and neck control. Aquatics become
an important physical therapy modality for strengthening and
gait training while the patients are non-weight bearing.
Physical therapy is performed on a daily basis through-
out lengthening, with the primary focus to limit loss of Fig. 12.23 Knee extension mobilization with hip in extension. This
motion through the use of soft tissue and joint mobilization. position allows for adequate knee extension stretch without compro-
mising or stretching sciatic nerve
If lengthening can be carried out simultaneously in all four
segments (bilateral femur and tibia), these patients are at a
high risk for joint contractures, despite their soft tissue lax- After lengthening is complete, patients are in external
ity. Loss of motion often occurs in ankle dorsiflexion, hind- fixators until proper bone healing has occurred. During this
foot eversion, knee flexion/extension, hip flexion/extension, consolidation phase, weight bearing is gradually increased
and hip abduction. Patients with monolateral rail lengthen- and strengthening can be progressed. Loss of hip extension
ing of the femur may experience increased tension in the and ankle dorsiflexion during lengthening leads to an
distal iliotibial band due to the tethering at the pin sites [17]. increase in lumbar lordosis while standing with a signifi-
Mobilizations can be performed to the distal iliotibial cantly flexed posture of the knees and hips. Standing stretches
band by performing a gentle varus mobilization of the knee. of the gastroc-soleus complex with use of tilt boards or
If circular fixators are being used for the tibia, then foot wedges helps to improve erect posture and allow for the
splints can be attached to the circular rings to help limit loss stretch-recoil necessary for proper push off during gait.
of dorsiflexion. Regaining hip extension is important to decrease the stress
Four-segment lengthening also contributes to increased on the lumbar spine and spinal cord. Increasing hip extension
risk of nerve injury. The physical therapist must continue to also restores the stretch-recoil of the hip flexor mechanism to
monitor for early signs and symptoms of neuropraxia dur- allow for appropriate propulsion of the lower extremity.
ing the lengthening process. Knee extension stretching Immediately after external fixators are removed, patients are
should be performed with the hip in less than 60° of flexion. non-weight bearing for approximately 4 weeks. During this
In severe cases the hip can be dropped over the side of the non-weight-bearing phase, the patient’s family performs gentle
table into slight hip extension to avoid stressing the nerve range of motion exercises. When sufficient bone healing has
pathway during knee extension mobilization (Fig. 12.23). occurred and weight bearing is allowed, physical therapy
Ankle dorsiflexion mobilization can be modified by being resumes with an emphasis on gait training. Patients habitually
performed with the knee in slight flexion. have a wider base of support after fixator removal, both from
194 A. Bhave et al.
compensatory gait prior to surgery and having walked with

external fixators. Treatment strategies should focus on normal-
izing the base of support, increasing stride length, and improv-
ing timing of hip musculature activation. Global lower
extremity and core strengthening facilitates the patient’s over-
all independence with their functional activities and gait.
Internal Lengthening Devices
There has been an increasing interest at some centers in per-

forming “internal lengthening” of the lower extremity using
intramedullary nails. In our anecdotal experience, patients
and families seem to prefer this method over using external
fixators. Currently, the use of intramedullary lengthening
devices in femur or tibia is generally limited to patients who
have reached skeletal maturity, have minimal or no angular
deformity and have adequate diameter of the intramedullary
canal of the lengthened bone. Weight bearing is usually
30–50 lb. during lengthening. It is also important to remem-
ber that it is not the magnitude of load but the frequency of Fig. 12.24 Knee subluxation in a patient undergoing internal femoral
loading that can have deleterious effects on the stability of lengthening for congenital femoral deficiency
the intramedullary lengthening implant During the distrac-
tion phase weight bearing is typically limited to activities of
daily living only, whereas in the consolidation phase weight
bearing can be increased as maturation of the lengthening
regenerate occurs.
Complications of internal lengthening are similar to that
of the external fixator excluding pin site problems. The rate
of complications or obstacles to lengthening using the
ISKD™ device has been reported as high as 65 % [18].
These rates are similar to rates reported in the limb lengthen-
ing performed with external fixator.
In addition, due to lack of external fixation stability, the
risk of joint subluxation is increased with the lengthening
nails. Knee flexion contracture with subluxation is a com-
mon complication of internal lengthening especially in
patients with congenital etiology (Fig. 12.24). Prophylactic
bracing and adequate physical therapy are critical to prevent
joint subluxation and development of permanent contrac-
tures. The magnitude of complications is also dependent
upon the ability to control the rate of lengthening. If the
internal lengthening device goes faster than 1–1.5 mm a day,
the severity of complications is greater. The authors have Fig. 12.25 Complete resolution and normal knee alignment of the
successfully treated this problem of excessively rapid length- patient in Fig. 12.24 after 6 weeks of customized therapy including
ening utilizing proper handling techniques as well as custom joint and soft tissue mobilization, quadriceps electrical stimulation and
use of CKD with special straps for 16 h per day
dynamic bracing (see Fig. 12.21). In a retrospective review
of this approach in 20 patients (with 22 contractures, 9 sub-
luxations), successful resolution of knee flexion contractures The authors prefer ASTYM™ to treat the entire kinetic
occurred in 19/20 patients (95 % success) and 8/9 sublux- chain for patient undergoing internal lengthening since there
ations (88 %) (Fig. 12.25) [19]. are no pins to interfere with the treatment. For femoral internal
Knee flexion deficits are also a common obstacle to internal lengthening, special attention must be given to the tensor fas-
femoral lengthening. Restoration of motion is best achieved cia lata and iliotibial band as well as the muscles that attach to
with daily therapy including patella and soft tissue mobilization. the tensor fascia lata. Modified Ober positioning (Fig. 12.26)
Fig. 12.26 Iliotibial band stretching in a modified Ober position
with gentle varus mobilization of the tibia as well as prone

knee flexion with external rotation of the hip and simultane-
ous internal rotation of the tibia over femur are useful tech-
niques to target these tissues. Patellar mobilization along
with regaining knee joint mobility is paramount. Special
emphasis should be given to inferior patella mobilization
with knee flexion mobilization and superior patella mobi- Fig. 12.27 Patients with femoral lengthening have a tendency for lat-
lization with knee extension mobilization. Patellar taping eral subluxation of the patella and patella alta. Patellar taping is a useful
adjunct to help mobilize patella medially and inferiorly
techniques are also a useful adjunct to therapy (Fig. 12.27).
The goal of patellar taping is to neutralize lateral subluxation
force due to tight lateral structures and encourage inferior children with LLD intuitively use compensatory mecha-
movement of the patella. nisms such as (1) equinus posturing of the ankle on the short
In tibial lengthening with an internal device, common side, (2) pelvic tilt with lowering of the pelvis on the short
joint problems include knee flexion contracture and ankle side, and (3) knee flexion on the long side (Fig. 12.28). Short
equinus contracture. In cases in which the osteotomy is at leg gait has the following characteristics (1) stance time
the distal tibia, patients may also develop equino-varus con- reduced on the short side, (2) stride length reduced on the
tracture due to over pull of the tibialis posterior, in addition short side, (3) cadence (steps/minute) increased, and (4)
to the tightness of gastrocnemius and soleus. Dynamic walking velocity marginally reduced. Limb equalization
splints for prophylactic treatment are recommended. CKD reduces these compensatory strategies and gait becomes
bracing with ankle dorsiflexion is useful to manage this. more symmetrical [20].
Knee extension mobilization and simultaneous ankle dors- When working with a child with a congenital cause of
flexion mobilization in addition to subtalar and foot mobili- limb length discrepancy, there are also angular deformities
zation is instituted. Due to the foot being a small lever, the of the bones to consider when evaluating and treating gait
therapist has to use good foot stabilization technique to abnormalities. At the hip, there may be coxa vara or coxa
obtain an effective stretch. As with all patients undergoing valga which can impact hip biomechanics and function of
limb lengthening, it is important to continue to work on the hip abductors. Considering the length-tension relation-
strengthening the quadriceps and hip abductors since these ship of muscles, the abductors rely on the normal femoral
muscles are often atrophied due to prior immobilization and neck-shaft angle to be 120°–135°. When this angle is
limited weight bearing. changed the muscles are unable to provide the trunk with a
stable base of support at the pelvis, leading to a compensa-
tory Trendelenberg gait pattern.
Gait Considerations If there is a fixed flexion deformity of the femur and/or an
anterior pelvic tilt, then the hip extension needed for proper
Children with leg length discrepancy (LLD) may also have propulsion at the end of swing is not available. The child will
skeletal deformities, muscle contractures, joint subluxations instead utilize their lumbar spine to allow their center of
and muscle weakness. In order to equalize their leg lengths, gravity to move over the stance limb. Even with compensation,
196 A. Bhave et al.
Fig. 12.29 Successful outcome for children undergoing limb length-

ening and deformity correction can only be obtained by a carefully
designed protocol for the individual patient that involves daily therapy,
static or dynamic splints and strict adherence to home exercise program
with parent or caregiver participation
from functioning normally in gait and prevents the body’s

weight from advancing over the foot. The knee will lock out
early in gait and will decrease the stride length of the unaf-
fected limb.
Fig. 12.28 Pelvic obliquity compensation for leg length difference. Rotational and angular deformities of the lower leg also
This results in hip abduction of the short limb and hip adduction of the present problems for all of the joints of the lower extremity
longer limb
as the ground reaction forces will become unevenly distrib-
uted at each joint causing abnormal wear and tear and inef-
ficient gait. Normal foot progression is difficult and the
the step length will be shorter on the effected side. An equinus possibilities of running and sporting activities are greatly
contracture further compromises the child’s propulsion. limited the more severe the deformity is.
During normal gait, knee range of motion provides
shock absorption and stability in stance and toe clearance
in swing. When one has a shortened limb, often the knee is Summary
quite stiff in gait, as there is limited need to flex it to clear
the ipsilateral foot. Femoral deformities and small patellae Treating patients with limb length discrepancies before, dur-
can limit quadriceps muscles ability to fully activate. ing, and after limb lengthening can be incredibly challeng-
Couple this with cruciate ligaments deficiencies, and the ing. It takes understanding of the anatomy of the human
affected child will generally lock his or her knee to prevent body and how is interacts in mobility to effectively deter-
buckling and potential injury. Often lack of range of motion mine a proper plan of care for these patients. Customized
at the knee does not affect these patients until their leg therapy (Fig. 12.29) is the greatest tool to overcome the com-
length has been equalized and they need to start functioning mon pitfalls in lower limb lengthening and deformity correc-
more normally. tion to ensure the best clinical outcomes.
Gastrocnemius tightness is certainly the most common
problem at the ankle, leading to difficulty in toe clearance
once the limb lengths are equalized and decreased propul-
References
sion in stance, since it is not able to stretch and recoil nor- 1. Paley D. Problems, obstacles, and complications of limb length-
mally. Congenital problems of the lower leg may present ening by the Ilizarov technique. Clin Orthop Relat Res. 1990;250:
with procurvatum or recurvatum of the distal tibia. If there is 81–104.
procurvatum of the distal tibia, then the patient will be in 2. Moraal JM, Elzinga-Plomp A, Jongmans MJ, Roermund PM,
Flikweert PE, Castelein RM, et al. Long-term psychosocial
excessive dorsiflexion throughout the gait cycle and propul-
functioning after Ilizarov limb lengthening during childhood:
sion will be difficult. With a recurvatum of the tibia, the 37 patients followed for 2–14 years. Acta Orthop. 2009;80(6):
ankle is in a plantigrade position, thus preventing the knee 704–10.
3. Coglianese DB, Herzenberg JE, Goulet JA. Physical therapy 13. Laklouk MA, Hosny GA. Hinged distraction of the hip joint in
management of patients undergoing limb lengthening by distrac- the treatment of Perthes disease: evaluation at skeletal maturity.
tion osteogenesis. J Orthop Sports Phys Ther. 1993;17(3):124–32. J Pediatr Orthop B. 2012;21(5):386–93.
4. Simard S, Marchant M, Mencio G. The Ilizarov procedure: limb 14. Chomiak J, Podškubka A, Dungl P, Ošt’ádal M, Frydrychová M.
lengthening and its implications. Phys Ther. 1992;72(1):25–34. Cruciate ligaments in proximal femoral focal deficiency: arthro-
5. Barker KL, Simpson AHRW, Lamb SE. Loss of knee range of motion scopic assessment. J Pediatr Orthop. 2012;32(1):21–8.
in leg lengthening. J Orthop Sports Phys Ther. 2001;31(5):238–46. 15. Paley D. Intra-articular osteotomies of the hip, knee, and ankle.
6. O’Carrigan T, Nocente C, Paley D, Herzenberg JE. Fractures Oper Tech Orthop. 2011;21(2):184–96.
complicating limb lengthening. J Bone Joint Surg Br. 2005;87-B 16. Walker JL. Pediatric orthopaedics: fibular deficiency. Curr Orthop
(Suppl III):312–3. Pract. 2011;22(2):162–6.
7. Launay F, Younsi R, Pithioux M, Chabrand P, Bollini G, Jouve 17. Venkatesh KP, Modi HN, Devmurari K, Yoon JY, Anupama BR,
JL. Fracture following lower limb lengthening in children: a series Song HR. Femoral lengthening in achondroplasia. J Bone Joint
of 58 patients. Orthop Traumatol Surg Res. 2013;99:72–9. Surg Br. 2009;91(12):1612–7.
8. Currier DP, Nelson RM. Dynamics of human biologic tissues. 18. Schiedel FM, Pip S, Wacker S, Pöpping J, Tretow H, Leidinger B,
Philadelphia: FA Davis; 1992. Rödl R. Intramedullary limb lengthening with the intramedullary
9. Herzenberg JE, Scheufele LL, Paley D, Bechtel R, Tepper S. Knee skeletal kinetic distractor in the lower limb. J Bone Joint Surg Br.
range of motion in isolated femoral lengthening. Clin Orthop Relat 2011;93(B):788–92.
Res. 1994;301:49–54. 19. Bhave A, Baker E, Specht S. Custom knee device (CKD) for the
10. Nogueira MP, Paley D, Bhave A. Nerve lesions associated with treatment of knee flexion contractures (KFC) after ISKD femoral
limb lengthening. J Bone Joint Surg Am. 2003;85-A:1502–10. lengthening. AAOS Annual Meeting Mar 2010, New Orleans, LA,
11. Ilizarov G. Clinical application of the tension-stress effect for limb Scientific Exhibit 60.
lengthening. Clin Orthop Relat Res. 1990;250:8–26. 20. Bhave A, Paley D, Herzenberg JE. Improvement in gait parameters
12. Brech GC, Guarnieiro R. Evaluation of physiotherapy in the treatment after lengthening for the treatment of limb-length discrepancy.
of Legg-Calvé-Perthes disease. Clinics (São Paulo). 2006;61(6):521–8. J Bone Joint Surg Am. 1999;81-A(4):529–34.
Amputation and Prosthetic
Management: Amputation 13
as a Reconstructive Option
John A. Herring
When a child presents with a major lower limb deformity, child with a very short tibia, marked anterolateral bowing,
whether congenital or acquired, many treatment options and a two ray foot can expect to achieve his or her full ath-
must be considered. Modern limb lengthening techniques letic potential with a single surgery and appropriate pros-
with external frames and intramedullary lengthening devices thetic management following amputation done at 11 months
have greatly expanded the treatment. In many cases these of age. (Fig. 13.4a, b) An Ilizarov approach would require
technological advances have enabled surgeons to effectively three or more periods of frame management, with consider-
manage deformities which were untreatable in earlier times. able loss of childhood experiences, with an end result of
In a similar way, amputation and prosthetic strategies compromised function and cosmesis. The decision for man-
have also advanced dramatically in recent years. Over the agement of the child with a deformity in between these two
last several decades a remarkable increase in social accep- extremes is more difficult. The decisions are complex and
tance of visible disabilities has come about. Many factors emotionally laden and require extensive knowledge of all
have contributed to this progress, including the Americans aspects of proposed treatment, and consideration of family
with Disabilities Act, inclusion of Paralympic sports in con- and social dynamics [8, 9].
cert with the Winter and Summer Olympic Games, and an
appreciation for the remarkable achievements of prominent
athletes with limb deficiencies (Figs. 13.1 and 13.2). Treatment Concepts
Children, who in the past sat quietly on the sidelines because
of their “disabilities,” are now competing as varsity athletes, Congenital deficiencies involving the lower extremities pres-
cheerleaders, and team captains, using highly functional ent with varying degrees of severity, some of which are best
state-of-the-art prostheses. treated with amputation. Amputation in this context is often
Initial enthusiasm for limb lengthening reconstruction for one part of a complex strategy involving removal of some
congenital deformities was very high. As the difficulties and bony and soft tissue elements while reconstructing other ana-
complications of such interventions came to light, especially tomic components. When applied appropriately, amputation
those encountered with “heroic” lengthenings, a reconsider- becomes a very positive step in achieving the best functional
ation of the role of amputation has been appropriate [1–7]. and cosmetic outcome for the child (Box 13.1). Conditions in
Let us consider the example of a child born with fibular which amputation is often useful include congenital femoral
hemimelia, or congenital absence of the fibula, and we will deficiency, fibular hemimelia, and tibial hemimelia.
compare Syme amputation to correction with the Ilizarov Amputation for congenital pseudarthrosis of the tibia may be
method. A child with a fibular hemimelia with a well-formed, appropriate after failure of other methods to obtain a stable
four ray foot and a 10 % limb length discrepancy can expect union with a functional extremity, and occasionally amputa-
an excellent result with one tibial lengthening and a contra- tion is chosen as the primary management for this condition.
lateral epiphysiodesis and is not usually a candidate for Deformity and dysfunction following severe trauma may
amputation (Fig. 13.3). At the other end of the spectrum, a also be an indication for amputation, either primarily or fol-
lowing attempts at reconstruction. Primary amputation may
be indicated in young children when major epiphyses are
J.A. Herring, MD, FRCS (Ire, Hon) (*) injured in such a way that future growth is severely limited.
Department of Orthopedic Surgery, The Texas Scottish Rite
Many methods of limb reconstruction are appropriately
Hospital for Children, University of Texas Southwestern Medical
School, 2222 Welborn Street, Dallas, TX 75219, USA used maintain lower extremity function after tumor excision.
e-mail: [email protected] Allograft and internal prosthetic reconstruction have made a

200 J.A. Herring
Fig. 13.1 (a) A young woman with proximal femoral deficiency. Her Lake City. (b) The same woman as she achieves another Paralympic
management was with Symes amputation and knee fusion. She is cele- medal, this time in track cycling. Overall she has now medaled in four
brating her first Paralympic gold medal in three-track skiing at Salt Paralympic games
Fig. 13.3 A boy’s foot with fibular hemimelia. This four-rayed foot
was functional and had good mobility. Note the Symes prosthesis on the
other side, which had a more severe fibular deficiency
Fig. 13.2 Cheetah type carbon blades. These enable highly competi- Box 13.1.
tive performance in a number of sports including track and field, basket- Amputation as a positive event:
ball, and football • PFFD
• Fibular hemimelia
• Tibial hemimelia
• Trauma
positive impact on the management of malignant and aggres-
• Others
sive benign tumors in children. Amputation at times becomes
a solution to intractable complications such as infection and
implant failure after these initial efforts. Amputation remains
the best primary treatment option when the size and location the patient presents with wide-spread disease, and amputa-
of the tumor exceeds the limits of reconstructive options tion can provide pain relief and the ability to ambulate over
[10–18]. In addition, amputation may be appropriate when a shortened life span.
13 Amputation and Prosthetic Management: Amputation as a Reconstructive Option 201
Fig. 13.4 (a) A more severe

fibular hemimelia with marked
shortening and angulation of the
lower limb. The two-rayed foot
was subsequently converted to a
Symes amputation. (b) A
radiograph of the same leg
showing marked shortening and
angulation of the tibia
The comparative costs of prosthetic management and

Patient and Family Management complex reconstruction are difficult to assess. Several stud-
ies have shown conflicting conclusions and consideration of
Management options for these abnormalities also vary all relevant factors is difficult. A most important cost to con-
greatly depending not only on the severity of the condition, sider is the lost childhood or adolescence which occurs with
but also on the available medical expertise and prosthetic multiple episodes of frame application and hospitalizations
support. Early on, the parents should be introduced to and to manage complications [19–21].
educated about treatment methods which involve amputation
as well as those which involve other methods of complex
reconstruction. This education is markedly enhanced when Specific Conditions
such parents trying to make a decision for their child meet
other parents, some of whose children are being managed by Congenital Femoral Deficiency
amputation and others by limb lengthening reconstruction.
These encounters allow parents to ask other parents ques- There is great variation of femoral anatomy among children
tions which they would not ask their doctors, and in fact, with congenital femoral deficiency (CFD), and several clas-
which the doctors are not really very good at answering. sifications are useful. The Gillespie classification [22–24] is
Historically, amputation was a last resort, dreaded by the based on length of the femur at presentation (Fig. 13.5). In
surgeon and feared by the patient and caretakers. As pros- type A the femur is at least 50 % as long as the normal femur,
thetic devices have become more functional and cosmetic, and in type B it is less than 50 % of the normal length. In type
and sports prostheses have allowed people with amputations C there is almost no development of the proximal femur. The
to compete in sports at very high levels, the concept of ampu- classification relates in general to treatment recommenda-
tation as a positive strategy has taken hold in society. Parents tions. The Hamanishi classification [25] illustrates the wide
and children need to be informed about modern amputation variety of anatomic presentations, but is not specifically tied
techniques and prosthetic fitting which provide high function to treatment (Fig. 13.6). The Paley classification [26] adds
with a minimum number of surgical encounters and morbid- consideration of the mobility of the femoral head within the
ity (Box 13.2). They will need the knowledge with which to acetabulum, and is useful for some reconstruction methods
compare amputation to limb lengthening reconstruction, (Fig. 13.7).
typically requiring multiple procedures over time. They should
be provided with honest information about complications Treatment in Gillespie Type A
and outcomes of all methods, frequency and duration of hos- These patients, with at least 50 % of normal femoral length
pitalizations, and the availability and quality of prosthetic care. can usually be managed with lengthening and reconstruc-
tive methods. The outcome is best when the hip is stable and
well formed, and when the upper femoral deformity or defi-
Box 13.2 ciency can be corrected. The greater the shortening of the
Education is essential: femur, the greater is the number of corrective and lengthen-
• By physician ing procedures that will be required. The lengthening pro-
• By other parents (who have had a child with a simi- cess in this condition is difficult and a number of authors
lar diagnosis) report a high frequency of complications and failures
• With psychologic support because of shortening and contracture of all the tissues in
the thigh [27–29].
202 J.A. Herring
Fig. 13.5 The Gillespie classification of femoral deficiency. In type A is almost no femoral length. This image was published in Tachdjian’s
the femoral length is more than 50 % of the normal side. In type B, the Pediatric Orthopedics, Herring JA, Limb deficiencies, 965–974,
length of the femur is 50 % or less than the other femur. In type C, there Copyright Elsevier 2014
Fig. 13.6 The Hamanishi classification of femoral deficiency. This classification notes the multiple variations of the deformity. This image was
published in Tachdjian’s Pediatric Orthopedics, Herring JA, Limb deficiencies, 965–974, Copyright Elsevier 2014
Gillespie Types B and C appropriate (Box 13.3). Patients with bilateral femoral defi-
In these patients the femur is less than 50 % of the normal ciency are almost never candidates for amputation, nor are
length, and lengthening and reconstruction will generally those with major upper limb anomalies requiring the feet for
require three or more episodes of lengthening. Each of these manual activities (Fig. 13.8).
will consume months of time, and complications often The treatment options in CFD can best be understood
increase with successive lengthening. Likewise, the ultimate relative to the presenting anatomy. Factors to consider
function likely decreases as the underlying anatomy is more include (1) the anatomy of the hip and its musculature, (2)
deficient in cases with substantial femoral shortening. Thus, relative shortening of the femur, (3) the range of motion of
in this situation some variation of amputation is usually the knee, (4) the anatomy of the foot. The anatomy of the hip
Fig. 13.7 The Paley classification of femoral deficiency. This classification takes note of hip and knee mobility. This image was published in
Tachdjian’s Pediatric Orthopedics, Herring JA, Limb deficiencies, 965–974, Copyright Elsevier 2014
varies from relatively normal to severe deformity or some-

times absence of the femoral head and acetabulum. When
possible, the anatomy of the upper femur should be recon-
structed to restore bony continuity of the femur and restore
the femoral neck shaft angle. Also, any acetabular dysplasia
should also be corrected (Fig. 13.9). When the femoral head
has not developed, or is immobile in the acetabulum, hip
reconstruction is generally not feasible.
Knee arthrodesis with Symes amputation has been used
for many years and provides good function. The child can be
fitted with a prosthesis at walking age incorporating the foot
in the prosthetic socket, or a Symes amputation can be done
at walking age to facilitate prosthetic fitting. Arthrodesis of
the knee is usually done at an older age, often age 3 or 4
years, when there is significant ossification of the knee struc-
tures. The arthrodesis improves the gait by reducing the
Fig. 13.8 A radiograph of severe bilateral femoral deficiency, Gillespie flexed position of the thigh segment and aligning the
type C, in which there is no femoral formation and the tibias articulate mechanical axis of the limb under the weight line of the
with the pelvis. Treatment alternatives are minimal in these children.
This image was published in Tachdjian’s Pediatric Orthopedics, Herring body. To have appropriate length of the thigh segment after
JA, Limb deficiencies, 965–974, Copyright Elsevier 2014 arthrodesis, the upper tibial epiphysis is usually fused to the
204 J.A. Herring
Fig. 13.9 (a) A Gillespie type B

deficiency with less than 50 % of
femoral length. There is a short
femoral neck with a marked
varus deformity. (b) A radiograph
of the pelvis after proximal
femoral valgus osteotomy and
acetabuloplasty. (c) The same
patient with her prosthesis. Her
anatomic knee is intact and flexes
just at the top of the prosthesis.
She is an active varsity
cheerleader with no functional
limitations. (d) Her shortened
thigh segment is evident as she
sits without her prosthesis
Fig. 13.10 (a) A radiograph of a type B femoral deficiency. Note the

absence of femoral head or acetabular development. (b) The same
patient after arthrodesis of the knee and Symes amputation
Fig. 13.11 A 10-year-old girl with a Van Nes type rotationplasty after
tumor resection
distal femoral metaphysis with removal of the distal femoral
epiphysis (Fig. 13.10). Ideally the end of the thigh segment has a tendency to derotate with growth, and may have to be
should be at least 10 cm shorter than the contralateral knee to repeated.
allow room for the prosthetic knee components. The relative When the proximal hip anatomy is not amenable to recon-
growth of this segment is less than the normal femur by the struction, several procedures have been developed in an
amount of growth of the contralateral distal femoral epiphy- attempt to reduce the abductor limp which is usually quite
sis, since the homolateral epiphysis was removed at the time noticeable. The most successful has been that developed by
of knee fusion. As a practical matter, if the child is reaching Ken Brown in which the femur is rotated 180° and fused to
adolescence and the knees are at the same level with a shorter the pelvis in a vertical position [30, 31] (Fig. 13.11). In this
prosthetic tibial segment, the surgeon should consider position flexion of the rotated knee becomes hip flexion. The
performing a proximal tibial epiphysis on the side of the fusion of the femur to the pelvis, with full rotation turns the
limb deficiency. foot backwards with the toes facing posteriorly. The foot is
As an alternative, rotation of the distal limb to achieve a placed in equinus into a prosthetic socket so that the ankle
Van Nes effect may be done at the time of knee arthrodesis controls the prosthetic knee. When the ankle dorsiflexes the
[30, 31]. When there is no hip stability, the distal segment prosthetic knee flexes and when the foot plantar flexes the
knee extends. The anatomic ankle also provides propriocep-

tion of “knee” position which is an important advantage for
the patient, for example when descending stairs.
Box 13.3.
Options with amputation:
Femoral length <50 %
Hip reconstruction
Knee fusion
Femoral-pelvic fusion
Symes amputation
Rotationplasty
Fig. 13.12 A radiograph of a patient who had a Brown procedure. The
proximal femur with a tumor was removed and the distal femur was
Congenital Fibular Deficiency rotated 180° and fused to the pelvis in an extended position. The staples
are placed to stop the growth of the distal femoral epiphysis
While several classifications of congenital fibular deficiency

(or fibular hemimelia) are used, we prefer the Birch classifi-
cation [32], which is based largely on the severity of foot
deformity and the relative limb length (Table 13.1). In gen-
eral, a patient whose foot can be made plantigrade and which
has three or more rays may be a candidate for lengthening.
When the overall limb length inequality is 10 % or less,
lengthening is preferred. With discrepancies between 10 and
30 %, either method may be appropriate. With discrepancies
greater than 30 %, often requiring more than two limb
lengthening procedures, we usually recommend an amputa-
tion (Box 13.4) (see Figs. 13.4 and 13.12).
Our preferred amputation technique for fibular hemimelia
is a variation of the Symes procedure in which the ankle is
disarticulated without removing either malleolus [33]. The
heel pad remains as an excellent weight bearing structure,
Table 13.1 Proposed classification of congenital fibular deficiency

based on clinical deformity and treatment based on classificationa
Type Characteristic Treatment anticipated
Type 1 (foot preservable)
1A <6 % inequality No treatment or orthosis or Fig. 13.13 A 2-year-old girl treated with Symes amputation for fibular
epiphysiodesis hemimelia
1B 6–10 % inequality Epiphysiodesis ± lengthening
1C 11–30 % inequality 1 or 2 lengthenings ±
epiphysiodesis or extension and the incision is anterior away from the weight bearing
orthosis surface (Figs. 13.13 and 13.14). Some surgeons prefer the
1D >30 % inequality >2 lengthenings or Boyd modification with fusion of the calcaneus to the distal
amputation or extension tibial articular surface.
orthosis
The limb length discrepancy can easily be equalized by
Type 2 (foot nonpreservable)
2A Functional upper extremity Early amputation
the varying the length of the prosthetic limb. A moderately
2B Nonfunctional upper extremity Consider salvage shorter limb is ideal so that there is room for prosthetic foot
a
Shortening is calculated as a percentage relative to the contralateral
components. Most patients can ambulate well without the
limb. In bilateral cases, the longer limb is classified as Type 1A, and the prosthesis and compensate for more severe length discrep-
shorter limb is classified as is done for unilateral cases ancy by walking with the other knee flexed.
206 J.A. Herring
When the proximal tibia is present and the quadriceps

actively extends the tibial segment, as in the Jones type 1b
and 2, a very functional reconstruction is available. The fib-
ula in this instance should be fused to the upper tibia, and the
foot amputated at the distal fibular level, maintaining the
heel pad. In the young child the upper tibial portion may be
mostly formed of cartilage. Fusion of the fibula to the tibial
segment requires enough bone formation in the upper tibia to
accomplish fixation and fusion. In this situation we often
will primarily amputate the foot to enable the child to ambu-
late in a prosthesis with support above the knee. As the bone
formation increases, we perform a fusion of the fibula to the
upper tibia with either pin or screw fixation. Once the fusion
Fig. 13.14 Appearance of a Symes amputation for fibular hemimelia
matures, the child will wear a Symes type prosthesis and
usually function at the same level as the child with fibular
hemimelia (Fig. 13.18).
Box 13.4.
Amputation considered for: Box 13.5.
• Three-rayed foot or less Amputation appropriate for:
• Limb length discrepancy >30 % • Jones 1a (knee disarticulation)
• Optional for LLD 10–30 % • Jones 1b, 2 (Symes amputation, synostosis of tibia
*Never with missing upper extremity function to fibula)
• Jones 3, 4-variable indications
We prefer to perform amputation when the child reaches

the developmental stage of cruising, meaning taking steps The Jones 4 type abnormality has a distal diastasis
while holding on. The prosthesis can be available just as the between the tibia and fibula. Some of these deformities are
child reaches walking age, and very little training is then best managed with Syme amputation and others with recon-
necessary. Within a few hours the child comprehends, almost struction (Fig. 13.19). These abnormalities vary consider-
magically, what this addition to his or her anatomy is there ably, and treatment must be individualized for the given
for, and away the child goes. anatomy. Elements which may favor amputation include
degree of limb length discrepancy, lack of a distal tibial artic-
ular surface, relative size and stiffness of the foot. As with
Congenital Tibial Deficiency other conditions, the choice of treatment is between a com-
plex and multistage reconstruction with an outcome which is
The Jones classification [34] is very useful in the manage- hard to predict and a relatively simple amputation surgery
ment of congenital tibial deficiency (or tibial hemimelia) which requires prosthetic wear for life.
(Fig. 13.15). In the type 1a, in which there is complete
absence of the upper tibia, we consider amputation through
the knee (knee disarticulation) to be the best management Congenital Pseudarthrosis of the Tibia
(Figs. 13.16 and 13.17) (Box 13.5). The older Brown proce-
dure, in which the fibula is centered beneath a bulbar distal In our experience, children with congenital pseudarthrosis of
femur, was widely abandoned after studies showed almost the tibia are candidates for amputation in two scenarios. The
universal long-term failure. Loder and Herring found that more common need for amputation arises following several
these created knees became stiff and non-functional over surgical interventions with either failure to achieve union, or
several years [35]. The femoral length should be monitored when refracture occurs. Often various types of surgery have
as the child grows, and a distal femoral epiphyseal arrest is been performed, and the likelihood of a subsequent proce-
often necessary so that the femur is 6 or 8 cm shorter than the dure being successful is low (Fig. 13.20).
contralateral femur at skeletal maturity. This allows space Over recent years a small number of families have been
for the prosthetic knee component so that the prosthetic and referred to us who desire primary amputation for their young
anatomic knees are at the same level. child. They have reasoned that the child with an amputation
Fig. 13.15 Jones classification

of tibial deficiency. Redrawn
with permission from Herring
JA, Cummings, DR. The Limb
Deficient Child. In: Morrissy RT,
Weinstein SL, editors. Lovell and
Winter’s Pediatric Orthopedics,
vol 2, ed 4. Philadelphia:
Lippincott-Raven; 1996
208 J.A. Herring
Fig. 13.16 (a) A 15-year-old

boy with untreated complete
tibial hemimelias, Jones type 1a.
(b) Incision lines have been
drawn for knee disarticulation
surgery. The suture line does not
impair the distal end bearing skin
In addition, current trends for sports prostheses require sig-

nificant available space for blades and other components that
cannot be used with a longer residual limb.
A modification we prefer for this amputation, which also
we use for traumatic amputations, is to remove a short seg-
ment of cartilage covered bone from the foot to block over-
growth of the tip of the residual limb. We will take a
metatarsal head with a short segment of diaphysis and use
this as an intramedullary osteochondral plug into the distal
tibia. To block the fibular overgrowth we usually use a pha-
lanx from a toe for the same purpose. I should note that we
have no firm evidence at this time of the efficacy of this
maneuver.
Tumor Reconstruction
While a discussion of the available amputation options for

patients requiring amputation for malignancy is beyond the
Fig. 13.17 A Jones type 2 tibial hemimelia. There is a proximal tibial scope of this chapter, certain procedures have proven to be
segment which is powered by the quadriceps and hamstrings. The sub- very functional. For proximal femur resections, the proce-
sequent treatment was a fusion of the tibia to the fibula and a Symes
amputation
dure described by Ken Brown with rotation plasty gives
excellent functional results [30, 31]. The distal femur is
rotated 180° and fused to the pelvis in a vertical orientation.
about the age of first walking will be able to function with The anatomic knee, which is placed at the level of the pelvis,
prosthetic management and minimal future surgery or no functions as a hip with hinge flexion and extension; the ankle
need for protection from vigorous life and sporting activities. and foot power the prosthetic knee. The resultant function is
We have performed such amputations after careful evalua- very good (Fig. 13.21) [37–40].
tion of the child and parents’ understanding. In addition to When a femoral lesion can be excised leaving the proxi-
meeting other children with similar amputations, our psy- mal femur and hip intact, fusion of the tibia to the proximal
chologists also evaluate and educate the family. femur in a 180° rotated position also provides excellent func-
We perform the amputation in this disorder at the level of tion, again using the foot to power the prosthetic knee
the junction of the proximal and middle third of the tibia. (Fig. 13.22).
Some have recommended Syme amputation with the sugges- With tumors distal to the knee, function of the knee joint
tion that the pseudarthrosis may heal [36]. In earlier years we should be preserved, if at all possible. In unusual circum-
had no such healing after this approach, and some patients stances the distal tibia can be “flipped” to be fused to a
had pain at the pseudarthrosis site requiring reoperation. remaining proximal tibial segment (Fig. 13.23).
Fig. 13.18 (a) A Jones type 2

tibial hemimelia before
treatment. (b) Radiograph of the
limb after fusion of the fibula to
the proximal tibial segment. (c)
Appearance of the limb after
fusion is complete. The patient
had excellent athletic function
Fig. 13.19 A radiograph of a Jones type 4 tibial hemimelia with a dis-

tal diastasis between the tibia and fibula. In this instance there was no Fig. 13.20 A radiograph of a congenital tibial pseudarthrosis in a girl
distal tibial articular surface and a diminutive foot, and a Symes ampu- with neurofibromatosis. After a number of procedures she developed a
tation was performed late recurrent pseudarthrosis and underwent trans-tibial amputation
210 J.A. Herring
Fig. 13.21 (a) A radiograph of a

patient treated for a proximal
femoral tumor. Her remaining
femur was rotated 180° and fused
to the pelvis. A revision will
likely be necessary to shorten the
femur and reduce the abducted
position. (b) A photograph
showing the rotated and very
functional foot
Amputation After Trauma
Whenever possible the knee joint should be preserved at the

time of amputation. Exposed, granulating areas on the upper
tibia can be maintained with skin grafting. While some areas
may break down, children have better healing properties
than adults, and there is great functional benefit to maintain-
ing the native knee joint (Fig. 13.24).
Prosthetic Considerations
First Prosthesis
We prefer to perform indicated amputations at the time when

the child is pulling up or beginning to take steps. We then fit
the child with a prosthesis at the time when he or she would
naturally begin walking. When the amputation is at the knee
level or above, the initial prosthesis is constructed without a
knee joint. We usually add the knee to the next prosthesis as
the child is 18 months or older. With bilateral absences we
may wait longer to add an articulated knee to either or both
Fig. 13.22 A radiograph of a patient who had a distal femoral tumor prostheses depending on the child’s walking abilities and the
excised and a fusion of the remaining proximal femur to the tibia,
which was rotated 180°
length of the residual limb.
Volume Changes with Oncology Prosthetic Replacement for Children
Patients undergoing chemotherapy or multiple other proce- Children in their growing years will require new prostheses
dures may experience significant volume changes of their about every 12–18 months. Varying the thickness of socks
residual limb from either weight loss or, conversely, edema. and liners can sometimes prolong the time of use of a pros-
These children may benefit from prostheses with sockets thesis. Children with end bearing residual limbs, such as
which can be expanded and contracted as necessary. knee disarticulations and Symes amputations, can often wear
Fig. 13.23 (a) A radiograph of a

patient who presented with a
painful valgus right knee
deformity. The density in the
proximal tibia was an
osteosarcoma. (b) An AP
radiograph showing a complex
allograft reconstruction of the
tibia. (c) A lateral radiograph
showing a complex allograft
reconstruction of the tibia. (d) A
radiograph showing the allograft
after removal of the fixation
devices. Subsequent infection
and non-union required
consideration for amputation. (e)
The proximal tibia was retained
and the allograft removed. The
distal tibial segment was reversed
allowing fusion of the more
healthy distal tibia to the
proximal segment with pin
fixation
their prostheses well after they have grown a fair amount

(Fig. 13.25). It is not uncommon for a child to prefer the
“old” prosthesis to the “new” one, especially if changes in
design have been made. Gradually, and often after some
modifications, the child will prefer the new limb. When pos-
sible the old limb can be modified to be used for swimming,
water skiing, or other “rough” activities.
Length of the Residual Limb
A major advantage of the Syme or Boyd amputation is the

ability of the patient to weight bear on the residual limb with-
out a prosthesis. This provides the patient with options for
activities without the prosthesis such as swimming, walking
indoors, and mobility when the prosthesis is not available.
Fig. 13.24 A clinical photo of a knee and residual tibia following
severe trauma. The patient had excellent function in spite of the com- The recent availability of prostheses with terminal devices
promised soft tissue coverage such as the spring devices have changed the desires of many
212 J.A. Herring
Fig. 13.25 A young girl

standing beside all of her
previous prostheses. In a growing
child a new prosthesis may be
needed as often as every 12
months
Fig. 13.26 A clinical photograph of a transtibial amputation with Fig. 13.27 A clinical photograph of a transfemoral amputation. In an
marked overgrowth of the fibula. It is remarkable that in spite of bone effort to keep extra length, a segment of bone was covered with split
penetration of the skin, infection is very rare skin graft. The extra femoral length was not needed and the wound
healed after the poorly covered segment was removed
patients. These powerful blades provide the ability to run, Residual Limb Overgrowth
jump, and participate in sports up to and including the
Olympic level, and require 10 or more cm of space below the Overgrowth at the distal end of trans-tibial amputations, as
end of the residual limb. Some patients with well-functioning well as trans-femoral amputations and others, produce annoy-
Symes amputations have returned requesting more proximal ing problems for prosthetic wear. Usually a slender spike of
amputation in order to use such terminal devices. Before bone grows from the distal periosteum and becomes prominent
doing such an operation we make sure that the patient under- or protrudes from the end of the limb. There is usually a bursa
stands the both the functional losses as well as the gains, around this bony spicule, which is filled with fluid and bone
especially relative to losing the ability to walk without the and cartilage fragments. These are exquisitely painful and
prosthesis. often prevent wear of the prosthesis (Figs. 13.26 and 13.27).
The first line of treatment is to resect the overgrowth and 2. Catagni MA, Radwan M, Lovisetti L, Guerreschi F, Elmoghazy
NA. Limb lengthening and deformity correction by the Ilizarov
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surgery and Ilizarov lengthening in fibular hemimelia of Achterman-
tive. Iliac crest bone and cartilage graft has been used for this
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7. Papakostidis C, Bhandari M, Giannoudis PV. Distraction osteogen-
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Schreuder HW, Beishuizen A, et al. Functional ability and physical
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E, Pollock R. Van Nes rotationplasty as a treatment method for
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Working in Resource-Challenged
Environments 14
Scott C. Nelson and Hugh G. Watts
in resource limited environments. The population on this

Introduction planet is now more than seven billion persons—which is
double what it was in the 1970s and quadruple what it was in
Due to the considerable responsibility that we as pediatric the 1930s. Income disparity between wealthy and poor con-
limb deformity surgeons have to our patients and their fami- tinues to increase as well (Table 14.1) [1, 2].
lies, we should never compromise our surgical principles The healthcare resource disparity is even higher at more
based on the circumstances or location in which we perform than 88–1 [3]. Meaning that in places like North America we
operations. The techniques outlined in the chapters of this spend more than 88 times the amount of resources on our own
book have applicability to every part of the world and thus healthcare than what is available for many people in the world.
will not be rewritten in this chapter. The limited resource envi- In the USA and in other developed countries, the primary con-
ronment presents some unique challenges which often create cern is toward the medical care of the aging population. But as
the need for improvisation and innovation, but this must not Fig. 14.1 [4] shows, children make up a much larger portion of
be allowed to compromise our results. Indications and surgi- the population in Africa than in the USA. These two popula-
cal techniques may vary based on the type and severity of tion samples exemplify the general trend in population pro-
limb deformities being treated, but surgical principles should portion between developed and developing countries.
remain the same independent of geographic location. As pediatric limb deformity surgeons, there is an increas-
Never before in history have both patient needs and avail- ing urgency to engage in service where needs are increasing
able treatment options been so great. As globalization, com- and resources are relatively decreasing (Box 14.1).
munication, and technology increase so do our opportunities International relationships and mutual understanding
to develop and maintain international relationships with other between health care providers around the world are neces-
health care providers. This presents possibilities for educa- sary to build sustainable programs that will best be able to
tion and enhances patient care. The potential reasons for the serve the needs of children with limb deformities. This chapter
high prevalence of congenital, post traumatic and post infec- is written both for surgeons wishing to provide humanitarian
tious limb deformities in resource limited locations includes service in foreign countries as well as practicing surgeons
the following: Lack of environmental regulation (teratogenic who are citizens of countries with limited resources.
pollutants in the air, soil and water), lack of education, urban-
ization, transportation-related injuries, natural disaster, lack
of prenatal care, consanguineous relationships, and limited Box 14.1. Factors affecting prevalence of limb
access to health care. The world population is growing fastest deformities in developing countries
• Lack of environmental regulation (teratogenic
pollutants)
S.C. Nelson, MD (*) • Lack of education
Department of orthopedic Surgery, Loma Linda University • Urbanization
School of Medicine, 11406 Loma Linda Drive, Suite 218, • Transportation-related injuries
Loma Linda, CA 92354, USA
• Natural disaster
• Lack of prenatal care
H.G. Watts, MD
Department of Orthopedic Surgery, Shriners Hospital for Children,
• Consanguineous relationships
Los Angeles, 3160 Geneva St, Los Angeles, CA 90020, USA • Limited access to health care

216 S.C. Nelson and H.G. Watts
follow-up period. Personal preparation as well as preparation

International Collaboration of material and supply needs can be optimized by detailed
communication.
Volunteering and Hosting
National and international collaboration between surgeons Commit to a Program

can be a rewarding experience, both for the host and visiting
surgeons and in the end, patient care is improved. Operating As limb deformity surgeons, we will be much more effec-
together with colleagues and other surgeons is a unique ben- tive if we make a long-term commitment to a program in a
efit of doing humanitarian work. Every opportunity to share resource limited environment rather than making first time
knowledge and techniques with others should be sought, as visits to a wide variety of different locations. With repeat
it provides an opportunity for learning and expanding our visits to a specific location expectations are established,
ideas. relationships exist, communication is simplified and work
Good communication is essential to developing a work- can be done more effectively. Also, the value of long-term
ing relationship between surgeons. This starts long before follow-up should be emphasized, both for the patient and
any planned time together and continues on long afterward. for the surgeon. The quest for exploration and adventure
In the era of electronic communication it is now easy to do is always intriguing and may be appropriate in certain situ-
remote preoperative planning, and provide consultation in the ations. However, this should not be the motivating factor
as such notions may dilute the quality and quantity of
Table 14.1 Relative wealth ratio between nations in the
patient care.
upper and lower percentiles of economic prosperity show-
ing a severely progressive disparity between the richest and
poorest countries in the world Common Mistakes Made by Volunteers
Year Wealth disparity
1820 4:1 1. Assume that you are going to teach someone your opera-
1913 11:1 tive techniques. It is important to go with a humble atti-
1950 35:1 tude to first learn and then teach. Often times it is
2002 75:1 surprising how this attitude benefits everyone.
Fig. 14.1 US and African population pyramids. Population pyramids showing the distribution of the population by age in the USA (a) and Africa
(b). 2010 statistics. http://www.worldlifeexpectancy.com/world-population-pyramid
14 Working in Resource-Challenged Environments 217
2. Operate late every night and get as many cases done as to the needed items at their home institution. Asking for
possible. Situations vary and this may not be a mistake in items not needed or that already exist in large quantities
all situations. However, relationships should be ultimately is often frustrating for volunteers who sometimes go to
prioritized over pure production. Consideration must be great efforts to acquire items only to find a large stock of
given to local staff that often repeatedly host visitors and them in the storeroom.
realize that your relationship with them is what will ben-
efit everyone in the long term. As much as it pains the
volunteer surgeon to leave behind a child with a d eformity Understanding Repercussions of Good Work
that could be corrected, it is sometimes best deferred to a
more favorable time for the team and local staff. It may be difficult to understand why, in spite of great need,
3. Use materials and supplies as if you own the place. you are not wanted. This is a common scenario and must be
Opening up multiple packs of sutures, extra gloves, and diplomatically dealt with as human needs sometimes may call
other materials that may not be essential to the operation is for you to help in a situation where you are needed but not
wasteful and may be offensive to the local staff. Many of wanted. It must be understood that our acts of charity can
these items that we take for granted in our own countries have secondary repercussions. For example, how does the
are in short supply and are used very sparingly. Even if local pharmacist feel when we bring in a big box of medica-
they are materials brought by yourself, it is best to respect tion and now his income is undermined? How do the surgeons
the use of these with the local standard of discrimination across town feel when some Americans come and advertise
since these items can be saved and used for future cases. for free surgery? How does the hosting hospital recuperate
4. Make your gifts of materials and supplies the center of costs from increasing the hospital census by 500 % with non-
attention. Bringing equipment and gifts of supplies is paying patients? Although licensing regulations for physi-
really an obligation to have the privilege of operating in cians and import taxes on gifts in kind are often annoying and
resource depleted hospitals. It must be remembered that have been subject to corrupt government processes, it should
volunteers coming to do charity work can be a financial be understood that the basis behind this is to protect the eco-
burden on the hosting institution. Patient volume is often nomic infrastructure of the medical community. There are no
increased as are overhead costs for the hospital. It is won- easy answers to these questions as the needs of the people are
derful if the hosting institution can afford to provide often times dire, and the barriers can sometimes seem insur-
transportation, food and lodging, but in many situations mountable. An important concept to keep in mind when navi-
they simply do not have the resources to do this and the gating these issues is to go with the idea that you are helping
mature volunteer should realize that it is often necessary more than just individual patients. Go with the idea that you
to help with these expenses. are going to help a doctor, an institution, a medical commu-
nity, and a country as well as the individual patient (Box 14.2).
Common Mistakes Made by Hosts

Keep Your Motives Pure
1. Not being responsive to pre- and post-trip communica-
tions. Even in the era of modern communication our lives There are many reasons to do charity work. Travel, adven-
are busy and sometimes important emails and phone mes- ture, learning, publicity, and feeling good about oneself are
sages go unanswered. some reasons that may motivate surgeons to volunteer, but
2. Not having patients prepared and available for surgery. should be put secondary to the sincere desire to provide a
Hosts must understand that a visiting surgical team often needed service in the most effective way. Is it OK to have
times sacrifices thousands of dollars of lost income to ulterior motives as long as we are doing good work and peo-
come and do charity work. It is disrespectful if controlla- ple are getting helped? This is similar to having a conflict of
ble factors have not been appropriately managed to make interest with a particular implant company or letting personal
the most of the trip. economics affect your surgical decision making. Yes, pati
3. Planning operations that are not appropriate for the skills ents can still be helped in a good way, but this can lead to
of the volunteers. This is avoided by adequate pre-trip poor judgment and places the priority on benefiting yourself
communication and/or long-term relationships. In any case more than the patient—a dangerous place to be.
it is essential to know the capabilities of the visiting group.
4. Appropriate equipment exists but it is not available or
missing. It is important to be organized and if equipment Credentialing and Liability
and materials exist then there is no excuse to not know
where they are at the moment they are needed. Many Credentialing and medicolegal concerns are becoming more
times stock is lacking and this can be communicated important issues even in resource limited environments and
ahead of time to the visiting teams who often have access there are a few basic precautions and diplomatic principles
that should be understood. Although it can be liberating to significant damage. It goes to emphasize that in any part of
practice in environments where bureaucracy and liability the world maintaining good communication with patients
issues do not take precedence over patient care, it must be can help to maintain a strong reputation and prevent misun-
remembered that, this freedom should not encourage renegade derstandings and possible litigation.
behavior. Doing what is right for the patient should always be
your guiding principle and you should make efforts to assure
that those working around you realize that. Never should a perative Challenges: Improvise
O
surgeon work outside his expertise and/or perform proce- Don’t Compromise
dures that he is not licensed to do in his home country. As a
guest surgeon respecting the credentialing rules of a host Indications
institution and country is important. Many resource limited
countries have licensing provisions for surgeons coming for In general, operative indications should follow the same
short periods of time to do charity work. The general motive basic concepts in any part of the world. This involves evalu-
of the licensing entity in these countries is to protect the eco- ating whether or not the benefits outweigh the risks. As cir-
nomic viability of local surgeons and make sure that there is cumstances vary, so does the relative relationship between
a minimum standard of quality amongst foreign health care benefits and risks. Efforts should not be spared in trying to
workers. Often times the credentialing process is rather sim- obtain the necessary equipment and expertise to do an opera-
ple or does not exist at all, but no matter what the case, having tion with the very best quality possible. We cannot let incon-
a gracious attitude and building relationships with the local veniences and lack of proper equipment dictate what we do.
staff and administration is an important part of the process. Following the principle of doing it right or not doing it at all
Medicolegal issues are bigger concerns in North America is important no matter where the location (Box 14.3).
than most other parts of the world. Nonetheless a few pre- Economics have to be considered but should not be the
cautions are warranted. The issues vary greatly depending on most important factor. They can have paradoxical implications
the location, type of patients being treated, and organiza- in environments where the reuse of external fixation is com-
tional affiliations. Most legitimate organizations can provide monplace. For example, treating a Blount disease with an
some information on the subject and have provisions in place expensive multiplanar fixator that can be reused many times
to protect volunteer surgeons. Many times in these environ- may be cheaper than using internal fixation. Whereas in North
ments it is the hospital or international organization rather America, those same sophisticated external fixators may be
than the individual surgeon that carries the burden of mal- cost prohibitive for correcting a simple uniplanar deformity.
practice. However, one unique and potentially serious issue The reuse of external fixator equipment can enable the surgeon
is the situation of a US surgeon operating on a US citizen in to operate without regard to the cost of each additional compo-
another country. There have been reports of such cases being nent that is added for strength and/or cosmesis of the external
litigated in the US court system. fixator. Return trips to the operating room for adjustments to
Aside from the subject of formal litigation, disgruntled fixators can be simple and inexpensive in the developing world
patients often access news media and internet venues that without the need for third party payers to approve the process.
provide an easy and cost free way to damage the reputation The indications for limb salvage versus amputation
of doctors and institutions. Whether the allegations are per- deserve mention here as these are complex issues even in our
ceived or real, these types of patients can do own country where we understand the economics and many
of the cultural issues. The relative importance of functional
outcome, cultural views, and cost of reconstructive proce-
dures versus cost of lifetime prosthetic maintenance take a
Box 14.2. Keys to Successful International Collaboration paradigm shift and differ greatly from country to country
• Good communication depending on the availability of prosthetic services. Often
• Develop relationships through long-term times the expectation of performance is different in less
commitments developed environments. In economically privileged coun-
• Be humble—first learn and then teach tries the debate centers on what option is best for high perfor-
• Do not overburden the system mance in sports and long distance running, whereas in
• Respect the local infrastructure economically challenged situations most people would be
• Understand the possible negative repercussions of happy for any type of mobility as long as they can keep their
good work own leg. With that being said, reluctance should be even
• Keep your motives pure—do not do the right thing higher to perform amputations in resource limited environ-
for the wrong reasons ments even if it means the functional result is not as good or
• Do not perform procedures that you are not prop- if a more complex operation or leg lengthening process is
erly qualified to do required. However, multiple attempts to repair a useless limb
need also to be avoided.
In the situation where fluoroscopy is not available, certain and is an efficient and cost effective way to evaluate
procedures are impossible to perform. Indications may preoperative hemoglobin levels (cuvettes cost ~$2 per

change based on whether or not fluoroscopy may be avail- patient depending on quantity purchased). The i-STAT (see
able in the future or at another accessible location or whether Fig. 14.2b) is approximately $10,000 USD, but is able
it is not an option at all. The philosophy of not doing an oper- to perform a variety of tests including CBC, chemistry
ation unless it can be done well should be respected. Many panels, and blood gases (cartridges ~$8–10 for each point
times the lack of fluoroscopy can be compensated for by of care test).
making larger incisions and/or performing more traditional Informed consent is something that should not be
operations such as open, instead of closed epiphysiodeses. forgotten. Even if it does not involve a piece of paper, it is
The Watson Jones anterolateral approach to the hip is another important to communicate to patients, family members and
example of this, allowing visualization of the femoral head even local staff the plan of the procedure and the possible
and neck for cases that could otherwise be done in a more risks and complications. This is especially important for
minimally invasive fashion if fluoroscopy were available. ablative procedures, complex surgery that involves consider-
able risk, and for procedures that involve gradual corrections
and limb lengthening which require additional patient and
Perioperative and Anesthetic Considerations family commitment.
Intra-operative blood loss needs to be minimized and
In areas where an extensive infrastructure does not exist, oper- carefully assessed. It is important for the surgeon to know
ations must be performed with a wider safety margin. This how to calculate the maximum allowable blood loss
calls for a careful preoperative evaluation, additional intra- (MABL). The following formula is used:
operative precautions, and careful postoperative vigilance.
MABL = EBV ´ ( HCL i - HCTf ) / HCTf
Preoperative evaluation should be done both by the sur
geon and anesthesia staff. It should be realized that in areas
where nutritional deficiencies and parasitic diseases are EBV—Estimated blood volume (average blood volume
endemic that anemia can be common. Even when preopera- [ml/kg] × weight [kg])
tive lab evaluations are not routinely indicated in first world Average blood volume
settings, it may be prudent to do additional work up in other Neonates 85 ml/kg
settings. Sometimes in spite of limited resources, local Infants 80 ml/kg
bureaucracy requires seemingly unnecessary testing which Adult men 75 ml/kg
has to be respected by volunteer staff. There are a couple of Adult women 65 ml/kg
portable devices which can be extremely helpful in these HCTi—Initial hematocrit
situations. The HemoCue device (Fig. 14.2a) is a relatively HCTf—Final hematocrit (lowest acceptable at end of
inexpensive piece of equipment (approximately $600 USD) operation)
Fig. 14.2 (a) HemoCue.

Reprinted with permission
of HemoCue America.
(b) I-STAT. Reprinted with
permission from Abbott Point
of Care
Fig. 14.3 WHO surgical safety checklist. WHO surgical safety checklist, URL: http://www.who.int/patientsafety/safesurgery/en, © World Health
Organization 2008 All rights reserved
A healthy patient with a normal starting hematocrit can Care must be taken to apply it as a wide band tight enough to
usually tolerate an acute loss of one third of their blood vol- avoid a venous tourniquet but not overly tight to cause bruis-
ume. If you consider that a total estimated blood volume ing or injury. Any time an Esmarch is used an audible alarm
(EBV) is approximately 70 ml/kg (slightly more in infants must be set in order to avoid inadvertently leaving it placed
and less in adult females) then a short way to estimate MABL beyond 2 h and causing irreversible injury.
is to divide 70 ml/kg by three, which is just over 20 ml/kg. The World Health Organization has created a Surgical
Most patients who are healthy and have normal starting Safety Checklist (Fig. 14.3) in the interest of reducing surgi-
hemoglobin will tolerate 20 ml/kg blood loss without need cal deaths and operative morbidity. This is not meant to be a
ing transfusion. In many situations blood may not be avail- regulatory document but rather a guide to reinforce accepted
able and when it is, often it can take days to obtain. Thus the principles of surgical safety and to promote teamwork
responsibility lies with the surgeon to make sure that there is and communication. The principles outlined here are basic
a wide margin of safety in regards to surgical blood loss and elements for performing safe operations and should be

to always be prepared for the worst possible scenario. implemented in every surgical venue.
By using these calculations, objective and well informed In the postoperative period, it must be remembered that
decisions can be made in regards to performing multiple pain is the fifth vital sign and in North America there is a mind-
procedures in one operation versus staging procedures to set that less is always better than more. This is not actually in
decrease blood loss. This is especially applicable to children the best interest of patient safety especially in an environment
with significant deformities in bilateral lower extremities where sophisticated monitors do not line the walls, nurses may
such as osteogenesis imperfecta, skeletal dysplasias, and not have the latest Pediatric Advanced Life Support creden-
rickets. Using the Esmarch bandage as a sterile tourniquet tials, and the code cart is missing or not well stocked. Until an
is a safe and effective method of controlling blood loss. entire infrastructure can be created to remedy these issues then
patient safety should be prioritized over tolerable levels of Cidex®

pain. Fortunately, in these environments patients expect that
major surgery will involve some pain and they are much more Cidex® is a disinfectant manufactured by Johnson & Johnson
tolerant of it. As surgeons working in this environment we also that comes in three different variations: Cidex® 14-Day
need to be trained and updated on resuscitation credentials (glutaraldehyde), Cidex® Plus 28-Day (glutaraldehyde), and
as we sometimes cannot depend on those around us to do Cidex® OPA (ortho-phthaladehyde). These provide a fast and
the task. effective method of sterilization for a wide variety of instru-
ments and external fixator parts etc. It can be useful for heat
sensitive materials that are not destroyed by submersion.
Sterility When used appropriately Cidex® is bactericidal, fungicidal,
tuberculocidal, and sporocidal. Although it can be quite
Sterility is a principle that should never be compromised no effective, it is very sensitive to dilution, storage methods,
matter what the situation. A brief discussion is warranted expiration dates and duration of use. Similar to opening a
about several options for sterilization, since we as surgeons carton of milk, it has an expiration date and a limited period
are not typically involved in the process and do not have the of use once opened and poured into a sterilization tray. Due
knowledge to assess whether or not various methods provide to this variability, it must not be used indiscriminately and
safe sterilization. In order for something to be considered we do not use it for implant sterilization. There are indicator
sterile with autoclave processing, it must reach 121 °C/250 °F strips available which can be helpful to prove efficacy of
with a pressure of 15 psi/100 kPa for 15 min. Indicators are sterilization.
available and should be used routinely. These are an easy and
compact addition to the list of essential surgical materials
that can be taken on a trip. This can be especially important Ethylene Oxide
in situations where aging autoclaves and maintenance sched-
ules may not be strictly adhered to. If a reliable autoclave is Ethylene oxide, known by the trade name Anprolene®, is
not available some portable stovetop units can be acquired especially useful for sterilizing heat sensitive materials. It is
and their cost is not prohibitive (Fig. 14.4). These units come a highly toxic and explosive gas which was used for making
in various sizes and can accommodate a variety of surgical bombs in World War II. It can provide safe and effective ster-
instruments; the larger sizes can accommodate larger instru- ilization of heat sensitive materials such as gowns, drapes,
ments and implants but are not able to accommodate intact gloves, Esmarch bandages, Ioban®, cautery pencils, suction
full size instrument trays. tubing, bulb syringes, suture packs (Nylon, Monocryl, Vicryl),
and electronic devices (drill, camera, etc.). Most items with
the exception of food, drugs, and liquids can be sterilized
with ethylene oxide. There are some commercially available
processing units that provide ventilation pumps, purge
pumps, aeration, and monitors that track cycle progress.
Special plastic sterilization bags and ethylene oxide ampules
are marketed to be used with these units. For emergencies
and austere conditions these plastic sterilization bags and
ampules can be safely used without the processing units if
appropriate precautions are taken.
Operating Room Set Up
Optimizing every controllable factor includes arranging the

operating room furnishings before an operation in order for
the operation to proceed as smoothly as possible. Many oper-
ating rooms are small and in order to comfortably operate
Fig. 14.4 All-American stove-top sterilizer autoclave. Reprinted with and prevent people from contaminating the field it is impor-
permission of Wisconsin Aluminum Foundry tant to remove all nonessential pieces of equipment from the
room and arrange the remaining furnishings in an ideal • If equipment can be reused:
position to work effectively. Don’t start operations with the –– Inventory is more easily maintained.
C-arm on the wrong side of the table or the cautery unit –– Expenses are minimized.
inconveniently located. Patient positioning and draping • Enables gradual corrections and bone transport in appro-
should follow the same philosophy. The surgeon must take priate situations.
the lead and cannot expect all these things to fall into place • Facilitates intentional displacement of osteotomies that
automatically as may happen with an experienced surgical are performed away from the apex of deformity.
team at their home institution. It is also essential that equip- • Postoperative alignment can be more easily adjusted.
ment outside the operating room is well organized and avail- Another option is to consider the use of Steinmann pins or
able at the moment needed. It is not acceptable to have K-wires for fixation. This is a very economical approach to
equipment on hand that is either not known about or not osteotomy fixation. In extreme situations bicycle or motor-
available for use at the moment needed. Part of being a good cycle spokes can be sharpened with a small bench grinder,
surgeon is to know your equipment and have it prepared to sterilized, and used for fixation. In addition to the biological
implement the preoperative plan and to also be prepared with benefits for making osteotomies in metaphyseal bone, the
a backup plan should the operation take a different direction larger surface area provides more stability and also facili-
than anticipated. This sometimes requires spending almost tates stable pin/wire fixation methods. These can be buried
as much time in the storage room organizing equipment and under the skin if there is a high risk of infection, but this
preparing for cases than is spent in the operating room actu- obligates another procedure for removal. If left in a percuta-
ally doing the cases. neous position then a large portion of wire (2 cm or more for
most situations) should be left outside the skin and gener-
ously bent in order to prevent migration and an otherwise
urgical Modus Operandi in Resource-
S unnecessary return trip to the operating room.
Challenged Environments
Do not exceed the capacity of the local infrastructure with euse of Implants and External Fixation
R
sophisticated operations that will not be correctly followed Components
up. Placing a Taylor Spatial Frame in an environment where
no one knows how to manage it would be an example of this. The reuse of implants (including plates, screws, intramedul-
Also performing highly invasive operations that potentially lary rods, and bone fixation half pins) is not something that
could result in medical complications that the local infra- can be universally recommended due to variability in implant
structure is not prepared to deal with could be disastrous. strength, size, material, and variability in the magnitude
The pros and cons of internal vs. external fixation in a of stress and number of cycles to which it was subjected.
primitive environment have a different dynamic than in the Catastrophic failure can occur when cyclic stresses that
USA. The “one and done” philosophy should be of primary exceed the endurance limit of a metal are applied to an
consideration. This means that gradual corrections, which implant over a given amount of time. The endurance limit is
require diligent follow-up and can involve delayed complica- an amplitude of stress below which it appears that even with
tions, should be avoided when possible. And when internal an indefinitely large number of loading cycles failure will not
fixation can be used, it is usually preferable. occur. The endurance limit is usually about half of the ulti-
mate tensile strength of an implant, unless it is notched,
which can significantly decrease the endurance limit. Some
I nternal vs. External Fixation in Resource- metals such as aluminum do not have an endurance limit and
Challenged Environment even small amounts of cyclic stress will eventually cause
failure. Titanium and the ferrous alloys like stainless steel are
Benefits of internal fixation: materials that do have an endurance limit, meaning that small
• Less postoperative maintenance necessary. amplitude (below endurance limit) loading cycles will theo-
• No pin site infections and less risk of late osteomyelitis. retically not cause failure. Practically speaking, it is not pos-
• Less soft tissue irritation allowing easier range of motion. sible to know the magnitude of stress and number of cycles
• May result in earlier return to function. to which an implant has been subjected nor is it possible to
• Better for treatment for femoral deformities due to less visually decipher this with the naked eye. Ultimately, the
soft tissue concerns. risks and benefits of reusing an implant must be left up to the
• Often does not need to be removed. judgment of the surgeon. If an implant is reused, it is advis-
Benefits of external fixation: able to carefully examine it, and consider using more robust
• Can be applied with minimal incisions. constructs to decrease the amount of stress on an implant
thus increasing the number of cycles to failure. Using com-

mon sense and appropriate reprocessing methods, most
implants can be reused without complication.
The implications of reusing external fixators in resource
limited environments are different from North America due
to perceptions and medicolegal issues. The real question is:
What is safe and effective for the patient? In parts of Europe
(where resources are not severely limited) and in developing
countries fixators are reused on a regular basis. In the USA
many external fixators are labeled “single use only.” Due to
an interest in cost containment and social responsibility
many institutions in North America have sought ways to
reprocess single use devices (SUDs). The Federal Drug
Administration (FDA) has thus classified SUDs into three
categories: noncritical risk (level 1), semi-critical risk (level
2), and critical risk (level 3) [5]. The reuse of fixator clamps Fig. 14.5 12v cordless variable-speed drill
and bars that do not penetrate the skin are considered level 1
SUDs by the FDA. They have approved various reprocess-
ing protocols for these devices. The reprocessing protocols
include cleaning with various solutions to remove all bio-
logic material, dismantling the device, inspection of compo-
nents for defects or fatigue cracks (usually with magnification
or microscope), and re-sterilization.
Biomechanical effects of cyclic loading and patient out-
comes with the reuse of fixators have been studied. Matsuura
et al. report the effects of various magnitude and quantity of
load cycles on the EBI Dynafix external fixator [6]. They
loaded one group with 100–450 N of compressive force for
three million load cycles to simulate three uses of the device.
They found that 17 % of initial stiffness was lost and that
some fatigue cracks began to develop at three million cycles.
Another group was subjected to higher loads and it was
found that fatigue of the device occurred much sooner. Their
advice is that external fixators should only be reused a lim-
Fig. 14.6 Cordless drill kit shown with sterile cover and surgical chuck
ited number of times and should be carefully inspected.
Fixators with more articulating parts certainly are more
prone to failure than simple rods and rings. Also fixators that Cordless Power
are applied to large patients, highly active patients, and fix-
ators that have been left in place for prolonged periods of Many orthopedic operations require a power drill. In limited
time have a higher risk of failure vs. those applied for dam- resource environments the prohibitive costs of the systems
age control orthopedics and have only been left in place for that are on the market have led us to develop a safe and effec-
a few days or weeks. Several clinical studies have been pub- tive alternative (Figs. 14.5 and 14.6). We utilize a common
lished showing no loosening of components, no loss of fixa- hardware store drill with a sterile surgical cover and stainless
tion, no increase in pin site infections, and no mechanical steel surgical chuck. We use a 12-v cordless variable speed
failures when reusing fixators [7, 8]. Even if there is a failure drill with revolutions up to 1,050 rpm. Drills that function at
of an external fixation device it can usually be resolved with rpms above 1,500 in general are not appropriate for orthope-
noninvasive and often times nonoperative means. dics as they create excessive heat and can cause osteonec
In spite of these results, commentaries have been pub- rosis. The stainless steel surgical chuck is custom made in
lished against the reuse of external fixation devices [9] that order to facilitate the use of K-wire placement and conve-
are not scientifically based, do not apply to limited-resource nient loading of various drill bits and it can withstand the
environments and add fuel to medicolegal fears. The reuse of demands of harsh solutions and repeated cycles of steam
external fixators can be safely done if the appropriate repro- sterilization. The system is used with an impervious cover
cessing methods and inspection are implemented. that can also be autoclaved. Theoretically, any drill could be
Fig. 14.7 Post-op plan
made sterile using ethylene oxide and thus not require special the operating note should be made a routine. Most importantly,
covers and surgical grade chucks; however, fluids and bio- the patient must be able to easily access someone who is
materials can easily enter vent holes and damage the internal knowledgeable of the operation and capable of managing
components of the drill. In addition hardware store quality any possible complications. Thankfully the ease and acces-
drill chucks used in this fashion do not stand up to the rigors sibility of international communication allows us to follow
required for appropriate cleaning and reprocessing. up remotely in ways never before possible.
Postoperative Care Other Aspects of Orthopedics in the “Wild”
At the end of any operation, good documentation is essential. Certain surgeons are attracted to a swashbuckling mindset
This includes writing clear orders, an operative note with all that leads them to perform difficult operations in the most
significant aspects of the operation including implants used, austere of conditions. Performing operations without the
and some clear postoperative instructions. We have found it appropriate facilities and equipment should not be seen as a
helpful to use a premade form for a detailed postoperative badge of honor, as it is mandatory to adhere to some mini-
plan that includes dates of follow-up, physical therapy mum requirements for safely performing operations. These
instructions, casting, dressing, and suture removal instr include running water and reliable electricity and lighting, as
uctions as well as any specific needs such as antibiotics well as an intact operating room which provides a well-
(Fig. 14.7). If you are not likely to be present at the time of protected barrier to the outside elements. As previously dis-
follow-up, it is even more important to leave detailed instruc- cussed, safe sterilization, safe anesthesia, adherence to basic
tions that can be easily understood by local staff. Leaving principles of sterile technique and stable orthopedic fixation
your contact information with the local surgeon or directly on are essential. These are factors that the surgeon should have
control over. Surgical risk and the difficulty of performing in these environments, severe clubfeet are present in older
operations are increased when doing surgery in the “wild.” children, and those who have had prior surgery. For this rea-
Some of these factors are controllable and others are not. son a review of the surgical options for clubfoot is briefly
Factors such as biology, patient compliance, and limitations described here.
in available resources and personnel are largely uncontrolla- The Ponseti treatment for clubfoot has revolutionized the
ble. The surgeon must optimize all controllable factors and treatment of clubfoot around the world. The results of this
take extra precautions to assure patient safety and stable fixa- treatment have been shown to be superior to previous meth-
tion in spite of other uncontrollable risk factors This may ods of casting and/or surgery [10–12]. The most rapid cor-
mean added points of fixation—extra bone pins on a fixator rections with Ponseti casting are seen in newborns; however,
or more robust plating patterns than would otherwise be nec- the technique continues to be effective in older children as
essary. Often times, communication is difficult and follow- well. When performing casting, it is important to follow all
up is scant; thus, it is necessary to build constructs not prone of the details of the Ponseti technique to get the very best
to failure and take extra precautions in order to avoid compli- results. These include: supinating the forefoot to lock the
cations. This also involves extra efforts communicating with metatarsal cuneiform joints in order to concentrate forces at
local staff and patients. the subluxed talonavicular joint, placing long leg casts in
order to provide sufficient abduction of the forefoot, and per-
forming percutaneous Achilles tenotomy in nearly all cases.
Box 14.3. Improvise—Don’t Compromise Neglected and recurrent clubfeet are a common cause of
• Techniques may need to be modified in situations disability in developing countries. The stigma of this defor-
of limited resources but surgical principles and mity often prevents those who have it from going to school,
expected outcomes should never be compromised integrating into society, and having a job. In older children
• Understand the capabilities and resources of your and adolescents the correction of the deformity has been
anesthesia team shown to be worthwhile for social reasons in spite of the fact
• Do not perform operations whose complexity that often times it does not restore normal mobility and shape
and risk exceeds the capacity of the post operative to the feet [13].
infrastructure The excellent results and logistics of effectively imple-
• Be extra careful about surgical safety while work- menting the Ponseti method add complexity to surgical deci-
ing in environments that may not have implemented sion making when patients in limited resource environments
the safety measures you are accustomed to are unable to return for weekly casting, or when short term
• Opioids must be used judiciously in environments humanitarian surgical trips do not permit the luxury of pro-
lacking appropriate monitoring, resuscitation equip viding treatment over time. Ultimately, the best outcome for
ment and properly trained personnel the child is what should be sought. The surgeon must remem-
• Principles of sterility and sterile technique should ber that no child has ever died just because they had a club-
never be compromised foot, and that a poor surgical result is worse than a deformity
• Make sure your equipment is prepared and your not previously operated. Thus immediate operative treatment
operating room properly arranged before embark- may not be in the best interest of all patients. Every possible
ing on an operation effort should be made to cast clubfeet even if an operation
• Principles of stable fixation should not be compro- will ultimately be necessary. In situations where casting
mised and constructs should often be made more is logistically difficult, an accelerated program has been
robust if limited follow-up is expected described where casting is done every 5 days and has been
• Economize shown to give similar results to the typical 7-day protocol
• Be flexible [14]. Even faster protocols have been tried but are not recom-
mended due to the amount of swelling that can occur and
diminishing returns in speed of correction.
In cases where operations are required, casting can be a
Specific Techniques helpful preoperative adjunct to surgery. Sometimes even just
a few days of casting can improve operative results, decrease
Clubfoot wound complications, and facilitate the ease of an operation.
A hybrid approach can be taken where a minimal release
Clubfeet seen in resource limited environments are not (including posterior tibialis tenotomy, plantar fasciotomy,
usually like the ones typically seen by surgeons in North and Achilles lengthening) is combined with preoperative and
America. In addition, the success of Ponseti casting is mak- postoperative casting for cases where extensive casting is
ing the operative treatment of clubfoot a lost art. Often times difficult or not practical. There is much debate about the
Table 14.2 Surgical options for neglected and recurrent clubfoota

Posteromedial release Soft-tissue release for cavus, adductus, varus, • Cincinnati
and equinus • 2 incision
• Turco
Additional procedures Treatment of residual adductus • Calcaneal closing wedge
• Lichtblau calcaneal resection
• Calcaneo-cuboid wedge resection/arthrodesis
Treatment of residual equinus • Excision head of talus
• Naviculectomy
• Distal tibia osteotomy
Treatment of residual heel varus • Calcaneal osteotomy (lateral slide)
Treatment of residual supination • Tibialis anterior transfer
• Medial cuneiform osteotomy
• Trans cuneiform osteotomy
Wound closure difficulty • Leave wound open
• Immobilize under-corrected then serial
casting postoperatively
• Dorsal foot rotational flap
• Distal tibia shortening osteotomy
Salvage procedures (for severe Older child with stiff/severe deformity • Triple arthrodesis—Lambrinudi modification
and recurrent clubfoot) • Posterior release with midfoot dorsal closing
wedge
Arthrogryposis and myelomeningocele • Talectomy
External fixation Ilizarov or Taylor Spatial Frame for recurrent/ • Soft-tissue distraction
residual deformities and severe clubfoot • Distraction osteogenesis (osteoplasty)
Adapted with permission from Penny NJ. The neglected clubfoot. Tech Orthop 2005; 20(2):153–166
a
maximum age limit for effective casting. In reality there is no like triple arthrodesis can be performed at an age closer to
specific age at which casting becomes ineffective; although skeletal maturity. The reader is referred to the excellent arti-
the time and materials required to correct the clubfoot defor- cle by Norgrove Penny for a detailed description of the
mity can significantly increase with age and stiffness of the options and techniques for neglected clubfeet [15].
foot. This is due to progressive ossification of the foot bones. Previously operated and neglected clubfoot can also be
As the child becomes older there is less cartilage and less managed with the use of external fixation. Two basic meth-
remodeling potential in the foot. ods of foot deformity correction using Ilizarov techniques
Posteromedial release has traditionally been a standard are: (1) soft tissue stretching (correcting through joints) and
treatment for clubfoot. Due to the successful implementation (2) bony correction (osteotomies) with distraction osteogen-
of the Ponseti method in North America this operation is esis. Due to the improved ability to correct rotational defor-
becoming less common. However, in severe, recurrent, or mity with Taylor Spatial Frame (TSF), we have applied
neglected clubfoot, especially in older children, it is still a this to clubfeet in a fashion that simulates Ponseti casting
viable option. For children with significant adductus, pos- (correction through soft tissue). Other fixator configurations
teromedial release can be combined with a lateral column with or without osteotomies are also effective. Any of these
shortening osteotomy (a Lichtblau anterior calcaneal resec- methods require experience and an infrastructure that can
tion, calcaneal cuboid wedge resection, or calcaneal shorten- support close follow-up and postoperative management. One
ing osteotomy). Other options for residual deformities of the biggest risks with external fixation is the risk of recur-
including residual equinus, heel varus, or forefoot supination rent deformity. The long treatment time, possibility of persis-
are outlined in Table 14.2. In older children with severe tent hyperesthesia, and other complications need to also be
deformities, triple arthrodesis is often necessary. This can be considered when entertaining the use of gradual distraction
done in children as young as 8 years old, especially in bilat- for surgically treating older children.
eral cases where a foot size discrepancy would not be cre-
ated. Another option that has been performed with success
for severe cases is a posterior release with a dorsal closing SIGN Nail
wedge midfoot osteotomy. The temptation to correct recur-
rences and residual deformity, particularly in the 5- to The SIGN nail (Surgical Implant Generation Network, www.
12-year-old age group, needs to be weighed against expected signfracturecare.org) is an intramedullary locking nail which
results, risk, and recuperation time. In many cases it is best to can be implanted without fluoroscopy. It is made by a non-
avoid repeated surgery and wait until a definitive operation profit organization for use in limited resource environments
Fig. 14.8 Hip spica application.

After appropriate anesthesia, the
patient’s torso is placed supine
on the end of the armboard.
Three assistants are used to
stabilize and position the
patient’s arms/head region and
each of the lower extremities.
Standard casting materials,
including a temporary towel to
pad the patient’s back, are used
to complete the hip spica cast.
Reprinted with permission from
Pasque C, Harbach G. Hip spica
application using an operating
table armboard. J Pediatr Orthop
2000; 20(6)
and made available to surgeons and institutions at minimal like a diving board. One person stabilizes the proximal end
cost. This has revolutionized long bone fracture care around of the board onto the table as well as the torso of the child
the world with more than 120,000 cases performed. SIGN who is placed over the side of the bed on the opposite end of
has established programs in more than 55 countries where the board. Bilateral and unilateral spica casts can be placed
surgeons have had the necessary training and experience. on any size child with the appropriate size arm board.
Accountability, quality control and maintenance of a sustain-
able inventory are realized through the use of a database that
requires uploading of preoperative and postoperative images Educational Resources
as well as input of case data and implant use. There is a
learning curve to using the system, which has some unique Having a collection of orthopedic textbooks available on a
features enabling distal interlocking without X-ray guidance. laptop or iPad is very helpful when performing orthopedics
This system is useful in limb deformity correction when stain resource-challenged environments. Many times demands
bilizing osteotomies or even performing lengthening over are made to perform difficult operations that one may not
nails. Fixator assisted nailing is also a useful technique for a commonly perform. The surgeon must first assess his/her
variety of long bone deformities. There is a pediatric version own abilities and again remember that a poorly performed
of the SIGN nail available; however, the standard nail is surgery is best not performed at all. But a review of operative
suitable for a trochanteric entry and may be used in older details and often a consult from a colleague can remind one
children. The pediatric nail is more useful for fractures and of the details needed to efficiently perform a quality opera-
has a unique distal interference fit without a distal interlock- tion and prepare the proper equipment for a variety of
ing option. The standard adult nail comes in sizes as small as operations that you may not routinely perform. Fortunately,
8 × 200 mm, making it versatile for deformity corrections in most textbooks are now available in electronic formats
relatively small patients. which can allow one to maintain an entire orthopedic library
on a small electronic device. The Global Help Website www.
global-help.org has a wide variety of textbooks and mono-
Spica Cast graphs available for free. They have an excellent monograph
on the Ponseti treatment of clubfoot which has been trans-
Many pelvic and femoral osteotomy surgeries involve the lated into more than 20 languages and serves as an educa-
use of a postoperative spica cast. It is mentioned here to tional tool both for surgeons and parents. Other patient
highlight the fact that a specialized spica table is not neces- specific educational materials are available on this site as
sary nor may it be available in many resource challenged well. An excellent resource is their “Pediatric Orthopedic
environments. Spica casts can be applied on a simple arm Library,” which on a single disc contains the Atlas of
board (Fig. 14.8) [16]. A plank of wood or other material ide- Pediatric Orthopedics Surgery (3rd Edition) (Morrissy
ally about ½″ thick and about 4″ wide can be slid under the and Weinstein); Bibliography of Orthopedic Problems in
pad on the operating table and be used for both an arm board Developing Countries (Spiegel); Lovell & Winter’s Pediatric
and spica table. In order to do this, the board is placed at 90° Orthopedics (5th Edition) (Morrissy and Weinstein); Neuro
to the table under a thin mattress and sticks out to the side muscular Disorders; Practice of Pediatric Orthopedics
(Staheli); Sequelae of Septic Arthritis of the Hip (Spiegel,

Penny, and Shrestha); Standards in Pediatric Orthopedics References
(Hensinger); The Easter Seal Guide to Children’s
Orthopedics (The Easter Seal Society); What Parents Should 1. Sachs JD. The end of poverty: economic possibilities for our time.
New York, NY: Penquin Books; 2005. p. 28.
Know About Flatfeet (Staheli, Staheli, and Mosca). The 2. Jimmy Carter – Nobel Peace Prize Laureate Lecture. Jimmy Carter
DVD cost is $1.00 USD. This allows a traveling surgeon to Library and Museum, Stockholm. http://www.jimmycarterlibrary.
carry a huge library and moreover offer an invaluable gift to gov/documents/jec/nobel.phtml. Accessed Dec 2002.
the local surgeons for an extremely low cost. 3. World Health Organization. World health statistics 2007. Geneva:
WHO; 2007. p. 19.
HINARI (Health Inter Network Access to Research 4. World life expectancy charts of the United States of America
Initiative) is a World Health Organization program which and Africa. 2010 statistics. http://www.worldlifeexpectancy.com/
provides free or very low-cost online access to up to 35,000 world-population-pyramid.
information sources/journals in biomedical and related social 5. Department of Health and Human Services, Food and Drug Admi
nistration. Medical devices; reprocessed single-use devices; termi-
sciences to local, nonprofit institutions in developing coun- nation of exemptions from premarket notification; requirement for
tries. This functions as an institutional subscription to jour- submission of validation data. Fed Regist. 2003;68:38071–83.
nals referenced by PubMed etc. An application process is 6. Matsurra M, Lounici S, Inoue N, Walulik S, Chao EYS. Assessment
required, and in the most resource challenged countries, sub- of external fixator reusability using load- and cycle-dependent tests.
scriptions are completely free of charge, whereas in other 7. Dirschl DR, Smith IJ. Reuse of external skeletal fixator components:
developing countries a small fee is assessed. More informa- effects on costs and complications. J Trauma. 1998;44(5):855–8.
tion and application materials can be obtained at www.who. 8. Sung JK, Levin R, Siegel J, Einhorn TA, Creevy WR, Tornetta
int/hinari/en/. P. Reuse of external fixation components: a randomized trial.
J Orthop Trauma. 2008;22:126–31.
9. Beck DJ, Seligson D. External fixator parts should not be reused.
J Orthop Trauma. 2006;20:39–42.
Summary 10. Halanski MA, Davison JE, Huang JC, Walker CG, Walsh SJ,

Crawford HA. Ponseti method compared with surgical treatment
of clubfoot: a prospective comparison. J Bone Joint Surg Am.
Working with limited resources and often times in unfamiliar 2010;92:270–8.
locations can be some of the most challenging but also most 11. Richards BS, Faulks S, Rathjen KE, Karol LA, Johnston CE, Jones
rewarding experiences in life. Many of these operations SA. A comparison of two nonoperative methods of idiopathic club-
foot correction: the Ponseti method and the French functional
are life changing for the patients we treat. Basic principles
(physiotherapy) method. J Bone Joint Surg Am. 2008;90(11):
should never be compromised; every effort should be made 2313–21.
to obtain outcomes equal to those where ample resources 12. Ippolito E, Farsetti P, Caterini R, Tudisco C. Long-term compara-
exist. This often requires more robust fixation, extra precau- tive results in patients with congenital clubfoot treated with two
different protocols. J Bone Joint Surg Am. 2003;85-A(7):1286–94.
tions to avoid complications, and clear communication.
13. Penny A. School access: children with motor disabilities in rural
As orthopedic surgeons with education and resources, we Uganda. MA thesis dissertation, University of Victoria; 2001.
should consider it our obligation to help those who are in 14. Morcuende JA, Abbasi D, Dolan LA, Ponseti IV. Results of an
need regardless of their ability to compensate us. This can be accelerated Ponseti protocol for clubfoot. J Pediatr Orthop.
2005;25:623–6.
in our own country or another. By going beyond stagnate and
15. Penny NJ. The neglected clubfoot. Tech Orthop. 2005;20(2):153–66.
repetitive routines, new perspectives are gained, new rela- 16. Pasque CB, Harbach GP. A pictorial essay: hip spica application using
tionships are built and lives are changed. an operating table armboard. J Pediatr Orthop. 2000;20:757–8.
Part III
Underlying Conditions
Metabolic Disorders
15
Mehmet Kocaoglu, I. Levent Eralp, and F. Erkal Bilen
General Box 15.1

• Metabolic problems related to vitamin D must be
Deficiency in the shape, strength and structure of bone tissue addressed prior, during, and after the surgical
due to altered bone mineral homeostasis is called metabolic treatment.
bone disease (1). The major factors affecting this homeosta- • There are various forms of metabolic bone diseases.
sis can be thought of as a three 3’s: the intracellular and Thus, collaboration with the pediatric/internal med-
extracellular levels of three ions (calcium, phosphorus, and icine department is essential.
magnesium), which are controlled by three hormones (para-
thyroid hormone, calcitonin, and 1, 25-dihydroxyvitamin D)
and act upon three tissues (bone, gut, and kidney) [1, 2].
Common clinical manifestations of metabolic bone disease seal growth and mineralization of the bone matrix in the
in children include electrolyte disturbances, fractures, bone growing child [1, 3, 4]. This decrease in the mentioned serum
deformity, abnormal gait, and short stature. ion levels may result from inadequate intake or diminished
The most commonly encountered forms of metabolic absorption of phosphorus, calcium or vitamin D, reduced
bone disease in children are the various types of rickets and conversion of vitamin D to its active form, end-organ insen-
renal osteodystrophy. Other less common but important sitivity, impaired release of calcium from bone, or phosphate
pediatric metabolic conditions include osteoporosis, wasting. In addition, there is evidence that insufficient vita-
malabsorption syndromes, inherited diseases such as hypo- min D may interfere with mineralization independent of cal-
phosphatasia, X-linked hypophosphatemia, and various cium or phosphate levels [1, 3].
forms of vitamin D-dependent rickets [1, 3] (Box 15.1). In renal osteodystrophy, glomerular damage leads to
phosphate retention, and tubular damage causes decreased
production of the active form of vitamin D (i.e., 1,25-
Pathophysiology dihydroxyvitamin D) due to absence of 1-hydroxylase
activity. These two factors severely impede intestinal calcium
Although there are several types of rickets, the basic patho- absorption and reduce plasma ionized calcium. The subse-
genesis is a relative decline in calcium or phosphorus (or quent hypocalcemia generates secondary hyperparathyroid-
both) of large enough amount so that it interferes with phy- ism, which remains ineffective in increasing intestinal
absorption of calcium. Consequently, the body’s only means
of increasing serum calcium levels is by bone resorption.
Metabolic acidemia may further deteriorate this condition
M. Kocaoglu, MD • F.E. Bilen, MD, FEBOT
Department of orthopedics and Traumatology, (Figs. 15.1 and 15.2) [1, 3, 4].
Istanbul Memorial Hospital, Piyalepasa Bulvari Okmeydani, In patients with osteoporosis, the bone is structurally nor-
Istanbul, Marmara 34385, Turkey mal but is reduced in overall amount. In children, osteoporo-
e-mail: [email protected]; [email protected] sis may be idiopathic, as in juvenile osteoporosis, or may be
I.L. Eralp, MD (*) due to disuse or chronic corticosteroid administration. The
Department of orthopedics and Traumatology, Istanbul Medical mechanism is uncertain, but numerous theories include
School, University of Istanbul, Hakki Yeten Cad. No: 14, Terrace
Fulya Center 1 D: 83 – Fulya, Sisli, Istanbul 34365, Turkey increased bone resorption versus decreased bone formation,
e-mail: [email protected] possibly due to deficient 1,25-dihydroxyvitamin D or calcitonin

232 M. Kocaoglu et al.
Fig. 15.1 Metabolic control of calcium levels
Fig. 15.2 Vitamin D metabolism
or due to a major interruption in transduction of mechanical maturation of the primary spongiosa in the physis. This con-
forces that stimulate new bone formation [1, 3]. dition leads to normal production of osteoid tissue but inad-
Hypophosphatasia results from a genetic error in the syn- equate mineralization, with resultant skeletal deformities
thesis of alkaline phosphatase, the enzyme necessary for the that resemble rickets [1, 3, 4].
15 Metabolic Disorders 233
Nutritional Rickets Box 15.2

• Metabolic bone diseases can be caused by certain
General medications as well.
• Remember that certain antiepileptics such as val-
The primary etiology of this condition is vitamin D defi- proic acid and phenytoin may lower vitamin D
ciency in the diet. This deficiency leads to failure of calcifi- levels in the body.
cation of cartilage and osteoid tissue. Vitamin D deficiency
results in an inability to absorb calcium and phosphorus.
Parathormone (PTH) is released in response to hypocalce-
mia. Subsequently, serum calcium levels become normal or Vitamin D-Resistant Rickets (Familial
slightly decreased, but phosphate and vitamin D levels Hypophosphatemic Rickets)
remain low (Table 15.1). Thus, radiologically, the physes
display an elongation and a hazy appearance related to alter- General
ations in the provisional zone of calcification [4, 5]. The wid-
ened growth plate differentiates rickets from more common Vitamin D-resistant rickets involves a group of disorders in
physiologic angular deformities of the lower extremity [6]. which normal dietary ingestion of vitamin D is insufficient to
With treatment, calcification occurs and radiographic appear- accomplish normal mineralization of bone [2, 6]. There are
ance gradually normalizes. four major forms of vitamin D-resistant rickets, the most com-
mon of which is inherited as an X-linked dominant trait, fol-
lowed in occurrence by an autosomal dominant type [2, 4, 6].
Treatment The inherent pathology is the renal tubule’s incapability to
retain phosphate, which causes hypophosphatemia. The third
The routine treatment for nutritional rickets include the group is characterized by failure of the kidney to accomplish
supplemental administration of vitamin D. Radiographs the second hydroxylation of vitamin D. This condition can
typically show improved mineralization within 2–4 weeks simply be treated medically, therefore orthopedic treatment is
of initiating medical treatment [4, 6]. If the child does not infrequently needed. In the fourth group, also known as renal
respond to vitamin D therapy, vitamin D-resistant rickets tubular acidosis, the kidney excretes fixed base and wastes
should be suspected. Because residual deformity is very bicarbonate resulting in wasting of calcium and sodium [3, 6].
rarely observed after adequate medical treatment of nutri- Laboratory findings are listed in Table 15.1.
tional rickets, there is no specific orthopedic treatment Vitamin D-resistant rickets typically becomes evident
besides follow-up to ensure acceptable lower limb between the ages 1 and 2 years, slightly older than nutritional
alignment. rickets. The major complaints are delayed walking and angu-
lar deformities of the lower extremities (Figs. 15.3 and 15.4).
Systemic manifestations are generally absent. The deformi-
Rickets of Prematurity ties are much more severe when compared to nutritional
rickets. Once affected children begin to walk, genu varum
Premature infants with comorbidities, followed up in inten- develops, although genu valgum may occur in some children
sive care units, sometimes present with pathologic fractures, [4, 6]. Short stature is also a feature of hypophosphatemic
probably caused by passive motion exercises. With treatment rickets, with their standing height often being 2 standard
of the underlying rickets, the fractures consolidate with deviations below the mean for their peers [8]. Radiologically,
orthopedic immobilization techniques [4]. the physes are widened, there is genu varum and coxa valga,
Furthermore, a varus deformity of the distal tibia often leads
to varus malalignment of the ankle joint (Fig. 15.5). The
Drug-Induced Rickets upper extremities are involved, to a lesser degree, as well [4].
Certain antiepileptic medications, such as valproic acid and

phenytoin, have been known to create rachitic changes in chil- Treatment
dren [7] (Box 15.2). These drugs lower vitamin D levels
through a P-450 microsomal enzyme system mechanism in Medical Treatment
the liver. Patients mostly present with pathologic fractures The standard treatment consists of oral supplementation of
while on treatment for seizures. Medical therapy with vitamin large doses of phosphorus along with administration of vita-
D supplementation and consultation with the treating neurolo- min D. Studies have revealed that longitudinal growth is
gist is helpful. superior in children who receive vitamin D treatment [4].
Table 15.1 Biochemical abnormalities in rickets

Biochemical abnormality
Type of metabolic bone disease Calcium Phosphate ALP PTH 25 (OH) Vit D 1,25 (OH)2 Vit D
Nutritional N N/↓ ↑ ↑ ↓↓ ↓
Vitamin D resistant N ↓ ↑ N N N
Vitamin D dependent I (inability to hydroxylate) ↓ ↓ ↑ ↑ ↑↑ ↓↓
Vitamin D dependent II (receptor insensitivity) ↓ ↓ ↑ ↑ N/↑↑ ↑↑↑↑
Renal osteodystrophy N/↓ ↑ ↑ ↑↑ N ↓↓
Fig. 15.3 Photograph of a patient with severe genu valgum deformity Fig. 15.4 Photograph of a patient with severe genu varum deformity
Furthermore, treatment by growth hormone administration alignment, multilevel osteotomies are often necessary [4, 11,
also increases height and has positive influence on bone den- 12]. The mechanical axis can be slightly overcorrected dur-
sity [9]. Recently, analogs of vitamin D3 (1,25-dihydroxy- ing surgery. The suggested fixation modality varies among
vitamin D3) have been proved to be more effective than the reports. While external fixation allows fine-tuning of the
previously used supplements [10]. alignment postoperatively [13] (Fig. 15.8), intramedullary
fixation and plate fixation have also been reported [12, 14–
Orthopedic Treatment 16] (Fig. 15.9; Table 15.2). Regardless of type of implants
The orthotic management of skeletal deformities in patients utilized, careful preoperative planning of the surgery for
with vitamin D-resistant rickets has not been successful. these multiplanar deformities is obligatory to successfully
If patients complain of increasing pain and difficulty walk- reestablish the alignment of the lower extremity.
ing, angular deformities should be surgically corrected [4, Recurrent deformity is a common sequela of osteotomies
11]. The postoperative management should be done in close in patients with hypophosphatemic rickets [4, 12, 14]
collaboration with the attending nephrologist or endocrinol- (Fig. 15.10). As expected, younger patients have a higher
ogist, since calcium levels tend to suddenly increase with risk of recurrence. Therefore, milder deformities should not
postoperative immobilization period. The most common be operated early in the infancy. At this stage guided growth
deformity seen in this patient group is a gradual anterolateral by hemiepiphyseodesis is an advisable alternative [17]. Only
bowing of the femur accompanied by tibia vara (Figs. 15.6 when gait is compromised by a thrust, or symptoms such as
and 15.7). In order to reach a physiologic lower extremity pain start, a corrective osteotomy should be done.
Fig. 15.5 X-ray showing distal tibial varus deformity Fig. 15.7 Photograph of a patient with severe anterior bowing of the
femur
Fig. 15.6 X-ray of a patient with a anterior bowing of the femur Fig. 15.8 X-ray of external fixation for the tibial deformity
We use the fixator-assisted nailing technique described by

Paley and Herzenberg [19], with osteotomies created at the
center(s) of the deformity(ies), followed by correction using
a monolateral fixator and stabilization with locked intramed-
ullary nailing (Figs. 15.13, 15.14, and 15.15). Removal of
the external fixator at the end of the operation reduces post-
operative discomfort and avoids pin-track infections, except
where patients require additional postoperative lengthening
over the nails. However, this technique does not allow resid-
ual correction or adjustment postoperatively. Thus, careful
analysis of the deformity and preoperative planning are cru-
cial. We recommend using this technique for the patient,
who has essentially reached skeletal maturity. Flexible intra-
medullary nails (elastic nails) can be used in the pediatric
age group [18].
Osteotomies in the long bones can be executed through
limited incisions percutaneously either by the Gigli saw
technique (Fig. 15.16) or by the multiple drill hole technique
(Fig. 15.17). This technique combines the accuracy, minimal
invasiveness, and safety of external fixation with the patient
convenience of internal fixation. The intramedullary nail pre-
vents the recurrence of the deformity, which is especially
important in patients with metabolic bone diseases who are
prone to recurrence of the deformity as the metabolic prob-
Fig. 15.9 Intramedullary fixation of a tibial deformity correction lem continues. In addition, the surgeon must be familiar with
both external fixation and intramedullary nailing techniques,
Short stature is also noted amongst children with hypo- as well as combined techniques, which can be technically
phosphatemic rickets. The common indication for long bone demanding.
lengthening is a shortening of the entire or segment of limb
in one or both legs [11]. The procedure can be performed by Surgical Technique
monolateral or circular external fixators or by lengthening The patient is placed supine on a radiolucent table and the
over an intramedullary nail (LON) (Fig. 15.11) [12]. The lower limb is checked for imaging on the radiography table
application of flexible intramedullary nails in limb lengthen- from the hip joint to the ankle joint on both frontal and side
ing for children is also an alternative with multiple advan- views (Fig. 15.18). In patients with genu valgum, resulting
tages [18]. This technique also respects the bone biology that both from the femur and/or the tibia, mini-open release of
is essential during the limb lengthening. the peroneal nerve is performed prior to correction of the
deformity to avoid neurapraxia due to traction. Two 6-mm
Author’s Preferred Method, Tips and Tricks conical Schanz screws are placed into the proximal and
The pathological change caused by hypophosphatemic rick- distal segments above and below the osteotomy to maintain
ets occurs very close to the growth plate, often leading to the stability, perpendicular to the anatomical axis, taking
juxta-articular multiplanar deformities and severe malalign- care to leave enough space for the inserted intramedullary
ment. Preoperative assessment usually reveals multiple cen- nail without making contact with the screws (Figs. 15.19,
ters of rotation of angulation (CORA) (Fig. 15.12); thus 15.20, and 15.21). The level of osteotomy is chosen based
several osteotomies may be required to fully correct the mul- on preoperative planning and performed percutaneously
tiapical deformities. Many methods of treatment have been using the multiple drill hole technique for the femur or a
described. The most common involve acute or gradual correc- Gigli saw for the tibia. Following the osteotomies, the
tion, using either circular or monolateral external fixators, deformity is corrected using a monolateral external fixator
which can secure accurate correction of the deformity and (Fig. 15.22). The correction is confirmed by obtaining fron-
address the limb-length discrepancy. However, these methods tal and side view long radiographs. If the desired correction
are uncomfortable for the patient, especially when both legs is not achieved, it is adjusted and confirmed with new
are involved. There are further disadvantages, such as the X-rays. Once the surgeon is satisfied with the correction, the
need for daily adjustments, weekly follow-ups, a high rate of intramedullary nailing is performed through a 2 cm trans-
pin-track infection, and a long duration of external fixation. verse incision over the ligamentum patellae (Fig. 15.23).
Table 15.2 Review of literature
Authors Journal (year) Title Number of patients Results
Fucentese J Child Metabolic and orthopedic 12 Single bilateral surgical correction was performed in six
et al. Orthop management of X-linked patients; one patient each had three and five corrections.
(2008) vitamin D-resistant Bone lengthening was performed in three patients. At last
hypophosphatemic rickets follow-up, the height of seven operated patients was within
normal range. In addition, leg axis was normalized in six
patients with mild genua vara in two. Bone healing was
excellent: surgical complications were rare. In case of bone
deformity, surgery can safely be performed, independent of
age or bone maturation
Kocaoglu J Bone Joint Combined technique for 17 (43 segments) 43 segments in 17 patients with metabolic bone disease
et al. Surg Br the correction of underwent surgical treatment by the fixator assisted nailing
(2011) lower-limb deformities technique. The deformity correction was achieved with a
resulting from metabolic low complication rate. The use of intramedullary nail
bone disease prevented recurrence of deformity and refracture
Kanel et al. J Pediatr Unilateral external (29 segments) Corrective osteotomies were performed on 29 bones in nine
Orthop fixation for corrective children with hypophosphatemic rickets. Use of the Orthofix
(1995) osteotomies in patients external fixator enabled precise correction of the deformities
with hypophosphatemic without interruption of medical management
rickets
Rubinovitch Clin Orthop Principles and results of 10 (44 Osteotomies were combined with shortening and
et al. Relat Res corrective lower limb osteotomies) compression plating. Recurrence of deformity occurred in
(1988) osteotomies for patients 27 % of the cases. While osteotomies were safe and
with vitamin D-resistant provided dramatic improvement to limb deformity,
hypophosphatemic rickets postoperative control of vitamin D metabolism was the one
constant factor for maintenance of correction
Petje et al. Clin Orthop Deformity correction in 10 (53 segments) 37 corrective operations were performed on ten children.
Relat Res children with hereditary Depending on the patient’s age, external fixation was used
(2008) hypophosphatemic rickets in 53 segments: Kirschner wires in 18, DynaFix in three, the
Taylor patial Frame device in 13, and the Ilizarov device in
19. Internal fixation with intramedullary nailing was
performed in 12. Deviation of the mechanical axis and knee
orientation lines was increased at the follow-ups conducted
during a period of 5–12 months. Additional follow-ups
revealed a recurrence rate of 90 % after the first corrective
procedure and 60 % after a second procedure
Song et al. Acta Deformity correction by 20 (55 segments) 55 segmental deformities (20 femora, 35 tibiae) from 20
Orthopedica external fixation and/or patients were examined retrospectively. Distraction
(2006) intramedullary nailing in osteogenesis was used in 28 segments and acute deformity
hypophosphatemic rickets correction in 27. External fixation was applied in 24
segments, intramedullary nailing in six, and external fixation
and intramedullary nailing in 25. Recurrent deformity or
refracture occurred in 10 of 21 segments with distraction
osteogenesis by external fixation only, 4 of 6 with acute
correction by intramedullary nailing, and 1 of 25 with
distraction osteogenesis or acute correction by external
fixation and intramedullary nailing. External fixation and
intramedullary nailing can be recommended to prevent
complications during or after deformity correction in
hypophosphatemic rickets
Eralp et al. J Bone Joint A correction of 2 (7 segments) Seven segments in two patients with vitamin D related
Br (2004) windswept deformity by metabolic bone disease were treated by fixator-assisted
fixator assisted nailing nailing technique. All deformities were accurately corrected
and there was no consolidation problem at the osteotomy
sites. The total treatment time was found less than with
other techniques
Stanitski Clin Orthop Treatment of deformity 8 (18 segments) Complications were limited to several pin-tract infections
Relat Res secondary to metabolic and mild translational deformity in two patients. Healing
(1994) bone disease with the index averaged approximately twice that seen in pediatric
Ilizarov technique femoral lengthening and was 25 % greater than for patients
undergoing tibial lengthening. The lack of implants
requiring removal, modularity, and reasonable treatment
time make this technique an attractive alternative to
conventional osteotomy for management of limb-length
deformity associated with metabolic bone disease
Fig. 15.12 Long sagittal bowing deformity at the femur: note there are
Fig. 15.10 Note the recurrence of the deformities in a patient despite
multiple centers of rotation in these type of deformities
the intramedullary nails
Fig. 15.11 Following deformity correction (fixator assisted nailing)

lengthening over nail was performed at the femur Fig. 15.13 X-ray showing a varus bowing deformity at the femur
Fig. 15.16 Gigli saw osteotomy technique
Fig. 15.14 The deformity has been acutely corrected with use of an
unilateral external fixator
Fig. 15.17 Drill hole technique which is completed by an osteotome
The standard ligament split approach is used to open the

tibial or femoral canals under fluoroscopic control. Over a
guide wire, the medullary canal is over-reamed 1 mm larger
than the diameter of the nail to be used (Fig. 15.24).
Interference blocking screws are placed before or after nail
insertion to reduce the larger diameter of the medullary
canal at the metaphyseal level to prevent motion of the nail
(Fig. 15.25). The nail is inserted and locked both proximally
and distally if no lengthening is planned. The positions of
the nail and the Schanz screws and the deformity correction
are once again confirmed under fluoroscopic control, and
then the fixator is removed (Figs. 15.26 and 15.27)
(Box 15.3).
Follow-Up
Isometric exercises are begun postoperatively and partial
weight-bearing is allowed with crutches as tolerated. Full
weight-bearing is allowed only after achieving the consoli-
dation of three of four cortices on AP and lateral radiography
Fig. 15.15 Once the satisfactory correction was achieved, intramedul-
lary static fixation was performed (FAN procedure) (approximately 3 months).
Fig. 15.18 Long X-rays for

deformity analysis provided by a
radiolucent table
Fig. 15.19 Two parallel Schanz screws distally at the femur Fig. 15.20 Two parallel Schanz screws proximally at the femur
Fig. 15.21 Schanz screws must leave enough space for intramedullary
nailing
Fig. 15.23 Transverse skin incision over the ligamentum patella
Fig. 15.24 The medulla is overreamed by 1 mm
toma tend to produce rachitic changes in bone. The condition

generally resolves with excision of the tumor [20].
Fig. 15.22 Following osteotomy the correction is performed and
maintained by a monolateral external fixator
Renal Osteodystrophy
Tumor-Related Hypophosphatemic Rickets
(Oncogenic Hypophosphatemic General
Osteomalacia)
As the use of renal transplantation for treating renal failure in
Certain tumors have been found to secrete phosphatonins children has increased, the prevalence of renal osteodystro-
that result in phosphaturia. Neurofibromatosis, fibrous dys- phy has also climbed. Manifestations of renal osteodystrophy
plasia, less commonly osteoblastoma and hemangiopericy- are present in 66–79 % of children with renal failure [21].
Fig. 15.25 Interference screws are used to help maintain the

correction Fig. 15.27 Lateral view following intramedullary static fixation
Box 15.3
• The deformities are multiapical (long bowing) and
tend to recur.
• Intramedullary nailing should be performed always
if possible to prevent recurrence.
• Preoperative planning is of paramount impor-
tance, and should contain paper-tracing to simu-
late the surgical procedure. This will increase the
precision of the procedure while shortening the
surgical time.
• Fixator-assisted nailing is very effective for accu-
rate deformity correction and for prevention of
recurrences.
Renal osteodystrophy is noticeably different from either

nutritional or hypophosphatemic rickets. It is often driven by
the presence of secondary hyperparathyroidism, which leads
to activation of osteoclasts and resorption of bone [2, 4].
Features of both rickets and hyperparathyroidism are
present in children with renal osteodystrophy. Rachitic
Fig. 15.26 AP view following intramedullary static fixation changes include lack of calcification in the zone of provisional
calcification of the physis while secondary hyperparathy- Angular Deformity

roidism induces osteoclastic resorption of bone. Angular deformity occurs in renal osteodystrophy because
Serum vitamin D and calcium levels are usually low, the bone is soft, undermineralized, and prone to bend with
accompanied by increased levels of blood urea nitrogen and weight bearing. Genu valgum is the most common defor-
creatinine in the blood along with acidosis (see Table 15.1). mity, but genu varum or a windswept deformity may also
Children affected by renal osteodystrophy are often short- occur [4, 24]. If the beginning of renal osteodystrophy occurs
statured and have fragile bones. These patients often have before 4 years of age, varus deformity may develop, because
bone pain and fractures occur easily. The most common the normal alignment of the leg is in slight varus, which then
orthopedic manifestations are skeletal deformities, usually is accentuated when the bone becomes weak. Similarly,
genu valgum, periarticular enlargement of long bones, older children are prone to the development of genu valgum
slipped capital femoral epiphysis (SCFE), muscle weakness, because of the physiologic valgus alignment of the lower
and Trendelenburg gait especially if SCFE is present [4]. On extremity. Valgus at the ankle may also accompany the genu
radiographs, widespread osteopenia with thin cortices and valgum [25].
unclear trabeculae are present. The underlying bone has a Some milder deformities will correct with medical treat-
ground glass appearance. The physes are increased in thick- ment of the renal osteodystrophy [4, 25]. Deformities do not
ness, with an indistinct zone of calcification. In severe and respond well to bracing. If the patient becomes symptom-
persistent renal failure, aggressive lytic areas in long bones atic, and has had optimum medical treatment without reso-
may develop (brown tumor). Since many patients with renal lution of the deformity, corrective osteotomy should be
failure, especially those who undergo renal transplant, are accomplished [4, 17, 25]. Usually, the greatest deformity is
treated with steroids, osteonecrosis can also develop. in the distal femoral metaphysis, but sometimes, a supple-
mentary proximal tibial osteotomy is also needed. Internal
or external fixation may be used. While external fixators
Treatment have been successfully applied, taking care of achieving
stable constructs and utilize of hydroxyapatite coated
Medical Treatment Schanz pins, but bone healing may be delayed [26].
Treatment of the causal renal disease is of crucial impor- Recurrence of the skeletal deformity is common in patients
tance. Dialysis and renal transplantation are prolonging the with continuing metabolic pathology, so medical treatment
survival of these patients. Medical therapy is initiated with should be adjusted before and continued after corrective
the use of 1,25 dihydroxy form of vitamin D. The use of cal- osteotomy. Elevation of serum alkaline phosphatase con-
citriol significantly decreases serum PTH levels and delays centration above 500 U/L is a worthy marker of ongoing
secondary bone changes [22]. The treatment of acidosis with metabolic bone disease [4, 25]. Milder deformities may
sodium bicarbonate also improves metabolic bone disease. respond to hemiepiphysiodesis [17].
Decreased skeletal growth and short stature is a signifi-
cant problem, probably due to disturbances in the growth Slipped Capital Femoral Epiphysis
hormone—insulin like growth factor axis. Recombinant The clinical picture of a child with SCFE secondary to renal
human growth hormone (rHGH) restores growth in these osteodystrophy differs from the usual cases of SCFE. Often,
children. However, rHGH, also weakens the physes and may these patients are younger, and obesity is not a part of the
predispose the child to develop SCFE [23]. In patients with clinical picture. Bilaterality is also very common. Radiologic
renal osteodystrophy, who are refractory renal osteodystrophy pathology in the physis is more pronounced, accompanied
to medical treatment, parathyroidectomy may be indicated. by physeal widening and generalized osteopenia [27]. The
goal of standard management of SCFE is to prevent further
Orthopedic Treatment deformity and promote early closure of the proximal femoral
Patients with renal osteodystrophy are generally referred to physeal growth plate. However, cessation of proximal femo-
the orthopedic surgeon for the treatment of three pathologies: ral growth may not be desirable in a young child with renal
angular deformity of lower extremity long bones, SCFE, and osteodystrophy. Moreover, physeal healing is compromised
avascular necrosis [4]. Any surgical intervention in this due to the underlying metabolic pathologic condition.
patient population should be carefully considered, as the peri- Fortunately, pain and physeal widening resolves with initia-
operative risks are amplified due to associated anemia, hyper- tion of appropriate medical treatment. If the slip is displaced
tension, bleeding tendencies, and electrolyte imbalances. The or symptoms continue despite of medical treatment, fixation
risk of infection is additionally increased in patients with a with one screw, accomplishing stability cross the physis
renal transplant who are under immunosuppressive therapy. without closing it, should be performed.
Avascular Necrosis bowing and provide structural support to the long bones [4].
Prolonged use of corticosteroids is the likely cause. Avascular Thus, when possible it is advised to utilize an intramedullary
necrosis occurs frequently bilateral, affecting the hip. nail in all corrected bone segments.
Treatment is usually symptomatic [4]. Medical control of the underlying disease is of paramount
importance as the deformities tend to recur in various meta-
bolic bone diseases. Therefore, consultation with the endo-
Hypophosphatasia crinologist should be done preoperatively and continued
after the surgical intervention.
General
Example Cases
Hypophosphatasia is a rare, genetic defect of alkaline Case 1: Figures 15.28, 15.29, 15.30, 15.31, 15.32, 15.33, and
phosphatase production, resulting in pathologic mineral- 15.34 show the treatment sequences of a patient (hypophos-
ization of bone. There is a wide variation in the severity of phatemic rickets) with a profound bilateral femoral varus
the disease and the prognosis depends on the age of onset; deformities.
perinatal, infantile, childhood and adult hypophosphatasia Case 2: Figures 15.35, 15.36, 15.37, 15.38, 15.39, 15.40,
can occur [4, 28]. The genetic defect for this disorder is 15.41, 15.42, and 15.43 show the treatment sequences of a
determined to be in the tissue-nonspecific alkaline phos- patient (hypophosphatemic rickets) with a profound genu
phatase gene (TNSALP), and many different mutations valgum deformity.
have been described [29]. Case 3: Figures 15.44, 15.45, 15.46, 15.47, 15.48, 15.49,
The pathology detected in hypophosphatasia closely 15.50, 15.51, and 15.52 show the treatment sequences of a
resembles that seen in patients with rickets. Osteoid produc- patient (hypophosphatemic rickets) with a profound genu
tion remains undisturbed, but deprived of effective ALP, varum deformity.
mineralization of osteoid is compromised [4, 28, 29]. As a Case 4: Figures 15.53, 15.54, 15.55, and 15.56 show the
results, the physes are widened with persistence of the zone treatment sequences of a patient (X-linked rickets) by guided
of calcification and metaphyseal remnants of cartilage growth technique.
islands. If hypercalcemia is added to the clinical picture, het-
erotopic calcification foci can be formed. In laboratory tests,
ALP levels are decreased not only in the serum but also in
tissues like kidney, bone, etc. [1, 4]. Serum phosphorus, vita-
min D, and PTH levels may remain within normal limits, but
hypercalcemia is sometimes present. Characteristically,
urine pyrophosphate levels are increased [1, 4].
In the severe perinatal form, the infants may be stillborn.
If they survive delivery, they frequently die from respiratory
infection in early infancy. All bones display severe deminer-
alization on radiographs. The infantile form starts later, usu-
ally around 6 months of age. These babies have demineralized
bones with severe rachitic changes. Fractures and bowing of
the extremities are frequent. If these children can survive
infancy, they tend to improve clinically, but they are short
statured throughout childhood due to absence of normal
endochondral bone growth [1, 4].
Treatment
Although there is no conventional medical treatment of

hypophosphatasia, successful bone marrow cell trans-
plantation with improvement of the disease has been
described [30].
Fractures and deformities need orthopedic management.
Fracture healing is commonly delayed. Multiple osteotomies Fig. 15.28 A patients orthoroentgenogram showing severe varus
with intramedullary fixation are often required to correct the deformities
Fig. 15.29 Right lateral orthoroentgenogram shows anterior femoral Fig. 15.31 Paper-tracing for deformity analysis
bowing deformity. Note there is pending stress fracture at the femoral
dyaphyseal level
Fig. 15.30 Left lateral orthoroentgenogram shows anterior femoral

bowing deformity Fig. 15.32 Paper-tracing for surgical simulation
Fig. 15.33 Orthoroentgenogram following FAN procedure at the Fig. 15.35 Orthoroentgenogram of a patient with severe genu valgum
femur and tibia on the right deformity
Fig. 15.34 Orthoroentgenogram following final FAN procedure at the

femur and tibia on the left Fig. 15.36 Right lateral orthoroentgenogram of the same patient
Fig. 15.37 Left lateral orthoroentgenogram of the same patient Fig. 15.39 X-ray of the right femur following FAN procedure
Fig. 15.38 Paper-tracing for surgical planning Fig. 15.40 X-ray of the left femur following FAN procedure
Fig. 15.41 Orthoroentgenogram at the end of the treatment Fig. 15.43 Photographic documentation of the same patient after the
treatment
Fig. 15.42 Photographic documentation of the same patient prior to Fig. 15.44 Right frontal orthoroentgenogram showing severe genu
surgery varum
Fig. 15.45 Left frontal orthoroentgenogram of the same patient show- Fig. 15.47 Left lateral orthoroentgenogram showing anterior bowing
ing severe genu varum (same patient)
Fig. 15.46 Right lateral orthoroentgenogram showing anterior bowing

(same patient) Fig. 15.48 Paper-tracing for deformity analysis on the right
Fig. 15.51 Photographic documentation of the same patient prior to

surgery
Fig. 15.49 Paper-tracing for surgical simulation
Fig. 15.52 Photographic documentation of the same patient after the

Fig. 15.50 Orthoroentgenogram at the end of the treatment treatment
Fig. 15.53 Orthoroentgenogram of a patient with X-linked rickets Fig. 15.55 Orthoroentgenogram of the same patient after 3 months
prior to treatment later
Fig. 15.54 Orthoroentgenogram of the same patient after application

of hemiepiphyseodesis
2. Mankin HJ. Rickets, osteomalacia and renal osteodystrophy:

an update. Orthop Clin North Am. 1990;21:81–96.
3. Zaleske DJ, Doppelt SH, Mankin HJ. Metaboic and endocrine
abnormalities of the immature skeleton. In: Morrissey RT, editor.
Lovell and winter’s pediatric orthopaedics. 3rd ed. Philadelphia,
PA: JB Lippincott; 1990. p. 203–61.
4. Herring JA. Metabolic and endocrine bone diseases. In: Herring
JA, editor. Tachdjian’s pediatric orthopaedics. 4th ed. Philadelphia,
PA: Elsevier; 2008. p. 1917–82.
5. Pitt MJ. Rachitic and osteomalacic syndromes. Radiol Clin North
Am. 1981;19:581.
6. Mankin HJ. Metabolic bone disease. Instr Course Lect. 1995;
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anticonvulsant therapy in a pediatric outpatient population.
Pediatrics. 1975;56:52–7.
8. Steendijk R, Hauspie RC. The pattern of growth and growth retar-
dation of patients with hypophosphataemic vitamin D-resistant
rickets: a longitudinal study. Eur J Pediatr. 1992;151:422–7.
9. Saggase G, Baroncelli GI, Bertelloni S, Perri G. Long-term growth
hormone treatment in children with renal hypophosphatemic rick-
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J Pediatr. 1995;127(3):395–402.
10. Peterson BR. Augmenting vitamin D to combat genetic disease.
Chem Biol. 2002;9(12):1265–6.
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orthopedic management of X-linked vitamin-D resistant hypophos-
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et al. Deformity correction by external fixation and/or intramedul-
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LT, editor. Pediatric orthopedic secrets. 3rd ed. Philadelphia, PA: 23. Greenbaum LA, Del Rio M, Bamgbola F, Kaskel F. Rationale for
Elsevier; 2007. p. 551–9. growth hormone therapy in children with chronic kidney disease.
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windswept deformity by fixator assisted nailing. A report of two lism, and hypophosphatasia. Curr Opin Rheumatol. 1992;4:
cases. JBJS Br. 2004;86(7):1065–8. 389–93.
25. Davids JR, Fisher R, Lum G, Von Glinski S. Angular deformity of 29. Cai G, Michigami T, Yamamoto T, Yasui N, Satomura K, Yamagata
the lower extremity in children with renal osteodystrophy. J Pediatr M, et al. Analysis of localization of mutated tissue-nonspecific
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Osteogenesis Imperfecta
16
François R. Fassier
Consequence: Measuring the size of the implant on the

Introduction lateral view of the deformed bone may lead to an over opti-
mistic size for a rod and intraoperative problems (fracture).
Until recently, the surgical treatment of osteogenesis imper- One fundamental aspect of OI bone surgery is that the
fecta (OI) was limited to open osteotomies and “Shish child needs more bone than metal: Reaming to enlarge the
Kebab” rodding [1] with multiple complications and high medullary canal should be limited as it removes bone, and a
reoperations rate. Since 1992, the use of bisphosphonates large implant takes most of the stress in the bone which dis-
has improved the prognosis of these children. Additionally, a appears around the rod. This is a very challenging situation.
team approach (medical treatment, surgery, and postopera- Prevention is the best way to deal with this problem.
tive rehabilitation) has led to better functional outcomes [2] The bone deformity is (like scoliosis) a 3D problem. The
even though the medical treatment is not a cure and the bone, 2D pre-op imaging (AP and LAT of the affected bone) gives
though stronger, remains brittle. Therefore, the surgeon and a limited understanding of the complexity of the deformity.
the family must be aware of the necessity for multiple surgi- Intraoperatively, prior to making an incision, it is useful to
cal interventions during growth. analyze the bone deformity with fluoroscopy: it allows
to define precisely the apex (or apices) of the deformity
(or deformities) and choose wisely where to perform the
The Problems osteotomy (or osteotomies).
The preoperative assessment of lower extremities deformi-

ties in OI children is crucial. Soft Tissue Problems
Soft tissues play an important role in the development of

Bone Deformity(ies) bone curvatures in OI. During growth, the soft tissue resists
the elongation of the bone. The tether effect leads to a pattern
The structure of the bone is affected: the deformed bone gets of deformities defined by the most important muscle groups.
thinner in the frontal plane (AP view) and wider in its sagittal In the femur, the posteromedial hamstrings are the most
plane (lateral view) (Fig. 16.1). As a consequence, the med- resistant muscles, and therefore the femur bows anterolater-
ullary canal is deceptively narrow and the evaluation for the ally. In the tibia, most muscles are posterior and lateral,
size of an implant is challenging. The cross section of the hence the anteromedial bow of the tibia. This “rule” can be
bones (femur and tibia) is more “rib-shaped” and the triangu- modified according to the antenatal position of the fetus: for
lar aspect of the normal bone has disappeared. instance, a child with OI born in a breech position may be
born with a recurvatum deformity of the femur due to the
extended knee with a flexed hip.
These soft tissues problems can, and will cause difficul-
ties at the time of surgical correction of the deformities.
F.R. Fassier, MD (*) Generally speaking beyond 60° of angulation, it is advisable
Department of orthopedics, Shriners Hospital
for Children – Canada, 1529 Cedar Avenue,
to shorten the bone (closing wedge resection) to be able to
Montreal, QC, Canada H3G1A6 straighten the bone without too much tension in the
e-mail: [email protected] muscles.

256 F.R. Fassier
The patient/family must be aware of the risks of compli-

cations that can be seen with any type of surgery but also
specific complications related to the OI:
• Anesthesia: There is a risk of malignant hyperthermia
[4, 5] during surgery, even though a recent paper [6] com-
paring patients with OI and those without OI did not find
a difference between the two groups.
• Difficult intubations (big head, big tongue).
• Ventilation problems (deformed thorax).
• Fracture: The risk is always present during the transfer of
patient, while finding an IV access, positioning on the OR
table, and during the surgical manipulations.
• Neurologic complications (patients with Type III OI may
have hydrocephalus).
• Hemorrhage.
Surgical Correction
Why?
A deformed bone (particularly when weak due to OI) loses

its biomechanical properties and is likely to break at the apex
Fig. 16.1 The medullary canal on the AP view (a) is smaller than on of the deformity. Usually the deformity gets worse after a
lateral view (b) fracture, leading to a vicious circle of fracture/deformity/
fracture.
Two important points to check prior to surgery: (1) cor-

rection of coxa vara: the amount of free adduction preopera- When?
tively gives the amount of possible skeletal correction
(adduction test); (2) if the foot is in equinus prior to correct- Rodding before walking age is technically more difficult,
ing procurvatum tibial bow, this equinus will be worse at the implant size is reduced and postoperative rehabilitation can
end of the correction. In such a case, a shortening of the tibia be challenging. Since the introduction of bisphosphonates,
can be done at the time of the osteotomy. A tendo Achilles stronger bones can be obtained in a few months, and there-
lengthening can also be done at the end of the procedure. fore there is no need to operate on children before walking
age.
Before Surgery
How?
At our center, most patients with severe OI receive bisphos-
phonates soon after birth. Intravenous Pamidronate is the “Guided growth” with hemi-epiphysiodesis using non-
most widely used treatment in North America. Zoledronate locking plates or staples is quite popular for managing angu-
is progressively replacing Pamidronate as it is more potent lar deformities of the lower extremity in children. This
and is administered only twice a year. The acute complica- technique is very effective when the angulation originates
tions of the bisphosphonates include a flu-like reaction in close from the growth plate; and the growth plate has a nor-
most children at first cycle treatment as well as risk of hypo- mal growth potential. It is unlikely to work on a diaphyseal
calcaemia (significant respiratory distress has been reported angulation like OI, and with a limited function of the growth
in children with preexisting respiratory compromise [3]). plate. Therefore, the indications of hemi-epiphysiodesis in
Fracture healing does not seem to be affected by the admin- OI are relatively rare. A recent paper from Yilmaz and col-
istration of bisphosphonates while osteotomy healing (slow laborators [7] demonstrates the safety and effectiveness of
remodeling process) is often delayed. Therefore, no bisphos- “guided growth” in patients with skeletal dysplasias. No case
phonates are given postoperatively (see Box 16.3). of OI is reported in this series.
16 Osteogenesis Imperfecta 257
Fig. 16.2 Type I OI, resorption of the bone at the level of the plate,
particularly the medial cortex
The use of IM rods was proposed by Sofield and Millar in

1959 [1]. The rod acts as a tutor in the bone, preventing pro-
gressive deformity during growth. It reduces the fracture
rate, and if a fracture happens, it eases the treatment, prevent-
ing the displacement of the bone fragments. Fig. 16.3 Type III OI, deformity of the femur under the tip of the nail
Application of plates is generally not indicated in patients with progressive migration through the cortex
with OI, since the plate creates a rigid segment and the bone
is likely to break above or below the implant [8]. Furthermore, from a distinct epiphysis, these implants move away of each
under the plate, the bone is not submitted to any stress and other during growth. Unfortunately, they do not provide the
resorbs (Fig. 16.2). same stability as telescopic nails, and it is common to see the
bone deform during growth (Fig. 16.4). In their report [14]
Different Types of Rods Can Be Used in OI Bones Laville and Al concluded that sliding rods were not superior
Regular rods (such as K-wires, Rush rods, elastic nails) have to telescopic rods.
the advantage of being readily available, relatively inexpen-
sive and come in varying diameters from 1 mm up (K-wires). Open or Percutaneous Osteotomy?
The Telescopic rods introduced by Dubow-Bailey [9] have The growing trend for minimally invasive surgery in orthope-
been modified by Sharrard [10] Karbowski [11] and more dics is beneficial, as it results in less scars and decreased soft
recently by Cho [12]. A new concept of telescopic rod, with- tissue trauma (including less blood loss and less postopera-
out the need for arthrotomies has been described by the tive pain); but is technically more demanding. For the femur:
author [13] with short term promising results. These implants before the era of bisphosphonates, OI bone was extremely
are more expensive, need to be ordered (and currently not brittle, but it was easier to do a percutaneous osteotomy, or
available in all countries), and the smallest diameter is even an osteoclasis. Since most patients receive bisphospho-
3.2 mm, which is sometimes too large for small children. nate before surgery, the surgeon must be aware that the bone
The major advantage of telescopic (or growing rods) is that which is harder will break suddenly and not necessarily at the
the implant gets longer as the child grows, reducing the need desired place! The amount of preoperative deformity as well
for reoperation (which is needed every second or third year as the absence of a medullary canal on X-rays should lead the
with non-telescopic rods because the bone deforms under the surgeon to prefer the “open” technique in selected cases.
tip of the nail during growth) (Fig. 16.3). If a telescopic rod For the tibia: because of the soft tissue problem (relative
is not available, it is also possible to use “sliding rods”: two equinus deformity) it is better in most cases to do an open,
regular rods (Rush or elastic nails) are inserted: one from closing wedge osteotomy. It allows appropriate shortening of
proximal epiphysis-distally, and the other rod from the distal the tibia so that the foot will be plantigrade following surgi-
epiphysis-proximally. As each of the two rods are inserted cal correction.
258 F.R. Fassier
minutes, allowing a smooth manipulation of the bone frag-

ment. If the bone breaks, it may just be a split of one of the
fragments: cerclage technique (with resorbable suture) is
often sufficient. If the bone bursts in multiple fragments, it
may be best to postpone rodding and leave the patient in skin
traction for a few weeks.
Do not accept poor alignment as it will likely increase
overtime. In the femur, if the nail is too anterior distally, it
will perforate the cortex (see Fig. 16.3a). It is easy to do a
percutaneous distal metaphyseal osteotomy to be able to cen-
ter the rod in the middle third of the epiphysis (for telescopic
rods) on both frontal and sagittal planes.
In the tibia, the deformity is most exclusively in procurva-
tum, particularly in the distal tibia. It is advisable to overcor-
rect to 5–10° of recurvatum to slow down the recurrence of
the deformity during growth.
Technique for Femoral Rodding (see Box 16.1)

With an open osteotomy through a posterolateral approach, it
is simple to ream the medullary canal to the appropriate size
of nail. (Remember that the child needs more bone than
metal; avoid excessive reaming!)
If the implant is to be a Rush nail; its introduction
through the tip of the greater trochanter (GT) is facilitated
with the retrograde insertion of a K-wire that is used as a
Fig. 16.4 Sliding rods. (a, b) After 4 years, progressive varus defor-
mity aggravated by a proximal femoral fracture (b) guide.
Note: The author has modified his own K-wires so that the
tip on the blunt end is “cup-shaped” allowing a better contact
Note: Author’s preferred method: Whenever possible, I use between the nail and the K-wire guide (Fig. 16.5).
percutaneous surgery for the femur, but if after 15–20 min, I The length of the nail should be calculated from the tip of
am not able to reduce the osteotomy and realign the bone, I the GT (or the ossified proximal metaphysis) to the inter-
switch to the open technique. condylar notch. Pushing the nail through the growth plate
The relative contraindications for percutaneous femoral (often the strongest part of OI bone) helps stabilizing the
osteotomies are as follows:
• Angulation > 60°.
• No medullary canal seen on X-rays. Box 16.1. Summary of Femoral Fassier-Duval Rod
Technique
Osteotomy 1. Male rod introduced with male driver.
The use of an oscillating saw for performing the osteotomy 2. Male rod positioned in the middle third of the distal
in patients with OI is not recommended: It burns the bone epiphysis on both AP and LAT intraoperative fluo-
ends, and as these patients receive bisphosphonates (which roscopic views.
reduces the number of osteoclasts), the remodeling is slow. It 3. Remove the male driver, keeping pressure on the nail.
takes time to remove the dead bone and this may lead to 4. Measurement of the female rod, which is cut intra-
delayed union or non-union. It is better to use osteotomes operatively (Midas Rex®)
(after preparing the bone with a few drill holes) and clean the 5. Female rod introduced: verify that the rod is not too
bone ends (in the open technique) with a rongeur. long, as when screwing it in the GT you may push
Reduction after osteotomy: the soft tissue tightness is here the male distally, into the knee joint.
again a challenge. If there is too much tension at the osteot- 6. The female rod threads are left proximal to the ossi-
omy site, do not fight with OI bone! Just shorten as much as fied metaphysis.
necessary to get a good alignment without too much tension. 7. Cut the male rod above the female and check
smoothness of the rod (as it has to slide down into
Note: At the time of reduction, I usually ask the anesthetist the hollow female nail).
to give adequate muscle relaxation to the patient for a few
nail which is often “floating” in an empty metaphysis. the femur will drive the nail to be too anterior. In such a case,
As long as just one attempt of placing the nail through the the nail is withdrawn a few centimeters, a percutaneous
growth plate is done, epiphysiodesis is unlikely to occur. metaphyseal osteotomy is done and the nail is pushed in the
For the use of the elastic nails, the original technique was center of the epiphysis (Fig. 16.6 and Box 16.4). The male
described [15] for normal bones and the entry point is in the driver is removed while maintaining pressure on the nail to
metaphysis. The technique has been adapted by Metaizeau avoid dislodging it. The female nail is measured, from the
for OI bones with a retrograde nail introduced through the ossified proximal femoral metaphysis to 1 cm above the dis-
epiphysis distally, and an antegrade nail inserted through tal femoral growth plate. The threads of the female nail
the greater trochanter. Both nails “slide” away of each other being left above the ossified metaphysis of the proximal
during growth [16]. For sliding rods, care must be taken for femur (see Fig. 16.6). (The measurement of the nail length
the distal rod to be anchored in the epiphysis without dis- does not take the threads of the female nail into consider-
turbing the knee motion. The tip of the rod must be bent and ation.) When the female nail has been cut (Midas Rex®) and
embedded. the lumen of the nail cleaned to allow a smooth telescoping;
The Fassier-Duval telescopic rod is inserted also from the the female nail is pushed down over the male nail. At the
GT and the introduction of the male driver is done first: A moment the proximal end of the nail disappears inside the
K-wire (same diameter as the male nail) is pushed up in the wound, a careful X-rays measurement is done to verify that
buttock from the osteotomy, and the male driver is pushed the distance between the distal end of the female nail and the
down on the K-wire as a guide. When the driver is at the “aisles” of the male nail (“A” on Fig. 16.7) is greater than
level of the osteotomy, the K-wire is removed and replaced the distance between the proximal threads of the female nail
with the nail. After reduction of the osteotomy, the male nail and the proximal metaphysis (“B”). If “B” is greater than
is pushed down in the shaft until it reaches the growth plate, “A”, while screwing the female nail in the non-ossified GT,
and screwed in the epiphysis. C-arm control of the position one will push the male nail into the knee joint. The male nail
in the bone is important: the nail should be in the middle is cut above the female and the smoothness of the section
third of the epiphysis both on AP and lateral. It is relatively checked with a little probe to ensure that the male nail slides
simple to be centered on AP but in lateral, any mild bow of smoothly distally during growth.
The technique for percutaneous osteotomy(ies) and rod-
ding with the Fassier-Duval is very well described by Paul
Esposito [17].
Technique for Tibial Rodding (see Box 16.2)

Whatever the type of rod used, the principles of the tech-
nique are the same:
• The osteotomy is done open through a direct anterior
approach at the apex of the deformity.
• The nail is introduced through a medial parapatellar ten-
don incision.
• The entry point of the nail is behind the patellar tendon,
on the edge of the proximal tibial epiphysis (which is
Fig. 16.5 Modified K-wires to serve as guide to introduce a Rush nail extra articular). A pre-bent K-wire is useful to find the
Fig. 16.6 FD rodding. Before screwing the female nail in the non- greater than the distance between the proximal threads of the female
ossified greater trochanter, verify (via C-arm) that the distance between nail to the ossified metaphysis B. If B >A the male nail will be pushed
the distal end of the female nail and the aisles of the male nail A is distally
260 F.R. Fassier
Fig. 16.7 “Ideal” positioning of

the FD rod in the femur at the
distal (a, b) and proximal ends (c)
Box 16.2. Summary of Tibial Fassier-Duval Rod Box 16.4. Optimal Position of Fassier-Duval Rods
Technique Male Rod
1. Use a bent K-wire to find the medullary canal. • Middle third of the distal epiphysis both on frontal
2. Ream over the K-wire (beware of posterior tibial and sagittal planes.
cortex breach if entry point is too anterior. Female Rod
3. The male driver is pushed down and the male nail posi- • The threads should be left above the ossified proxi-
tioned in the middle third of the distal tibial epiphysis. mal metaphysis for the femur, and within the proxi-
4. The male driver is removed, keeping the male nail mal epiphysis for the tibia.
in place.
5. Cut the male nail.
6. Measure the female nail.
7. Slide the female nail over the male. when such a child needs to have both femurs and tibiae oste-
8. Check that the knee extension is not limited by the otomized and rodded, different strategies can be used. The
male nail. most popular is to rod one side at the time, femur first, then
the tibia can be done with the use of a sterile tourniquet. The
second leg is operated a week later and the postoperative
immobilization is 3 weeks (4 weeks for the first side). This
surgical approach has the advantage of reducing blood loss
Box 16.3. Surgery and Bisphosphonates
and operating time. The disadvantage is to have the child
• The medication can be given 48 h before surgery
undergo two separate sessions of general anesthesia. Other
(no bisphosphonates in the blood stream 48 h after
options depend on patient’s needs; if the tibiae are straight
infusion).
and do not need rods; it is common to do both femurs at the
• No infusion for 4 months postoperatively, or until
same time. It is likely though that the patient will need blood
clear evidence of bone healing is seen on X-rays.
transfusion (except for percutaneous surgeries). Such an
option implies that the surgical set-up and the draping are
changed between the two sides (see “positioning”).
medullary canal. If the tibia is deformed into valgus, it is
better to use a lateral parapatellar tendon approach. The Positioning of the Patient
direction of the rod will be more in varus, allowing a bet- Most children with OI are smaller than average, and the soft
ter correction of the deformity. tissue tightness can lead to intraoperative difficulties. A good
Figure 16.8 shows the “ideal” position of the Fassier- way to stay out of trouble is to have the patient semi-supine,
Duval rod in the tibia, proximally and distally (see Box 16.4). with a radiolucent cushion (such as a saline bag) under the
ipsilateral buttock and another support under the ipsilateral
shoulder (Fig. 16.9). The patient is brought to the edge of the
Surgical Tips table (beware of metallic supports that may interfere with
C-arm use). This maneuver has two major advantages: at the
Long-term immobilization is known to be detrimental to beginning of the surgery, the leg can hang off the edge of the
bone health, particularly for children with OI. Therefore, table, allowing good control of the proximal femur deformity
Fig. 16.8 “Ideal” positioning of

the FD rod in the tibia at the
proximal (a) and distal ends (b, c)
Fig. 16.9 Positioning of the

patient on the OR table. The
operated side is raised, as is the
ipsilateral shoulder. (a) This
position allows to do lateral shoot
through of the knee without
changing the C-arm position. (b)
One must be sure that objects
such as the patient’s upper
extremity, suction tube, cautery
cable will not interfere with
adequate intraoperative
fluoroscopic visualization of the
operative field
and rule out a “pseudo coxa vara” (see next section on Coxa
Box 16.5. Coxa Vara in OI
Vara). The second advantage is to be able by flexing, adduct-
ing, and internally rotating the hip to get a lateral view of the Preoperative examination includes:
femur without changing the C-arm position. Beware during • AP view of the hip in extension to eliminate a
positioning that the contralateral arm is not left parallel to the pseudo coxa vara.
trunk, as the hand will be in C-arm field during the surgery. • Adduction test to assess the amount of possible
Verify as well that towel clips, suction tube, cautery cable or correction.
plate do not prevent a good imaging of the knee during sur-
gery. Another major advantage of this position is at the time
of the introduction of the nail through the greater trochanter:
it is much easier to manipulate the long tools (male driver in anteriorly bowed proximal femur [18]. To do so, the patient
case of Fassier-Duval rodding or the nail itself in other types is positioned on the edge of the table (semi-lateral as for
of rodding) as they can be “outside” the OR table and often femoral rodding) (Fig. 16.10), and before surgery, a true AP
lean lower than the level of the table (which would be impos- view of the hip is obtained in letting the leg hang out of the
sible if the patient was in the middle of the OR table). table. In addition, the “adduction test” will give a precise
idea of how much correction can be obtained. The surgical
technique for coxa vara correction is illustrated in the
Coxa Vara Correction (see Box 16.5) Fig. 16.11 [19]. The advantages of such a technique are that
it is adaptable to varying bone size and the implants (K-wires
This deformity is present in about 60 % of Type III OI and cerclage wires) are available in most operating rooms.
patients and may progressively develop after IM rodding of Depending on the stability of the fixation in the bone, in
the femur [18]. True coxa vara must be differentiated, on most cases, there is no need for postoperative spica cast
X-rays, from “pseudo” coxa vara due to the projection of an (which both the patient and parents appreciate). For bilateral
262 F.R. Fassier
Fig. 16.10 Before correcting a

coxa vara, a true AP view of the
hip is obtained with the
ipsilateral hip extended off the
edge of the table. The anterior
bow of the femur is eliminated
and a true assessment of the
neck-shaft angle is appreciated
Fig. 16.11 The sequential steps

of the coxa vara correction: The
two K-wires can be used as a joy
stick (a–b) and the reaming of
the proximal fragment is done
through the lateral cortex (c).
The angle between the drill bit
and the K-wires determines the
final neck-shaft angle. The
removal of a medial wedge of
bone from the distal bone
fragment (d) allows better
stability of the fixation with
rodding and cerclage wire (e, f)
cases (done 1 week apart), an “A” frame can be used, Postoperative Rehabilitation
particularly in young patients (Fig. 16.12). No weight-bear-
ing for 6 weeks followed by a week of intensive physiother- Third and a crucial part of the team work after medical and
apy starting in the pool, allows the patient to recuperate surgical treatment is physical rehabilitation. It generally
quickly. The results of this technique [19] show that an aver- starts 3 weeks postoperatively with the use of braces. Pool
age 30° of correction of the neck shaft angle can be obtained therapy and a tilting table are used to progressively help the
(Fig. 16.13). upright position.
Fig. 16.13 (a) Preoperative and postoperative (b) radiographs of the

Fig. 16.12 Postoperative immobilization after bilateral femoral and femur of a patient with coxa vara associated with Type IV OI
tibial rodding. The tape between the legs avoids spontaneous external
rotation of the hips
Stretching of Tight Muscles The AFOs have an anterior shell to serve as a support as
the tibia may deform anteriorly. Patients who have tele-
Most patients with severe anterolateral bow of the femur scoping rods in the tibia, do not need long term use
have a fixed flexion contracture of the hip postoperatively of AFOs.
that needs to be stretched. Similarly, the Tendo Achilles • SMOs: Very useful to control excessive foot/ankle valgus,
needs to be stretched after most tibial rodding. When due to hyperlaxity.
patients are able to support the standing position, they start • Molding of braces can be done at the end of the surgery
using a pediatric walker and progress toward free ambula- while the patient is still under general anesthesia. It has
tion depending on the severity of the disease. It must several advantages: no pain; no fear for the child; limita-
be noticed that the knowledge on muscles in OI is very tions of range of motion easily detectable with less risk of
limited [20]. fracture; and finally the braces are ready to be fit on the
patient when the cast comes off after 3 weeks.
Use of Braces
Results
The ultimate goal of an OI team is to see their patients walk-
ing independently. Nevertheless, because of extreme The combination of medical treatment, surgical realignment-
hyperlaxity seen in few cases, a concern of fracture, particu- rodding and postoperative rehabilitation has proven to
larly at the beginning of the walking age, braces are useful. improve “ambulation, gross-motor function, self-care and
• HKAFOs are no longer used by the author. mobility beyond physiological expectations due to develop-
• KAFOs are used initially postoperatively for patients who mental growth” [2]. Such results have also been demonstrated
have never walked before surgery. As soon as the phy- in the long term in adults [21]. Generally speaking, sliding
siotherapist confirms that the quadriceps strength is rods or telescopic rods are superior to a single rod with
sufficient for independent ambulation, the thigh cuff of regards to the longevity of the rod [22]. However,
the KAFO is removed and the knee is unrestrained. complications are frequent and the reoperation rate is high
• AFOs: Used at the initiation of walking postoperatively, [23] as despite bisphosphonates, the bone remains brittle
to help the patient get a good balance. These AFOs must though harder. The children feel better and their level of
be flexible enough to allow the bones to share the load. activity raises, and with it, the risk of fracture increases.
264 F.R. Fassier
During growth, the rod can migrate through the cortex (see 2. Ruck J, Dahan-Oliel N, Montpetit K, Rauch F, Fassier F. Fassier-
Fig. 16.3), which is common with non-telescopic nails, and Duval femoral rodding in children with osteogenesis imperfecta
receiving bisphosphonates: functional outcomes at one year.
these patients need revision surgery. In other cases, particu- J Child Orthop. 2011;5(3):217–24.
larly with telescopic rods (if the female rod threads are 3. Munns CF, Rauch F, Mier RJ, Glorieux FH. Respiratory distress
screwed in the ossified metaphysis), the greater trochanter with pamidronate treatment in infants with severe osteogenesis
grows proximally and the tip of the FD rod becomes com- imperfecta. Bone. 2004;35(1):231–4.
4. Peluso A, Cerullo M. Malignant hyperthermia susceptibility in patients
pletely sunk into the bone. The indications for the reopera- with osteogenesis imperfecta. Paediatr Anaesth. 1995;5:398–9.
tion in such a case are not clear [24] as the patient may be 5. Posborg P, Astrup G, Bendixen D, Lund AM, Ording
completely asymptomatic. Retrieving the rod in the bone H. Osteogenesis imperfecta and malignant hyperthermia. Is there a
without an osteotomy is challenging despite rescue tools; and relationship? Anaesthesia. 1996;51:863–5.
6. Bojanic K, Kivela JE, Gurrier C, Deutsch E, Flick R, Sprung J,
a common solution is to wait until a significant complication et al. Perioperative course and intraoperative temperatures in
(such as fracture) occurs to change the rod. No telescoping patients with osteogenesis imperfecta. Eur J Anaesthesiol.
may occur after a fracture, but also spontaneously (progres- 2011;28(5):370–5.
sive bowing of the bone and the rod): in such cases, the rod 7. Yilmaz G, Oto M, Thabet AM, Roger KJ, Anticevic D, Thacker
behaves as a regular rod. The indication for revision should
skeletal dysplasia with hemiepiphysiodesis: a preliminary report. J
then be the same as for solid rods (angulation, fracture, Pediatr Orthop. 2014;34(3):336–45.
migration of the implant beyond the confines of the bone). 8. Enright WJ, Noonan KJ. Bone plating in patients with type III
Delayed union/non-union rate was as high as 20 % in OI osteogenesis imperfecta: results and complications. Iowa Orthop
J. 2006;26:37–40.
before the era of bisphosphonates [25]; but with these medi-
9. Bailey RW, Dubow HI. Studies of longitudinal bone growth result-
cations, the risk is much higher [26] and the treatment of ing in an extensible nail. Surg Forum. 1963;14:455–8.
such a complication remains challenging. In many patients, 10. Stokely I, Bell MJ, Sharrard WJ. The role of expanding intramedul-
the pseudo-arthrosis is mildly symptomatic (recurrent pain lary rods in osteogenesis imperfecta. J Bone Joint Surg Br.
after minimal trauma) while in others the pain is debilitating. 1989;71(3):422–7.
11. Karbowski A, Schwitalle M, Brenner R, Lehman H, Pontz B,
An innovative solution has been proposed by Dr. Cho [27]: Worsdorfer O. Experience with Bailey-Dubow rodding in children
The use of a temporary locking plate, particularly in the sub- with osteogenesis imperfecta. Eur J Pediatr Surg. 2000;10(2):
trochanteric area. However, long term results of this tech- 119–24.
nique are currently not available. 12. Cho TJ, Choi IH, Chung CY, Yoo WJ, Lee KS, Lee DY. Interlocking
telescopic rod for patients with osteogenesis imperfecta. J Bone
Another challenge for the surgeon is the long and thin Joint Surg Am. 2007;89(5):1028–35.
bone often seen in adolescents with Type III OI. In such 13. Fassier F, Glorieux FH. Osteogenesis imperfecta. Surg Tech Orthop
cases, a single Rush rod can be used, but a locking nail is a Traumatol. 2003;SS-0S0 D-30:8 p.
safer implant with no need for post-op immobilization. 14. Boutaud B, Laville JM. L’embrochage centro-médullaire coulissant
dans l’ostéogenèse imparfaite : Quatorze cas avec un recul moyen
Unfortunately, most locking nails are 8.5 mm in diameter de 8 ans [Elastic sliding centromedullary nailing in 14 children with
and greater, which may be too large for patients with OI. The osteogenesis imperfecta]. Rev Surg Orthop. 2004;90:304–11.
GAP nail developed by Drs. Galban and Para [28] seems 15. Ligier JN, Metaizeau JP, Prévot J. Closed flexible medullary nailing
promising in such cases (with diameters between 4.8 and in pediatric traumatology. Chir Pediatr. 1983;24(6):383–5.
16. Metaizeau JP. L’embrochage centromédullaire coulissant.
8 mm). Applications au traitement des formes graves d’ostéogenèse impar-
Surgical correction of the lower limb deformities in OI faite. Chir Pediatr. 1987;28:240–3.
has become more frequent since these patients benefit from 17. Esposito PW. Multiple percutaneous osteotomies and Fassier-
medical treatment, including the use of bisphosphonates. In Duval telescoping nailing of long bones in osteogenesis imperfecta.
In: Flynn JM, Wiesel SW, editors. Operative techniques in pediatric
the future, other drugs such as Denosumab (for OI Type VI), orthopaedics. Philadelphia: Lippincott Williams & Wilkins; 2011.
TGF beta-blockers and sclerostin antibody will likely further p. 259–69.
improve the bone quality in such patients, while awaiting 18. Arabi M, Rauch F, Hamdy RC, Fassier F. High prevalence of coxa
other advances such as gene therapy for this condition. The vara in patients with severe osteogenesis imperfecta. J Pediatr
Orthop. 2006;26(1):24–8.
challenge for the orthopedic surgeon is to continue to 19. Fassier F, Zeeshan S, Aarabi M, Odent T, Haque T, Hamdy
develop minimally invasive techniques and better implants RC. Results and complications of a surgical technique for correc-
until metallic implants will no longer be necessary for tion of coxa vara in children with osteopenic bones. J Pediatr
patients with OI. Orthop. 2008;28:799–805.
20. Puvanesarajah V, Sponseller PD. Muscle, tendon and ligament in
osteogenesis imperfecta. In: Shapiro JR, editor. Osteogenesis
imperfecta – a translational approach to brittle bone disease.
New York: Elsevier; 2014. p. 349–52.
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ullary rod fixation of deformities of the long bones in children: a age follow-up of nineteen years. J Bone Joint Surg Am.
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22. Benjamin J, Gleeson R, Chandra KB. The choice of intramedullary imperfecta patients receiving pamidronate. J Bone Miner Res.
devices for the femur and the tibia in osteogenesis imperfecta. 2004;19(11):1779–86.
J Pediatr Orthop B. 2005;14:311–9. 27. Cho TJ, Lee K, Oh CW, Moon HJ, Park MS, Yoo WJ, et al.
23. Birke O, Davies N, Latimer M, Little DG, Bellemore M. Experience Unictorical locking plating in addition to intramedullary rodding
with the Fassier-Duval telescopic rod: first 24 consecutive cases with a for long bone stabilization in osteogenesis imperfecta patients –
minimum of 1-year follow-up. J Pediatr Orthop. 2011;31(4):458–64. preliminary report. In: Anticevic D, editor. 11th international con-
24. Frick SL, Sponseller PD, Leet A. Pediatric limb reconstruction in ference on osteogenesis imperfecta final program and abstract
osteogenesis imperfecta. In: Shapiro JR, editor. Osteogenesis book. Dubrovnik: Croatian Pediatric Orthopaedic Society of
imperfecta – a translational approach to brittle bone disease. Croatian Medical Association; 2011. p. 101.
New York: Elsevier; 2014. p. 443–51. 28. Galban M. Intramedular rodding in adult with osteogenesis imper-
25. Gamble JG, Rinsky LA, Strudwick J, Bleck EE. Non-union of frac- fecta developing of a new device. In: Anticevic D, editor. 11th inter-
tures in children who have osteogenesis imperfecta. J Bone Joint national conference on osteogenesis imperfecta final program &
Surg Am. 1988;70:439–43. abstract book. Dubrovnik: Croatian Pediatric Orthopaedic Society
26. Munns CFJ, Rauch FT, Zeitlin L, Fassier F, Glorieux FH. Delayed of Croatian Medical Association; 2011. p. 81.
osteotomy but not fracture healing in pediatric osteogenesis
Lower Limb Deformity
in Neuromuscular Disorders: 17
Pathophysiology, Assessment, Goals,
and Principles of Management
Unni G. Narayanan
Children in level I walk independently without a walking aid

Cerebral Palsy and can perform most activities of typically developing chil-
dren, with some difficulties with speed, balance, and coordi-
Cerebral palsy (CP) refers to disorders of the development of nation. In level II, children are independently ambulant
movement and posture that arise from a disturbance to the without a walking aid, but require support in more challeng-
developing fetal or infant brain. These motor disorders are ing situations such as on uneven surfaces or while climbing
permanent but not progressive and can be accompanied by stairs. In GMFCS level III, children rely on walking aids
deficits of sensation, perception, cognition, and communica- such as crutches or walkers for at least some of their func-
tion; seizure disorders; and secondarily acquired musculo- tional mobility. Some may use wheelchairs for longer dis-
skeletal deformity [1, 2]. tances. Children in GMFCS levels IV and V rely entirely on
CP accounts for the most common cause of chronic child- a wheelchair for any functional mobility. In level IV, children
hood disability, with a prevalence of 2–3 per 1,000, and is can usually sit without support and do some functional
associated with a wide range of etiologies [3]. Prematurity is weight bearing for transfers. Walking, if possible, is limited
one common risk factor due to the fragility of the cerebral to short distances with a walker, for exercise purposes.
vasculature and immaturity of the autonomic nervous system Children classified as level V are unable to sit without sup-
that controls cerebral perfusion. Some additional risk factors port, and weight bearing, if at all, is limited to supported
include congenital brain malformations, intrauterine infec- weight bearing in a stander for exercise rather than func-
tions, placental malformations, prenatal brain hemorrhages, tional purposes. These children depend on a caregiver for
or strokes; perinatal causes of hypoxic-ischemic encepha- much of their activities of daily living.
lopathy and hyperbilirubinemia; and postnatal strokes, infec- CP is also classified by the type of abnormal tone or move-
tions, or traumatic and other kinds of brain injuries. A number ment disorder. The location and extent of brain involvement
of genetic disorders (Rett syndrome, fragile X syndrome, account for the type and severity of the abnormal tone and
familial or hereditary spastic paraparesis) and metabolic dis- movement disorder as well as the topographical distribution of
orders (mucopolysaccharidoses, phenylketonuria, etc.) can involvement of the trunk and limbs. Lesions involving the pyra-
mimic features of CP. midal tract are associated with spasticity or velocity-dependent
hypertonia, whereas lesions of the basal ganglia are associated
with dyskinesias including dystonia or choreoathetosis.
Classification of Cerebral Palsy Dystonia is characterized by jerky involuntary movements of
the limbs or trunk and neck. Choreoathetosis is characterized by
CP is best classified by the Gross Motor Function involuntary writhing movements. Cerebellar involvement is
Classification System (GMFCS), which has been shown to associated with ataxia and hypotonia. Some children have fea-
be reliable, valid, consistent, and highly prognostic [4, 5]. tures of more than one type or “mixed”-tone abnormalities. The
The GMFCS comprises five levels of severity (Fig. 17.1). commonly used topographical classification, hemiplegic, diple-
gic (lower extremities involved more than upper extremities),
and quadriplegic or total body involved, has some clinical and
U.G. Narayanan, MBBS, MSc, FRCS(C) (*) prognostic utility, but these terms are not very reliable. Instead,
Department of Surgery, The Hospital for Sick Children,
University of Toronto, 555 University Avenue, S-107, Toronto,
the regional involvement may be categorized as being “unilat-
ON, Canada M5G1X8 eral” or “bilateral,” which can be symmetric or asymmetric
e-mail: [email protected] with one side more involved than the other.

268 U.G. Narayanan
Fig. 17.1 GMFCS levels.

Reproduced with permission
from copyright © Kerr Graham,
Bill Reid, and Adrienne Harvey,
The Royal Children’s Hospital,
Melbourne
Pathophysiology of Musculoskeletal Deformity and coordination, and muscle weakness [6]. These children
in Cerebral Palsy often have a delay in the development of the motor
milestones.
Musculoskeletal deformities in CP are acquired secondarily. Hypertonia is associated with “dynamic” muscle contrac-
Characteristically, upper motor neuron disorders are associ- tures. Muscles are taut due to the increased muscle tone, but
ated with hypertonia, hyperreflexia, and co-contraction of they are of sufficient length. Muscles typically grow in
agonist–antagonist muscles. Other features can include loss response to the stimulus of stretch. In a child with CP, devel-
of selective motor control, poor centrally mediated balance opmental delay prevents the child from engaging in the typi-
17 Lower Limb Deformity in Neuromuscular Disorders: Pathophysiology, Assessment, Goals, and Principles of Management 269
Fig. 17.2 Pathway to secondary

musculoskeletal pathology in
cerebral palsy. Adapted from
Noordin S, Narayanan
U. Neuromuscular Disorders,
Chapter 64. In: Mercer’s
Textbook of Orthopedics and
Trauma, 10th Edition. Boca
Raton: CRC Press; 2012
cal physical activities that would provide the source of positioning, and mobility; and poorer quality of life.
stretch. The hypertonia further impedes the extent of any Although the primary lesion in the brain is not progressive,
stretch. Consequently, muscle growth is impaired relative to the secondary musculoskeletal consequences become worse
bone growth. This results in the development of “static” con- with growth [9–12] (Fig. 17.2).
tractures where the muscles are both tight and too short [7].
The acquisition of milestones, from rolling over into the
prone position, sitting upright, crawling, pulling up to stand, Specific Deformities of the Lower Extremity
cruising, and walking, typically occurs during the first 12–15
months of a child’s life. The growing skeleton undergoes sig- Pelvis and Hip
nificant changes during this time, in response to the natural Across the hip joint, adductors and flexor muscle groups are
stresses applied to the skeleton. The delay in motor mile- typically more involved, overpowering their antagonist
stones, or in some cases failure to achieve them at all, counterparts, the hip abductors and hip extensors. This mus-
deprives the skeleton of these important corrective forces, cle imbalance often results in adduction and flexion contrac-
resulting in the retention of infantile morphology of the skel- tures of the hip in the coronal and sagittal planes, respectively.
eton (e.g., increased femoral anteversion and coxa valga in The hip flexion contracture arising from the iliopsoas can be
the proximal femur). Furthermore, the abnormal muscular accompanied by anterior pelvic tilt with associated lumbar
forces acting on the growing skeleton contribute additionally lordosis. Anterior pelvic tilt might also be secondary to weak
to the development of bony deformities and joint instability hip extensors or weak anterior abdominals or a combination
over time. Joint instability in these patients is often a conse- of these [13, 14]. In contrast, contracted hamstrings can pull
quence of both the bony deformity and the unbalanced forces down on the ischium resulting in a relative posterior tilt of
of the agonist–antagonist muscles that bridge across a joint. the pelvis. In the transverse plane, the hip commonly lies
The skeletal deformities result in lever arm dysfunction [8]. internally rotated. This is more likely due to increased femo-
In the lower extremity, the interaction of joint contractures, ral anteversion rather than an internal rotation contracture as
muscle weakness, bony deformities, and joint instability at such [15]. In bilaterally involved children, the muscle con-
multiple levels is associated with inefficient (higher energy tractures and bony deformities may not be symmetric. The
consuming) pathologic and compensatory gait patterns in more severely involved side might be more adducted and
ambulant children. This leads to limitation in physical func- internally rotated. If this is accompanied by relative abduc-
tion or increase in symptoms such as pain and fatigue. In tion and/or external rotation of the contralateral side, the
nonambulatory children, contractures and deformities of the deformity is categorized as “windswept” towards the
lower limbs contribute to discomfort; difficulties with care, abducted/externally rotated side (Fig. 17.3).
270 U.G. Narayanan
anteversion also increases with GMFCS level but seems to

plateau at level III to level V, where it averages 40.0° (range
25° to 67.5°). Whether the abnormal proximal femoral geom-
etry is further affected by the abnormal forces associated
with adduction/flexion contractures is less clear. The reduced
femoral head coverage associated with increased femoral
anteversion is compensated for by increased internal rotation
of the femur and increased anterior tilt of the pelvis [15].
At birth, the hip joint is typically normal in children with
CP. Hip instability is usually acquired secondarily due to the
interaction between the adduction and flexion contracture
with the increased neck-shaft angle and anteversion, contrib-
uting to the (postero) lateral displacement of the femoral
head. The magnitude of the hip displacement (subluxation) is
quantified using the Reimer’s migration percentage (MP)
[22] (Fig. 17.5). The “hip at risk” is defined as the hip that is
likely to progress to hip dislocation if left untreated. This has
been variously defined as the hip that reaches a migration
percentage of 30–40 %. The proportion of children who will
reach this threshold is directly correlated with increasing
GMFCS level in three population-based studies [23–25].
Using 30 % as the definition of hip at risk, the incidence is
virtually 0 % for GMFCS level I but increases to 90 % for
level V [25]. If, however, a threshold of 40 % is used to define
a hip at risk, the incidence is 65 % for GMFCS level V [24].
This is probably a more valid estimate of the risk because it
has been observed that approximately one-third of hips with
Fig. 17.3 Windswept hips
an MP between 33 and 40 % will improve spontaneously or
will not deteriorate [24]. Progressive subluxation is very
In CP, the morphology of the proximal femur is often likely if the MP is greater than 40 % in a child still growing
characterized by a relative increase in the neck-shaft angle in (Fig. 17.6a–c). The posterolaterally displaced femoral head
the coronal plane (coxa valga) and excessive femoral ante- pushes up against the superolateral surface of the acetabulum
version in the transverse plane (Fig. 17.4a). Femoral antever- leading to increasing acetabular dysplasia. In association
sion is the angle in the transverse plane, by which the neck of with hip flexion contracture, the acetabular deficiency tends
the femur is directed (forward) from the transcondylar or to be more posterior [20]. The acetabular dysplasia is quanti-
coronal plane [16] (Fig. 17.4b). The terms femoral antever- fied by the acetabular index when the tri-radiate cartilage is
sion and femoral (medial) torsion are used interchangeably, open, or by the acetabular angle of Sharp when the tri-radiate
the latter term suggesting that the orientation of the proximal cartilage is closed [26]. Initially the subluxated femoral head
femur relative to the coronal plane is a consequence of tor- might reduce in the abducted position, but eventually the dis-
sion occurring along the shaft of the femur rather than in the location becomes irreducible. The uncovered femoral head is
neck [17–19]. In the newborn, both femoral anteversion and subject to the abnormal forces of the overlying capsule and
neck-shaft angle are increased. This might be related to the abductors draped over the superolateral surface of the femo-
fetal position. As the growing infant begins to roll over and ral head (Fig. 17.6d). This leads to loss of sphericity and pro-
crawl and pull up to stand and walk, the progressive exten- gressive flattening of this surface leading to increasing
sion of the hip is believed to push back the femoral neck axis femoral head deformity. Ultimately, there is erosion of the
to the physiologically normal values of older children and articular cartilage and degenerative changes consistent with
adults by the age of 1 or 2 years. The developmental delay of osteoarthritis (Fig. 17.6e).
CP results in the retention of uncorrected higher degrees of
fetal alignment [20]. This is borne out by the finding that the Knee and Lower Leg Segment
mean neck-shaft angle increases with the severity of CP by At the knee, the most common deformity is in the sagittal
GMFCS level from 135.9° (range 130° to 145°) at GMFCS plane, knee flexion deformity. The knee flexion deformity
level I to 163.0° (range 151° to 178°) at level V [21]. Femoral has a wide range of etiologies. It can arise from contracture
Fig. 17.4 (a) Coxa valga. (b)

Femoral anteversion. Adapted
with permission from Narayanan
UG. Chapter 26. In: Weisel SW
(editor). Pediatrics in Operative
Techniques in Orthopedic
Surgery. Vol. 2, Part 4.
Philadelphia: Wolters Kluwer
Health; 2011
at play. Initially a flexion deformity present during weight

bearing or walking may not be associated with a fixed flexion
contracture. Over time passive extension is likely to become
restricted with increasing magnitude of fixed knee flexion
contracture due to inadequate length of the hamstrings and/or
a posterior capsular contracture. Weight bearing in the crouch
position increases the joint reaction force on the patella-fem-
oral joint which can cause anterior knee, patella alta, and
avulsion fractures of the inferior pole of the patella (Fig. 17.7).
Box 17.1. Multiple Etiologies of Knee Flexion Deformity

1. True contracture of the hamstring/s
2. “Functional” contracture of hamstring/s: Increased
anterior pelvic tilt due to any cause raises the origin
(ischial tuberosity) of the medial hamstrings and
long head of biceps, which in turn contributes to
flexion at the distal insertion. Excessive anterior
Fig. 17.5 AP radiograph of the pelvis with radiographic parameters pelvic tilt can be due to:
quantifying hip displacement and acetabular dysplasia
(a) Hip flexion contracture
(b) Weak hip extensors
(c) Weak anterior abdominal muscles
of the medial and/or lateral hamstrings. Since the medial
(d) Compensatory strategy for excessive femoral
hamstrings (semitendinosus and semimembranosus) and part
anteversion
of the lateral hamstrings (long head of biceps femoris) take
3. Weak plantar flexion—Knee extension couple leads
origin on the ischium which is above the hip joint, a “func-
to forward lean of the tibia shank over the foot,
tional” contracture of the hamstrings is possible even in the
leading to the ground reaction force passing poste-
presence of normal or even excessive length of the ham-
rior to the knee, causing an increased external flex-
strings when there is an associated anterior pelvic tilt that
ion moment. Reasons for this include:
raises the origin of these muscles [13, 14, 27]. Knee flexion
(a) Weak gastrocsoleus complex, often iatrogenic
deformity might also be consequence of a weak gastrocso-
due to over-lengthened tendo-Achilles, in a
leus complex leading to the forward translation of the tibial
child with spastic diplegia
shank over the foot, leading to the ground reaction force
(b) Hindfoot valgus with midfoot break and fore-
passing posterior to the knee resulting in a (external) knee
foot abduction
flexion moment that has to be counteracted with a corre-
(c) Excessive external tibial torsion
sponding (internal) extension moment using the quadriceps.
4. Gastrocnemius contracture (dynamic or static):
If the knee extensors are weak, the knee goes into progres-
Knee bends to allow foot to be plantigrade by tak-
sively increased flexion. A gastrocnemius contracture can
ing the stretch off the contracted gastrocnemius.
also lead to flexion deformity of the knee to take the tension
5. Posterior capsular contracture of the knee joint.
off the gastrocnemius, especially if the foot is being held
6. Combinations of above.
plantigrade in a brace. A combination of these factors can be
272 U.G. Narayanan
True coronal plane deformity at the knee is uncommon.

However, the medial rotation of the hip, due to increased
femoral anteversion, which is characterized by a correspond-
ing medial rotation of the knee (patella), leads to an apparent
(pseudo) valgus appearance of the (flexed) knee. This appear-
ance is exacerbated if the femoral medial torsion or antever-
sion above the knee is accompanied by external tibial torsion
below the knee. The tibial segment can be associated with
normal torsion, and increased external or increased internal
tibial torsion. At birth, the torsion of the tibia is relatively
internal, with the bimalleolar axis typically being neutral in
the coronal plane. The tibia undergoes external rotation max-
imally during the first 4 years and an average of 1 degree per
year until skeletal maturity when it reaches an average of 28
degrees external [28]. In some children with CP, this internal
torsion may persist due to the failure of the natural lateral
rotation to occur. In others, significantly increased external
tibial torsion can occur often in association with valgus hind-
foot, flattening of the midfoot, and forefoot abduction. This
is associated with a poor lever arm for push off in gait and is
often accompanied by crouch position at the knee due to the
weak plantar flexion-knee extension couple (Fig. 17.8).
Ankle and Foot

Equinus of the ankle is the most common deformity of the
lower extremity in CP. Equinus arises from an imbalance
between the plantar flexors, which might be hypertonic and/
or contracted, and the dorsiflexors due to weakness of the
tibialis anterior. What begins as a dynamic contracture due to
spasticity is likely to become fixed when the gastrocnemius-
soleus complex fails to keep up with the tibial growth. In
bilateral CP, the gastrocnemius is usually more involved than
the soleus. Consequently, the ankle might have sufficient
dorsiflexion when the knee is flexed but with the knee
extended, the ankle equinus becomes evident due to the
stretch of the gastrocnemius muscle at its origin above the
knee (Fig. 17.9). In unilateral CP (hemiplegia) the soleus
tends to be equally involved as the gastrocnemius, such that
the equinus contracture is unchanged with the position of the
knee. This distinction is important to assess as it will influ-
ence which musculotendinous structure should be targeted
for intervention: gastrocnemius alone or both the gastrocne-
mius and the soleus or tendo-Achilles.
In the coronal plane, the hindfoot might be in physiologic
valgus position, or deformed into excessive valgus or varus.
These coronal plane deformities typically arise in combina-
tion (coupled) with ankle equinus. Equinovalgus deformities
are more common in bilateral CP (diplegics). With the hind-
foot in valgus, the talus and calcaneus are typically plan-
tarflexed. There is a collapse of the subtalar joint (midfoot
break) with the prominence of the head of the plantarflexed
Fig. 17.6 (a–c) Hip at risk progressing to increased subluxation and talus, and the midfoot is everted taking the forefoot with it
acetabular dysplasia. (d, e) Dislocated femoral head leading to loss of
sphericity and advanced degenerative changes
into an abducted position (Fig. 17.10a, b). There is a flatten-
Fig. 17.7 Bilateral patella alta

due to long-standing crouch with
avulsion fractures of the inferior
pole of patella
dorsiflexing the ankle. Failing to do so allows dorsiflexion to

occur about the unlocked subtalar joint and provide a spuri-
ous estimate of dorsiflexion. The equinovalgus foot with fore-
foot abduction provides a very ineffective lever arm to
generate any power during push off (Fig. 17.11a, b). The tibial
shank falls forward over the foot due to dorsiflexion through
the subtalar joint, and shifts the ground reaction force behind
the knee resulting in a flexed knee or crouched position.
Equinovarus deformities are more common in unilateral
CP (hemiplegics). The hindfoot varus might be a conse-
quence of an overactive tibialis posterior and/or tibialis
anterior muscle in combination with weak peroneals. The
deformity might be flexible or rigid. The hindfoot varus
might be accompanied by midfoot cavus due to the tight
plantar fascia or foot intrinsic muscles, and forefoot adduc-
tus. Weak tibialis anterior along with overactive peroneus
longus might lead to increased plantar flexion of the first ray.
This might be the primary problem resulting in a compensa-
tory hindfoot varus to maintain a weight-bearing tripod.
This can be tested using the simulated Coleman block test.
In the prone position, the lateral border is pushed down on to
simulate standing on the Coleman block [29]. The hindfoot
varus corrects to normal valgus and the increased plantar
flexion of the first metatarsal ray becomes apparent relative
to the remaining rays, confirming that the hindfoot varus is
secondary to the plantarflexed first ray. The hindfoot varus
leads to increased weight bearing along the lateral border of
the foot leading to callus formation and even stress fractures
causing discomfort and difficulties with brace and shoe wear
Fig. 17.8 Torsional malalignment: Pseudo-valgus due to increased
(Fig. 17.12).
femoral internal torsion and increased external tibial torsion
ing of the longitudinal arch and a pronated appearance, Lower Limb Assessment in the Ambulant Child
although in fact the foot might be relatively supinated to com- (GMFCS Levels I–III)
pensate for the hindfoot valgus. Passive correction of the
hindfoot out of the valgus position unmasks the supinated The physical examination of the ambulant child begins with
position of the foot. The abducted forefoot is often accompa- a visual assessment of the gait to identify overall abnormal
nied by a hallux valgus deformity that is acquired over time gait patterns and specific problems. This dynamic assess-
due to the foot rolling off the medial border during push off. ment is followed by an on-table examination of the lower
To test the true dorsiflexion of the ankle, the hindfoot valgus limb tone abnormality, range of motion of the major joints,
must be passively corrected to lock the subtalar joint prior to and skeletal alignment to explain the gait abnormalities identified
274 U.G. Narayanan
Fig. 17.9 Equinus contracture involving the gastrocnemius more than the soleus (Silfverskiold test)
Fig. 17.10 (a) Equinoplanovalgus deformity with hindfoot valgus, midfoot break, forefoot abductus, hallux valgus. (b) Weight-bearing lateral
radiograph of equinoplanovalgus deformity
Fig. 17.11 (a) Lever arm of the normal foot. (b) Lever arm deficiency Springer-Verlag; 2003, with kind permission from Springer Science
of the equinoplanovalgus and abducted foot. Figures reproduced with and Business Media
permission from Paley D. Gait considerations. Berlin, Heidelberg:
Observation of Gait
Box 17.3. Five Prerequisites of Gait
1. Stability of the weight-bearing side during stance
2. Clearance of the foot in swing phase
3. Appropriate pre-positioning of the swing-phase
foot for the next step
4. Adequate step length
5. Energy conservation
Observation of Gait
Inspect the child’s walk from the front, back, and the side to
best estimate the gait abnormalities in all three planes. The
child should walk both in their usual orthotics and footwear
and barefoot if possible. The assessment is both qualitative
and quantitative. A useful framework to evaluate the global
quality of the gait is to assess the five prerequisites or attri-
butes of gait [30, 31]: (1) stability in stance; (2) (foot) clear-
ance in swing; (3) correct pre-positioning of foot for the next
step; (4) adequate step length; and (5) energy efficiency
(minimizing the excursion of the body’s center of mass dur-
ing walking). This is followed by a systematic assessment
for specific abnormalities at each level: foot and ankle;
Fig. 17.12 Equinocavovarus deformity in a hemiplegic child. Note the
knees, hips, and pelvis; and trunk and upper extremities, with
plantarflexed first ray and the stress fracture of the 5th metatarsal due to due consideration of the coronal, sagittal, and transverse
excessive loading of the lateral border planes to estimate the reasons for any compromise in any
five priorities of gait. This quantitative assessment can be
greatly enhanced by capturing the gait with video (ideally
during walking. These should in turn be linked to specific split screen to see the frontal and sagittal planes simultane-
gait-related problems reported by the child during the elicita- ously and in slow motion) [32].
tion of the history prior to the physical examination. Gait analysis provides a more sophisticated assessment of
gait patterns and deviations. Three-dimensional gait analysis
generated in a motion laboratory provides kinematic data
Box 17.2. Assessment of the Ambulatory Child that describes the dynamic position of each limb segment in
1. Generate gait-related problem list from child and each of the three planes; kinetic data which includes moments
parent/s and powers about the joints; dynamic electromyography
2. Observational assessment of the gait: Dynamic which documents the timing of specific muscle activation
exam during the gait cycle; and pedobarographs which provide
(a) With and without orthotics useful information about the weight-bearing characteristics
(b) With and without walking aids if possible of the stance-phase foot. 3-D gait analysis provides insight
3. Examination on the table: Static exam beyond what is derivable from observational analysis alone,
(a) Muscle tone and can help distinguish primary abnormalities (which might
(b) Muscle length: Passive range of motion benefit from treatment) from secondary abnormalities that
• Fast stretch (R1) represent coping responses (which requires no treatment)
• Slow stretch (R2) [32–34]. Gait analysis has the potential to influence or alter
(c) Muscle strength treatment decisions for at least some patients [35–37]. Gait
(d) Selective control analysis has identified recognizable gait patterns which can
(e) Bony alignment be classified and used for making treatment decisions [38,
4. Radiographic imaging 39]. Finally, the effectiveness of these interventions can be
5. Video and 3-D gait analysis assessed objectively using gait analysis as a measure of gait
outcomes [40].
276 U.G. Narayanan
Abnormal Gait Patterns in Cerebral Palsy to facilitate clearance of the plantarflexed foot, leading to
In bilateral CP, there are broadly recognizable patterns [38] foot drag and tripping. Step length is typically shortened
(Fig. 17.13). because the plantarflexed foot leads to an early forefoot
True equinus pattern is characterized by the absence of a strike. The decreased step length might also be a consequence
heel strike and a toe-toe pattern because the ankle remains of poor stability in the stance-phase limb forcing the contra-
plantarflexed throughout stance phase. If the equinus is not lateral swing-phase limb to contact the ground sooner.
severe, initial contact may be with the forefoot and the heel Jump gait pattern is characterized by toe-toe pattern due
might even come in contact with the ground in midstance. to equinus in combination with a pathologically flexed
The knee can be flexed during stance phase to accommodate knee and flexed hip that resembles someone preparing to
the equinus, or it may come to full extension or even hyper- jump. All five priorities of gait are affected in this pattern.
extend to allow the plantarflexed foot to remain flat on the Step length is further compromised as the swing-phase
ground for more stability (Fig. 17.14a). True equinus pattern knee might not be able to extend if increased hamstring
can be associated with poor stability due to the narrow base activity prevents the knee from reaching full extension in
of support and poor clearance if the knee does not flex enough terminal swing.
Fig. 17.13 Common gait

patterns in bilateral (diplegic)
cerebral palsy. Reprinted with
permission from Rodda J,
Graham HK. Classification of
gait patterns in spastic
hemiplegia and spastic diplegia:
a basis for a management
algorithm. Euro J Neurol 2001; 8
(Suppl 5): 98–108
Fig. 17.14 (a) Knee

hyperextended to accommodate
equinus contracture with flatfoot
contact despite plantarflexed
position of ankle. (b) Forefoot
contact (apparent equinus) to
accommodate fixed flexion
deformity of the knee
Fig. 17.15 (a) Crouch gait pattern: Flexed knee with forward transla- hip, resulting in ankle plantar flexion, knee extension, and hip extension
tion of the tibia over the dorsiflexed ankle. (b) Crouch gait pattern in the (internal) moments to counteract the abnormal external moments gen-
presence of equinovalgus foot. (c) When in crouch the ground reaction erated by the ground reaction force
force passes in front of the ankle, behind the knee and in front of the
In apparent equinus pattern, the child’s heels do not come bottom-like foot. In either situation the tibial shank is flexed
in contact with the ground even though the ankle is not in forward over the foot segment throughout stance. This shifts
equinus (Fig. 17.14b). This happens when there is a fixed the ground reaction force well behind the knee, creating an
flexion deformity of the knee. If there was an adequate range external flexion moment that has to be neutralized by a cor-
of ankle dorsiflexion this fixed knee flexion deformity can be responding internal extension moment by the activation of
accommodated to keep the center of gravity over the base of the quadriceps. In order to keep the center of gravity over the
support, which is what one finds in the crouch gait pattern. If, base of the support, the hips must flex (Fig. 17.15c). Crouch
however, the ankle has only sufficient range to come to just pattern seems to be the consequence of the natural history in
neutral dorsiflexion, keeping the foot flat on the ground children with more severe bilateral CP (GMFCS III or
would cause the child’s center of gravity to fall well behind greater). All five priorities of gait are affected, and the energy
the base of support in the presence of an uncorrectable flexed consumption associated with this pattern is substantial.
knee. To stop from falling backward the body is forced to Weight bearing in the crouch position increases the joint
shift the center of gravity forward by flexing the hip and reaction force on the patella-femoral joint which can cause
increasing forward trunk lean or tipping forward on tiptoe. anterior knee pain, patella alta, and avulsion fractures of the
This pattern is characterized by problems with all five priori- inferior pole of the patella.
ties of gait. Each of the above patterns can be associated with a stiff
Crouch gait is characterized by flexed knee during stance knee pattern. Stiff knee gait is characterized by limited flex-
phase accompanied by dorsiflexed foot segment relative to ion in initial swing due to tightness of the rectus femoris,
tibial shank. This dorsiflexion can occur through the ankle which affects clearance of the foot in swing phase.
(true calcaneus) when there is unrestricted range of dorsi- Consequently the feet might drag, slowing the gait, causing
flexion of the ankle, which could be iatrogenic following tripping and increased shoe wear.
prior over-lengthening and weakening of the gastrocsoleus Any of the patterns can also be associated with transverse
from tendo-Achilles lengthening (Fig. 17.15a). However, plane abnormalities that can arise from one or more of the
crouch pattern is often present even in the presence of equi- following levels: the forefoot (adductus or abductus), hind-
nus of the hindfoot, when the equinus is accompanied by foot (varus or valgus), tibial torsion (internal or external),
increased hindfoot valgus (Fig. 17.15b). In this position, the femoral anteversion (or torsion), hip rotation (internal or
subtalar joint collapses and “dorsiflexion” occurs through external), or pelvic rotation (protraction or retraction). The
the subtalar joint accompanied by midfoot break and a rocker foot position can be quantified by the foot progression angle
278 U.G. Narayanan
both in swing and stance phases with a toe-toe or toe-heel

pattern in stance phase. This is due to a contracture of the
gastrocsoleus complex which might be dynamic due to spas-
ticity or fixed due to static contracture. In Type 3 pattern, the
knee is involved as well, showing abnormal flexion (jump) or
stiff knee pattern. Finally, in Type 4 there is additional proxi-
mal involvement at the hip, including varying combinations
of hip adduction, flexion, and internal rotation. This pattern
can be particularly troublesome as it is often accompanied by
progressive hip displacement and dysplasia.
These gait patterns are recognizable, but the distinctions
are not always clear with many permutations and combina-
tions involving one or both lower extremities.
On-Table Physical Examination

The static examination (on the table) includes assessment of
muscle tone and length, bony alignment, muscle strength,
and selective control.
Assessment of Muscle Tone and Length

Muscle tone tested is assessed for each muscle group by test-
ing the relevant passive joint range of motion affected by that
muscle group. Range of motion is assessed passively with a
fast stretch that elicits a catch at an angle (R1) often well
before the maximal passive range obtained by a slow, sus-
tained stretch (R2) [42, 43]. This is a feature of spasticity,
which is velocity-dependent hypertonia. The larger gap
between the R1 and R2 represents the dynamic component
of the contracture, and is more amenable to spasticity man-
agement. R2 represents the functional muscle length. AS R2
Fig. 17.16 Internal rotation gait pattern approaches R1, there is increasing static contracture or true
shortening of the muscle tendon unit (in addition to tight-
(FPA), normally, an average of 5 degrees external [41]. The ness). Often, biarticular muscles are more involved than
knee progression angle should also be assessed as this will muscles that cross only one joint. It is therefore important to
help determine the source of rotational abnormality. A normal separately test the length of both groups of muscles (when
FPA does not rule out torsional malalignment. In children possible) that contribute to range in order to intervene selec-
with bilateral CP it is not unusual to have increased internal tively on the biarticular muscles.
rotation of the hips due to increased femoral anteversion The following specific joints (muscle groups) of the lower
characterized by internal knee progression angle (patella extremity are tested in the supine position. Hip flexors are
points medially) and apparent “scissoring” (Fig. 17.16). Foot tested by extending the hip (Thomas test) while with the
progression angle may be internal, normal, or even external if opposite hip is held sufficiently flexed to neutralize the ante-
accompanied by increased external tibial torsion and pla- rior pelvic tilt and, but not overcorrect, the lumbar lordosis.
novalgus abducted foot. Internal rotation of the hip in the face Hip abduction is tested first with the knees flexed to test the
of external rotation of the tibial segment creates a pseudo- adductor muscle length. Abduction is then tested with the
valgus appearance at the knee (miserable malalignment). hips and knees held in extension to assess the additional con-
In unilateral CP (hemiplegia) four types of gait have been tribution of the gracilis which is a hip adductor and a knee
described [38, 39] (Fig. 17.17). Type 1 pattern is character- flexor. The Galeazzi sign assesses the relative lengths of the
ized by equinus in swing phase only. This is a drop foot pat- thigh segment. A shorter thigh segment in combination with
tern due to weakness of the tibialis anterior which is unable an adduction contracture might be indicative of hip sublux-
to adequately dorsiflex the ankle during swing phase. Initial ation on that side. Knee extension is quantified to rule out a
contact might be with the forefoot but the heel comes in con- knee flexion contracture. This could be due to one or more
tact with the ground early in stance with adequate dorsiflex- of the following: shortened hamstrings, shifted hamstrings
ion in midstance. Type 2 pattern is characterized by equinus due to increased anterior pelvic tilt, shortened proximal
Fig. 17.17 Common gait patterns in unilateral (hemiplegic) cerebral palsy
Fig. 17.18 Unilateral and

bilateral popliteal angles: The
hamstring shift test
gastrocnemius, or a posterior contracture of the knee joint test) to distinguish contractures arising from the gastrocne-
capsule. The popliteal angle tests the functional length of the mius alone from those where the soleus is involved as well.
hamstrings. This is first done unilaterally (one leg at a time) At the time of testing ankle dorsiflexion, any hindfoot valgus
with the hip on the tested side held in 90° of flexion, while must be corrected to neutral by supinating the foot to ensure
the contralateral hip is held in full extension. The bilateral that the subtalar joint is “locked” to ensure that dorsiflexion
popliteal angle or the hamstring shift test requires testing the is occurring through the ankle joint rather than spuriously
popliteal angle with the contralateral hip and knee flexed to through the subtalar joint (see Fig. 17.9). Hindfoot is tested
neutralize the pelvic tilt similar to the Thomas test [27]. This for its flexibility to correct any valgus or varus. If a cavovarus
is a measure of the “true” hamstring contracture (Fig. 17.18). foot deformity is present, simulate a Coleman block test, as
A large difference (reduction) in the popliteal angle (increased described earlier, to identify whether the hindfoot varus is
available hamstring length) suggests that anterior pelvic tilt fixed or not and if not confirm that it is secondary to the plan-
might be responsible for the relative elevation of the ischium tarflexed deformity of the first metatarsal ray or a primary
which is the origin of the medial hamstrings and the long problem [29]. In a cavovarus foot, the hindfoot might be in
head of the biceps femoris. Ankle dorsiflexion is tested both equinus, neutral, or even relative calcaneus with the forefoot
with the knee flexed and the knee extended (Silfverskiold plantarflexed relative to the hindfoot.
280 U.G. Narayanan
Fig. 17.19 Femoral anteversion

with trochanteric palpation test
In the prone position, knee flexion is tested rapidly and then

slowly for the Duncan–Ely (DE) sign. A positive sign would
be associated with a significant elevation of the buttock off the
bed, due to an increase in hip flexion and anterior pelvic treat
created when the short and/or spastic rectus femoris is being
stretched and pulls down on its origin at the anterior inferior
iliac spine causing the hip to flex and pelvis to tilt anteriorly.
Assessment of Bone Alignment

Assessment of the bony alignment includes the examination
of the torsional profile of the lower limbs in the prone posi-
tion to evaluate for excessive femoral anteversion and tibial
torsion. The magnitude of femoral anteversion can be esti-
mated using the trochanteric palpation test. In the prone
position, the knee is flexed to 90° and held upright or perpen-
dicular to the examination table. In this baseline position, the
patella is pointing directly “anterior” into the table. The
prominence of the greater trochanter is palpated in the proxi-
mal lateral thigh. In this baseline position, the maximum Fig. 17.20 Assessment of tibial torsional profile: The TFA and TMA
prominence of the greater trochanter sits more posteriorly.
The lower leg (tibia) serves as a goniometer. The lower leg is
rotated outward which corresponds to internal rotation of the fibula. This is best quantified by the TMA which is the angle
hip, until the prominence of the greater trochanter is palpated between the trans(bi)malleolar axis and the transverse knee
most laterally, which corresponds to the femoral neck (axis) axis, which is usually between 10 and 30 degrees external.
being horizontal and parallel to the floor. The number of TMA less than or greater than this range represent internal
degrees of outward rotation of the tibia relative to the verti- and external tibial torsion, respectively [46] (Fig. 17.20).
cal position at baseline represents the femoral anteversion on
that side [44, 45] (Fig. 17.19). Tibial torsional alignment is Assessment of Muscle Strength and Selective Control
based on thigh foot angle (TFA) and the transmalleolar In children with CP, there is increasing evidence that muscles
(bimalleolar) axis (TMA). With the ankle held in neutral dor- are weak, and that motor function is directly associated with
siflexion, the TFA is the angle between the long axis of the muscle strength [47]. It is therefore important, whenever pos-
foot (heel bisector to second toe) and the thigh axis, which is sible, to assess muscle strength for prognostication and clini-
usually between 10 and 20 degrees external [41]. If the lat- cal decision making [48]. Muscle strength is tested for each
eral border of the foot is straight, one can infer that there is of the lower limb muscle groups using manual muscle testing
no deformity arising from the forefoot and that an abnormal (MMT) [49]. These tests are somewhat subjective and reliant
TFA is arising from the torsional alignment of the tibia and on the examiner’s experience, and are only possible in older
children who are capable of cooperating with the manual

testing. In the gait laboratory and in research settings, isomet- Box 17.4. Goals of Treatment: Ambulatory CP
ric muscle strength testing using dynamometers or isokinetic (GMFCS Levels I–III)
evaluations has some value. 1. Optimize gait efficiency (correct biomechanics) in
Selective motor control is impaired in children with CP order to optimize energy conservation
and contributes to ambulatory function and patterns [50, 51]. (a) Preserve or improve physical function
The ability to isolate movements can be graded into three lev- • Walk longer distance
els of control: (0) no ability; (1) partial ability; and (2) normal • Walk faster
ability to isolate movements [52]. These are tested and • Decrease fatigue
reported along with the strength for each muscle group tested. • Better stability: Reduced tripping and fewer falls
• Keep up with friends
(b) Pain relief or pain prevention and increased
Lower Limb Assessment in the Non-ambulant endurance
Child (GMFCS Levels IV–V) (c) Preserve or increase activities and participation
• More physically active
In the non-ambulant child the physical examination should • More independence
include an assessment of the child seated in the wheelchair • More participation in sports/recreational activity
or seating system, and assessment lying on the table. In the 2. Improve appearance of gait
chair, specific attention is paid to the presence, flexibility, (a) Reduced reliance on walking aids
and impact of any spinal deformity in the coronal (scoliosis) (b) Reduced use of orthotics
and sagittal (kyphosis/lordosis) planes, and pelvic inclina- (c) Feet flat on the ground
tion on sitting balance, ischial weight bearing, skin integrity, (d) Feet pointing forward
and hygiene. Particular areas that are vulnerable to skin (e) Reduced dragging of feet
breakdown are over bony prominences including those due (f) Stand and walk taller (knees less bent)
to kypho-scoliosis, sacrococcygeal area, ischial weight- (g) More symmetry
bearing area, and prominences of the greater trochanter or
dislocated femoral heads in the lateral proximal thigh.
Additionally, in the presence of a large, rigid scoliosis there Goals of treatment for nonambulatory children (GMFCS
can be skin breakdown on the side of the concavity where the IV and V) are completely different from those for ambula-
inferior aspect of the rib cage might rest against the iliac tory children. These more severely involved children are at
crest and skin crease in the flank might be hard to reach or high risk of developing joint contractures, progressive hip
maintain hygiene due to stiffness of the deformity. displacement, and spinal deformity that interfere with com-
The lower extremity examination of the non-ambulant fort and caregiving, positioning, transfers, mobility, and seat-
child is done in the supine position. The examination of the ing. The musculoskeletal involvement occurs in the context
hip joint, knee joint, and foot and ankle is identical to that of other associated comorbidities including seizure disor-
described for ambulant children. Usually, there is little value ders, visual and hearing impairments, cognitive delays, swal-
in turning the child prone to assess the torsional profile, as lowing difficulties, drooling, aspiration and pneumonias,
these can be reasonably assessed supine. gastroesophageal reflux, malnutrition, constipation, and
incontinence. The goals of treatment are to prevent or relieve
pain, facilitate caregiving, improve health, and optimize the
Goals of Treatment in Cerebral Palsy quality of life [54].
by GMFCS Level
For ambulatory children (GMFCS levels I–III) the primary

goals of treatment are to optimize gait efficiency in order to Box 17.5. Goals of Treatment: Nonambulatory CP
preserve or improve independence and physical function that (GMFCS Levels IV–V)
will permit increased participation in real-world life activi- 1. Relieve or prevent pain and discomfort
ties. Functional problems of gait impact walking distance, 2. Facilitate ease of care
speed, balance and stability, endurance, and fatigue, and may (a) Dressing, toileting, bathing/hygiene
lead to increasing discomfort. Improving the appearance of (b) Positioning: Seating and lying down
gait and minimizing the reliance on walking aids and orthot- (c) Transfers and mobility
ics are also identified as important priorities by children with 3. Preserve or improve health
CP and their parents [53]. 4. Improve quality of life
282 U.G. Narayanan
appearing promising in small series of selected patients

Management of Lower Extremity Problems: [68–70]. Baclofen is a GABA agonist that can reduce hyperto-
Principles of Treatment and Techniques nia. The relatively large oral doses required for any meaning-
ful effect can be associated with undesirable side effects.
In general, the principles of treatment include (1) prevention When administered intrathecally, very small doses (micro-
or slowing the development of progressive joint contrac- grams) are required to reduce severe generalized hypertonia
tures, bony deformities, and joint instability in the younger due to spasticity or dystonia. Intrathecal baclofen adminis-
child, and (2) correction of these clinically significant bony tered continuously by an implantable pump is usually reserved
deformities and joint instability when these have already for more severely involved children (GMFCS level V), as it
occurred. These objectives are best achieved using a multi- can be associated with significant complications [71–73].
disciplinary approach. Established deformities are most efficiently addressed
Early intervention focuses on dealing with dynamic con- with single-event multilevel orthopedic surgery (SEMLS)
tractures in order to delay the need for more extensive surgi- using a combination of fractional muscle or intramuscular or
cal procedures (Fig. 17.21). Multiple strategies are used tendon lengthening, tendon transfers, and corrective osteoto-
including physical therapy to stretch muscles, orthotics mies and joint stabilization procedures to address bony
(braces) to maintain stretch or stabilize joints, and serial cast- deformities and joint instability that contribute to lever arm
ing in an attempt to simulate the stretch that would normally dysfunction [74, 75]. Surgery is delayed until the gait pat-
arise from typical physical activity. Stretch is facilitated by terns have matured and any functional gains from the natural
local or systemic tone management by pharmacologic (botu- history have plateaued [76]. The optimal timing is sometime
linum toxin-A, phenol injections) or neurosurgical methods between 7 and 11 years of age, to reduce the likelihood of
(selective dorsal rhizotomy, intrathecal baclofen). Effective recurrence of deformity with further growth necessitating
tone management improves range of motion and tolerance of repeat surgery (see Fig. 17.21). In the short term, SEMLS is
brace wear, and might prevent or delay the onset of static associated with substantial improvements in gait quality,
contractures and bony deformities [55–60]. Severe spasticity with more modest corresponding functional improvements
involving bilateral lower extremities in children with good [77–79]. In general, the children with more abnormal gait
balance and muscle strength might benefit from more exten- patterns stand the most to gain, but about 25 % show little
sive spasticity control from selective dorsal rhizotomy measurable benefit. It is unclear whether these patients
(SDR), which has been shown in randomized trials to pro- would have deteriorated further without the intervention
duce significant reduction in muscle tone, but only modest [80]. There is very little information in the literature about
benefits on physical function [61–64]. SDR does not neces- the long-term effects of multilevel orthopedic surgery at
sarily reduce the need for subsequent orthopedic surgery skeletal maturity or adulthood [77, 81–83].
[65–67]. In a systematic review, the functional effects and Table 17.1 summarizes the most common surgical proce-
benefits of SDR in the long term remain uncertain, despite dures for cerebral palsy and their indications.
Fig. 17.21 Management

strategies and timing principles.
Reprinted from Current
Orthopedics, 17, Bache CE SP,
Graham HK, The management of
spastic diplegia, 88-104,
Copyright 2003, with permission
from Elsevier
17
Table 17.1 Common soft-tissue and bony procedures performed during multilevel surgery for cerebral palsy
Indications for surgery Procedure
Hip flexion contracture: Psoas Ambulatory Child Ambulatory Child
• Reduced hip extension in terminal stance Selective intramuscular lengthening of the psoas tendon over the brim
• Excessive anterior pelvic tilt + lordosis of the pelvis (spare the iliacus)
• Hip flexion contracture >20° Note: Proximal femoral derotational osteotomy done above the lesser
• Hip subluxation trochanter can functionally lengthen the psoas
• Kinematic: Excessive anterior pelvic tilt with double-bump pattern in
diplegia; single-bump pattern in hemiplegia
• Kinetics: Delay in transition from hip extensor (internal) moment to hip
flexor (internal) moment (beyond the typical 25 % of gait cycle)
Nonambulatory Child Nonambulatory Child
• As part of the management of hip displacement Iliopsoas tenotomy or release of the lesser trochanter
Hip adduction contracture: adductors Ambulatory Child Ambulatory Child
Note: During normal gait the arc of hip • Decreased abduction in swing phase (scissoring) Percutaneous or open release of adductor longus alone is usually sufficient
motion in the coronal plane is from 10° of • Adduction contracture: <15°
abduction to 10° of adduction. 15° of Nonambulatory Child Nonambulatory Child
abduction is sufficient for normal gait • Adduction contracture interfering with care: dressing, toileting, perineal Open release of adductor longus, adductor brevis, gracilis + iliopsoas tenotomy
hygiene
• As part of the management of hip displacement
Hip internal rotation: Anteversion Ambulatory Child Ambulatory Child
• Excessive internal rotation of the hip due to increased femoral anteversion Proximal femoral (intertrochanteric) derotational osteotomy with internal
• Increased “scissoring” with knees rubbing fixation
• Internal foot progression angle—causing tripping Alternative: Distal femoral derotational osteotomy
• As part of the management of hip displacement Combined proximal femoral derotation + varus osteotomy with stable
internal fixation
Proximal femur: Coxa valga and hip • Increased neck-shaft angle Proximal femoral (intertrochanteric) varus and external derotational
subluxation • Migration percentage >30 % osteotomy (VDRO)
• Break in Shenton’s arc
• Increased anteversion
Pelvis: Acetabular dysplasia • Acetabular index >30° Periacetabular pelvic osteotomy (e.g., Dega) to restore posterolateral coverage
• Subluxated hip
• Older than 5 years
Knee flexion: Hamstrings Ambulatory Child Ambulatory Child
• Decreased knee extension in terminal swing Medial hamstring lengthening: Intramuscular tenotomies of the gracilis,
• Increased popliteal angle, provided that semitendinosus; aponeurotic lengthening (striping) of semimembranosus
– Bilateral popliteal angle >30° Lateral hamstring lengthening: Fascial striping of the biceps femoris
• Kinematics: aponeurosis. This is avoided unless there is posterior pelvic tilt present to
– Posterior pelvic tilt prevent excessive weakness of hip extensors.
– Decreased knee extension in terminal swing
• Knee flexion contractures due to hamstring contracture interfering with care, Percutaneous or open hamstring lengthening: Medial and lateral
dressing, seating, and positioning. Goal is to decrease contracture
(and popliteal angle) and not to straighten the knee entirely
Lower Limb Deformity in Neuromuscular Disorders: Pathophysiology, Assessment, Goals, and Principles of Management
Knee flexion contracture • Knee flexion contractures >20° despite hamstring lengthening Distal femoral extension osteotomy: This must be accompanied by patella
• Hamstring length is sufficient (normal) tendon shortening or patellar tendon distal advancement to re-tension the
• Older children (closer to skeletal maturity): >12 years elongated knee extensors following the extension osteotomy
• Significant crouch pattern in gait (GMFCS II–III) or during standing for
transfers (GMFCS IV)
283
(continued)
Table 17.1 (continued)
284
Indications for surgery Procedure

Stiff knee: Rectus femoris • Stiff knee pattern: Limited flexion at initial swing Rectus femoris tendon is separated from the underlying vasti and the
• Foot drag resulting in poor clearance detached tendon is tenodesed to the semitendinosus, gracilis, or sartorius
• Positive Duncan–Ely sign
• Kinematic: Peak knee flexion in initial swing is decreased in amplitude and
delayed
• EMG: RF active throughout gait cycle
• Avoid in more severe GMFCS III and IV
Tibial torsion: External • External torsion: Bimalleolar axis >30° external Distal tibial internal derotational osteotomy to correct bimalleolar axis to
– Out-toeing: External foot progression angle >30° 10° external (concomitant fibular osteotomy may not be needed for
– Foot drag derotations of <30°)
– With planovalgus/abducted foot
– Relatively normal foot progression angle in the presence of increased femoral
anteversion that is being corrected by external derotation of the femur
Tibial torsion: Internal • Internal torsion: Bimalleolar axis >10° internal Distal tibial external derotational osteotomy to correct bimalleolar axis to
– Intoeing: Internal foot progression angle >10° internal 10° external (concomitant fibular osteotomy may not be needed for
– Tripping derotations of <30°)
Equinus: Gastrocnemius-soleus Ambulatory Child Gastrocnemius contracture alone (Silfverskiold): Isolated lengthening of
• Equinus in stance phase: Poor stability the gastrocnemius (Strayer technique)
• Equinus in swing: Poor clearance Gastrocnemius > soleus contracture (Silfverskiold test):
• Tripping and falling Strayer + soleus fascial lengthening by striping the aponeurosis leaving
• Increased shoe wear muscle intact or gastrocsoleus aponeurotic lengthening (e.g., Baker, Vulpius)
Nonambulatory Child
• Contracture interfering with bracing or footwear necessary for weight bearing
for transfers (GMFCS IV)
• Contracture interfering with bracing or footwear, or resting of feet on the
footplate of wheelchair (GMFCS V)
Note: These apply to any foot deformity in nonambulatory children
Equinus: Tendo-Achilles • Fixed equinus contracture involving both gastrocnemius and soleus, seen Tendo-Achilles lengthening is the most aggressive method of lengthening
commonly in hemiplegia the gastrocsoleus complex. This can be done by 2 or 3 hemitenotomy
sliding lengthening technique or open z-lengthening
Equino (plano)valgus • Increased hindfoot valgus with collapse of subtalar joint (rocker bottom) and Lateral column lengthening through the calcaneus, along with plantar
abducted forefoot flexion osteotomy of first ray. May need lengthening of peroneus brevis;
– Poor push off (tendency to crouch) lengthening of gastrocsoleus as needed to address the unmasked equinus
– Pain with weight bearing after correction of hindfoot valgus
– Blistering or callosity over medial prominence of talar head Triple C osteotomy: Medial slide osteotomy of calcaneus; opening wedge
– Loss of flexibility: Unbraceable osteotomy of cuboid: closing wedge osteotomy of medial cuneiform
Equino (cavo) varus • To prevent or correct weight bearing on lateral border of the foot which leads to Split tibialis anterior tendon transfer to the cuboid if hindfoot varus is
– Poor stability in stance flexible and occurs primarily in swing phase
– Pain and callosity Split tibialis posterior tendon transfer to the peroneus brevis when the
– Difficulty with brace and shoe wear hindfoot varus is flexible but occurs in stance phase as well
– Stress fracture of the fourth or fifth metatarsal Requires lengthening of tibialis posterior: Intramuscular tenotomy. Tibialis
– Poor clearance in swing posterior tendon lengthening may be combined with the split tibialis
• Flexible hindfoot varus is amenable to tendon transfers anterior tendon transfer
• Fixed contractures of tibialis posterior require lengthening Plantar fascia release along with extension osteotomy of the base of first
• Hindfoot varus driven by the presence of plantar flexion of the first ray metatarsal or medial cuneiform; consider transfer of the peroneus longus to
(confirmed by the Coleman block test) the peroneus brevis. These may be combined with tibialis posterior tendon
lengthening
All of these may require gastrocsoleus or tendo-Achilles lengthening as
U.G. Narayanan
needed to address the equinus contracture

Orthopedic Procedures at the Hip Femoral Derotation and Varus Derotational

Osteotomy
Iliopsoas Lengthening When there is a clinically significant internal rotation gait
The indication for psoas lengthening in ambulatory children due to increased femoral anteversion, the excessive antever-
is controversial [14, 84]. In the presence of a hip flexion con- sion can be corrected by derotational osteotomy proximally
tracture, accompanied by increased anterior pelvic tilt result- (inter-trochanteric) or distally [88, 89]. The proximal oste-
ing in reduced hip extension in terminal stance, selective otomy allows for the addition of varusization when there is
intramuscular lengthening of the psoas (sparing the iliacus) true coxa valga and some subluxation [90] (Fig. 17.22a). The
can be done over the brim of the pelvis [85]. In nonambula- osteotomy is proximal to the insertion of the iliopsoas on the
tory children with hip instability, iliopsoas tenotomy is per- lesser trochanter, resulting in some functional lengthening of
formed at the level of insertion into the lesser trochanter. the psoas that occurs with external rotation of the lesser tro-
chanter. The proximal osteotomy can be done supine or
Adductor Releases prone, the latter having the advantage of providing reliable
In ambulatory children adductor lengthening is only neces- (bilateral) intraoperative assessment of the torsional profile.
sary if the adduction contracture is severe. During normal Internal fixation with a blade plate or locking plate often pro-
gait the coronal plane arc of motion of the hip is limited from vides sufficient stability to avoid postoperative casting [91]
10° of abduction to 10° of adduction. Abduction of 15° with (Fig. 17.22b, c).
the hip and knee in extension is compatible with normal gait Windswept deformities are not uncommon in more
[86]. In the ambulatory child with CP, adductor surgery is severely involved nonambulatory children. These hips
usually restricted to percutaneous or open release of adductor require an approach that addresses the asymmetric hip
longus alone. A proximal tenotomy of gracilis may be added deformity. The adducted and internally rotated side requires
if the range of hip abduction is further reduced when exam- releases of the adductors, flexors, and hamstrings along with
ined with the knee in maximal extension. More extensive an external derotational osteotomy of the femur. On the
adductor releases to include the adductor brevis are seldom abducted and externally rotated side, the abductors, iliotibial
indicated in ambulant children unless there is a more severe band, and lateral hamstrings may have to be released, and
adduction contracture interfering associated with increased derotational osteotomies of the femur are based on whether
hip displacement [86]. In a non-ambulant child, adductor and by how much the femur is anteverted. The “abducted”
releases help to relieve adduction contractures that can inter- side may just be less adducted and require similar but less
fere with care, dressing, and perineal hygiene, and are rou- extensive procedures than on the more adducted side.
tinely lengthened when the contracture is associated with
increased hip displacement either in isolation or along with Periacetabular Pelvic Osteotomy
proximal femoral osteotomies to center the hip. The adductor In the presence of hip subluxation, acetabular dysplasia
longus, adductor brevis, and gracilis are generally released develops secondarily due to the mechanical pressure of the
from their origins through a medial transverse groin incision subluxating femoral head. The acetabular deficiency is usu-
just distal to the groin crease, centered over the adductor lon- ally posterolateral. Following proximal femoral varus dero-
gus tendon. The same approach allows access to the iliopsoas tational osteotomy to redirect the femoral head into the
tendon insertion at the lesser trochanter. Adductor releases center of the acetabulum, posterolateral coverage is improved
alone are insufficient to stabilize hip subluxation and more with a Dega-type periacetabular osteotomy or one of its
than 75 % of the time for GMFSC levels IV and V children, adaptations, which comprises a lateral opening wedge oste-
subsequent bony reconstructive surgery is required [87]. otomy through the outer table, hinging on or just proximal to
Fig. 17.22 (a) An 8-year-old girl with bilateral hip subluxation, coxa and periacetabular pelvic osteotomy (Dega). (c) Frog-leg lateral radio-
valga, and acetabular dysplasia. (b) AP radiographs after bilateral prox- graph after bilateral proximal femoral varus derotational osteotomy
imal femoral varus derotational osteotomy with blade plate fixation, with blade plate fixation, and periacetabular pelvic osteotomy (Dega)
286 U.G. Narayanan
Fig. 17.23 (a) Deformation of

the dislocated femoral head with
loss of sphericity and flattening
of superolateral surface. (b)
Femoral head resection,
subtrochanteric valgus osteotomy
of the proximal femur
the tri-radiate cartilage [92, 93]. Bone graft is derived from indications and effectiveness of this procedure remain uncer-
the proximal femoral varus osteotomy and the anterior iliac tain [86]. In non-ambulant children, hamstring lengthening
crest (Fig. 17.22b, c). can be performed percutaneously. Hamstring lengthening is
often performed in conjunction with hip reconstructive sur-
Femoral Head Resection and Related Procedures gery, to reduce the tension on the relocated hips and to
Salvage procedures are indicated for long-standing hip dislo- address knee flexion contractures that might be interfering
cations that are painful but not reconstructible because of the with positioning, dressing, or caregiving.
advanced changes associated with the long-standing disloca-
tion including loss of sphericity of the femoral head and Distal Femoral Extension Osteotomy
severe acetabular dysplasia. The femoral head typically In the presence of fixed knee flexion contractures (15° to
deforms along its exposed superolateral surface due to the 30°), a distal femoral extension osteotomy might be the only
pressure from the overlying abductors (Fig. 17.23a). In gen- way to achieve “full” extension of the knee acutely [97]. An
eral, salvage procedures are not as effective as reconstructive anteriorly based closing wedge osteotomy is performed
procedures to achieve the goals of pain relief. Proximal fem- through a lateral incision using a subvastus approach to the
oral head resection at the base of the neck, along with a sub- distal femur stabilized with a fixed-angle plate for internal
trochanteric valgus osteotomy of the femur, tenodesis of the fixation (Fig. 17.24a–e). The sciatic/peroneal nerves are at
ligamentum teres to the lesser trochanter, and capsular clo- risk of stretch neuropraxia with acute corrections (extension)
sure, in addition to the adductor and psoas lengthening is an of knee flexion contractures (especially with the hip flexed)
effective strategy to provide sufficient abduction for perineal intraoperatively or in the immediate postoperative period,
hygiene and pain relief from the previous dislocation but not which can result in dysesthesias in the lower extremity.
without a significant period of recovery [94] (Fig. 17.23b). Postoperative immobilization in some flexion is recom-
Heterotopic ossification or proximal migration is uncommon mended for a few days following surgery before the knee is
with this procedure but occurs more frequently with other stretched to full extension.
salvage procedures that involve more extensive proximal
femoral resections. Patellar Tendon Shortening or Advancement
This procedure is often done in combination with the distal
femoral extension osteotomy to re-tension the relatively
Orthopedic Procedures at the Knee and Lower lengthened quadriceps following the femoral osteotomy. The
Leg Segment (Tibia) patellar tendon realignment can be done by an infrapatellar
shortening procedure of the patella tendon [76]. Alternately,
Hamstring Lengthening patellar tendon advancement via a distal transfer of the tibial
In ambulant children, just the medial hamstrings are typi- tubercle in the skeletally mature child or the anterior portion
cally lengthened [86, 95]. Using a posteromedial approach of the tibial tubercle apophysis in a skeletally immature child
(patient supine) or posterior midline approach (patient may be performed [98].
prone), intramuscular tenotomies of the gracilis and semiten-
dinosus and fractional lengthening of semimembranosus Anterior Hemiepiphysiodesis of Distal Femoral
aponeurosis can be performed. Lateral hamstring (biceps Physis
femoris) lengthening is generally avoided in ambulatory Guided growth with the use of anterior staples or extraperi-
children due to the increased risk of knee hyperextension and osteal tension-band plates might gradually correct fixed flex-
weak hip extension [96]. Transfer of the tenotomized semi- ion deformities in children who have sufficient growth
tendinosus to the adductor tubercle might preserve hip exten- remaining. The long-term effectiveness of these procedures
sion and prevent increased anterior pelvic tilt, but the remains uncertain [76].
Fig. 17.24 (a–e) Distal femoral

extension osteotomy with blade
plate fixation
Rectus Femoris Transfer Distal Tibial (Supramalleolar) Derotational

Co-spasticity of the rectus femoris and the hamstrings is Osteotomies (Fig. 17.25)
associated with a stiff knee gait pattern, characterized by Excessive external or internal torsion of the tibia can result in
decreased knee flexion in early swing phase resulting in poor abnormal foot progression angle, associated with foot drag
foot clearance or foot drag [99]. The Duncan–Ely sign is and a poorer lever arm of the foot for push off. Torsional
positive on the physical examination. Gait kinematics show deformities are corrected through distal tibial derotational
decreased amplitude and delay of peak knee flexion in swing. osteotomies and stabilized with internal fixation (ideally
Dynamic EMG shows abnormal activation of the rectus fem- plates/screws) [103]. A concomitant osteotomy of the fibula
oris throughout swing. Rectus femoris transfer is indicated to is usually only necessary when derotation exceeds 30° [104].
improve knee flexion in early swing in order to improve foot No fixation is required for the fibular osteotomy.
clearance [100]. The rectus femoris tendon is separated from
the underlying vasti; the detached tendon is transferred
medially over the surface of the vastus medialis and teno- Orthopedic Procedures at the Ankle and Foot
desed to the semitendinosus, gracilis, or sartorius. Early
postoperative range of motion (immediate CPM) is impor- Gastrocnemius and Soleus Lengthening
tant to prevent adhesions. Postoperative rehabilitation includ- The soleus typically contributes less to equinus than the
ing muscle strengthening and retraining exercises is crucial gastrocnemius in children with bilateral CP, whereas in
for the success of this procedure. This procedure has been hemiplegics the soleus is just as involved in the contracture.
shown to be more effective in higher functioning ambulatory Based on the relative contributions of both muscles options
children who are motivated and capable of participating in a include selective lengthening of the gastrocnemius alone
rehabilitation program [99, 101, 102]. [105], with complete sparing of the underlying soleus;
288 U.G. Narayanan
Fig. 17.25 Distal tibial

derotational osteotomy
addition of the aponeurotic striping of the underlying dynamic and the hindfoot is passively correctable to normal,
soleus; combined gastrocsoleus lengthenings; and tendo- a split tibialis posterior tendon can be transferred to the pero-
Achilles lengthening. Tendo-Achilles lengthening in bilat- neus brevis [111]. Transfers are more likely to be effective
eral CP has been implicated in development of iatrogenic and last longer when done in younger children.
crouch, and should be avoided unless the contracture is
severe and involves both gastrocnemius and the soleus Split Tibialis Anterior Tendon Transfer
[106–109]. Surgery in children under 8 years is associated If the hindfoot varus deformity occurs primarily in swing
with increased risk of recurrence and overcorrection. The phase of gait and sets up the foot to land on the lateral border,
gastrocnemius lengthening is performed at the junction of a split tibialis anterior tendon transfer to the cuboid can be
the proximal and mid-calf, at the level of the inferior border very effective [112]. A tibialis posterior tendon lengthening
of the muscle bellies of the medial and lateral heads of the can be combined with a split tibialis anterior tendon transfer
gastrocnemius, where the aponeurosis of the gastrocnemius (SPLATT) and tendo-Achilles lengthening [113]. The goal is
is separable from the underlying soleus [105]. In older chil- to achieve a braceable, plantigrade foot in stance so that
dren with long-standing equinus, there may be additional weight bearing on the plantar surface rather than along the
though milder contracture of the soleus. In these children, it lateral border and in swing phase prevents difficulties with
is possible to obtain additional dorsiflexion by dividing the foot clearance.
aponeurosis of the soleus immediately deep to the divided
gastrocnemius while preserving the underlying muscle Foot Osteotomies
fibers of the soleus [76]. In hemiplegics, both gastrocne- Equinoplanovalgus deformities are more common in bilat-
mius and soleus are usually equally tight and therefore a eral CP. A lateral based opening calcaneal osteotomy just
tendo-Achilles lengthening can be performed by a percuta- proximal to the anterior process will lengthen the lateral col-
neous or mini-open sliding technique (Hoke or White) or umn, correcting the hindfoot valgus and forefoot abductus
open z-lengthening [110]. Recurrence of equinus is the and restoring the medial arch. Bone graft (iliac crest or
most common complication of a tendo-Achilles lengthen- allograft) is used to fill the gap at the osteotomy site. Peroneal
ing in a hemiplegic, and is usually a concern when done in brevis tendon lengthening is usually necessary as it is ten-
children less than 8 years old. sioned when the foot is swung out of abduction. Gastrocsoleus
recessions might be needed to address any accompanying
Tibialis Posterior Tendon Lengthening or Split equinus, which will be unmasked when the valgus is cor-
Tibialis Posterior Tendon Transfer (SPOTT) rected [114]. To minimize the chance for recurrent defor-
Equinovarus deformities are more common in unilateral CP mity, medial procedures are often added, such as capsulodesis
or hemiplegia. Fixed contractures need lengthening of the of the talonavicular joint and advancement of the tibialis pos-
tibialis posterior tendon. An intramuscular tenotomy of the terior tendon. Plantar flexion osteotomy of the first ray to
tibialis posterior at the distal calf is effective and distal restore the normal tripod of the weight-bearing plantigrade
z-lengthenings are seldom required. When the contracture is foot might be necessary if following the correction of the
hindfoot valgus, and mid/forefoot supination is apparent. An peripheral consequences of CP while the primary pathology
alternative to the lateral column lengthening strategy is the in the brain remains unaddressed. There is an imperative for
“triple C” method, which includes (1) a closing wedge varus stronger evidence for the effectiveness of many of these
producing osteotomy of the calcaneus along with some interventions based on outcomes that are meaningful to this
medial slide all done from the lateral approach, and (2) an population [54, 117–119]. The longevity of these outcomes
opening wedge osteotomy of the cuboid using a wedge of up to and beyond skeletal maturity into adulthood needs to
autologous bone graft obtained from (3) a plantar-based be established [81]. Improved understanding of the biology
closing wedge osteotomy of the medial cuneiform [115]. of brain development and repair and epidemiological
More severe or rigid varus or valgus deformities of the hind- research might provide the means to prevent or mitigate
foot are best managed by calcaneal osteotomies using clos- many of the etiological causes of CP. Until that time orthope-
ing wedge or sliding osteotomies. Once the hindfoot dic surgery will continue to play an important role in the
deformity is corrected, a subtalar (extra-articular) arthrodesis management of the deformities associated with CP.
can be performed to correct and hold the deformity perma-
nently [116]. Others recommend a talonavicular arthrodesis
to accomplish the same objective. Myelomeningocele (Spina Bifida)
In the presence of a plantarflexed first ray, plantar fascia
release and extension osteotomy of first metatarsal or medial Defective closure of the neural tube in the first month of
cuneiform will address a midfoot cavus and secondarily embryonic life leads to a spectrum of neural tube defects,
eliminate the compensatory hindfoot varus. The peroneus which affects about 0.5–1.0/1,000 pregnancies in the USA
longus tendon is tenodesed to the peroneus brevis to prevent [120]. The overall incidence has declined with oral folic acid
further plantar flexion of the first metatarsal. Hallux valgus supplementation and termination of pregnancy that is facili-
deformities are only addressed if painful or are interfering tated by maternal prenatal screening with ultrasound and
with shoe or brace wear. Correction of the deformity should elevated levels of α-fetoprotein and β-human chorionic
be accompanied by an arthrodesis of the first metatarsopha- gonadotrophin levels. The neurologic deficit has both motor
langeal joint. and sensory components and the specific musculoskeletal
deformities of the lower extremity depend on the level of the
lesion and the resulting imbalance between the effects of
Summary neurologic deficit present at the level (mixed upper and
lower motor neuron) and below the level (lower motor neu-
The lower extremity musculoskeletal consequences of CP ron) of the lesion and the relatively unopposed action of the
are significant both in ambulant and non-ambulant children. uninvolved neurologic levels above the lesion (Table 17.2).
The goals for these two groups are quite different (see Boxes Unlike in cerebral palsy, where the deformities are absent at
17.4 and 17.5). These goals are best achieved using a multi- birth, many of the musculoskeletal deformities associated
disciplinary approach, of which orthopedic surgery plays an with spina bifida might be present at birth. However, similar
important role. However, orthopedic surgery deals with the to CP, such deformities usually become worse with growth.
Table 17.2 Musculoskeletal deformities by level of lesion in myelomeningocelea

Level Motor examination Orthopedic manifestations Functional manifestations
Thoracic • Flaccid lower extremities • Scoliosis (neuromuscular, congenital) • Household ambulation with
• Kyphosis HKAFO bracing when small
• Possible clubfeet, hip dislocations • Wheelchair when bigger
Upper • Hip flexion/adduction partially intact • Hip dislocation from unbalanced • Ambulation with HKAFO
lumbar • Knee flexion/extension, ankle power, hip muscles common bracing when small
extension/abduction absent • Clubfoot deformities • Often wheelchair when bigger
Lower • Knee extension intact (L4—quadriceps) • Clubfeet • Ambulation with KAFO
lumbar • Ankle dorsiflexion possible (L5—tibialis anterior) • Calcaneus (L5) bracing
• Hip dislocation or subluxation sometimes
Sacral • Hip flexion/adduction, knee flexion/extension, • Cavovarus foot deformity • Very good ambulation with
ankle power present • Hip dislocation much less common AFO bracing
• Hip extension/abduction partially intact • Ulcers may lead to osteomyelitis
• Gastroc and foot weakness
a
Reprinted with permission from Narayanan UG, Caird MS: Neuromuscular disorders in children, in Canada LK (ed): Orthopedic Knowledge
Update 11. Rosemont, IL: American Academy of Orthopedic Surgeons, 2014, pp. 933–946
290 U.G. Narayanan
Hip Dysplasia equinovarus deformities are stiffer than idiopathic clubfeet,

but can still be successfully managed with serial casting
Hip dysplasia is most common with higher level lesions (tho- using the Ponseti method in some cases, with careful atten-
racic and high lumbar) and is often present at birth and gen- tion paid to the health of the skin during casting. A well-
erally more resistant to reduction than idiopathic balanced but floppy foot does not need surgery but can be
developmental hip dysplasia. The management of hip dys- successfully managed with a brace as can a passively cor-
plasia in this patient population is controversial. rectable deformity. Surgery is indicated for persistent or
Reconstructive approaches to relocate and stabilize the hips recurrent deformity in children who can walk. In general,
are unnecessary for the non-ambulant children as pain is proximal deformities should be corrected before distal ones
generally not a feature of hip dislocation in spina bifida. In and soft-tissue releases are done before corrective osteoto-
ambulant children, gait function may be more affected by the mies. Equinus is often correctable with a tendo-Achilles
presence of contractures than the instability of dislocated tenotomy. Severe equinovarus deformities often require
hip. Furthermore, complications associated with surgery extensive posteromedial releases, which might be facilitated
(decrease in strength and increased fracture risk) may out- by excision of the talus. There is little role for tendon trans-
weigh the benefits of reduction [121]. fers in this patient population. Recurrent deformities might
benefit from talectomy or triple arthrodesis. Cavovarus
deformities can be managed by plantar fascia releases, calca-
Knee Flexion Deformity and Tibial Torsion neal osteotomy, and osteotomy of the bases of the metatar-
sals. Ankle valgus can be managed by supramalleolar
Functional walking is reliant on the presence of some quad- osteotomy or distal medial tibial hemiepiphysiodesis with a
riceps action (present in lower lumbar level lesions or more screw [122, 123].
distal neurologic level). In the presence of knee flexion
deformity, the excessive external flexion moment at the knee
cannot be adequately counteracted by an extension moment Poliomyelitis
generated by the weak knee extensors. The use of knee-
ankle-foot orthoses (KAFO) might facilitate some walking Poliomyelitis is an acute enteroviral infection affecting the
in such children. Fixed flexion deformities are amenable to anterior horn cells of the spinal cord and the brain stem
radical posterior capsular releases which might provide some motor nuclei. The musculoskeletal deformities associated
functional benefits in ambulatory children with low lumbar with polio provided the mainstay of clinical practice of pedi-
or sacral lesions who are in crouch [122–124]. There may atric orthopedic surgeons in the last century. With the advent
also be a role for addressing knee flexion contractures in of widespread vaccination since the 1950s, polio is on the
nonambulatory patients with higher level lesions, to facili- brink of eradication, with a few remaining endemic areas in
tate standing. Marked external tibial torsion may contribute South Asia, Nigeria, and Afghanistan, where the penetration
to gait-related difficulties in ambulatory children who can of vaccination has been suboptimal.
benefit from derotational tibial osteotomies. This pure motor condition manifests in three stages. The
acute stage is characterized by initial gastrointestinal symp-
toms and fever, followed by meningeal symptoms and an
Ankle and Foot Deformities asymmetric paralysis within 48 h, which affects the lower
extremity more commonly than the upper extremity, particu-
A variety of specific foot deformities are associated with larly the glutei, hip flexors, quadriceps, tibialis anterior, and
neurologic level of the neural tube defect. Some deformities medial hamstrings. During the acute phase, management
are present at birth (e.g., equinovarus or clubfeet) while oth- involves bed rest, positioning, and range of motion to avoid
ers are acquired over time. Common deformities include contractures. During the convalescent stage muscle strength
equinovarus (clubfoot) most common with the higher level improves, much of it in the first month, but this can last for
lesions; cavovarus is often seen with L-4 lesions; and calca- up to 2 years. During this time, treatment includes range of
neus and calcaneovalgus are seen with L-5 lesions. Pure motion, splinting and bracing (orthoses) to prevent fixed
equinus might arise from isolated reflex segments of the cord contractures, and strengthening of the affected muscle
causing spasticity. A late-onset cavus deformity that is pro- groups. However, overactivity of muscles in the convalescent
gressive with growth might be associated with tethered cord. phase has the potential to inhibit recovery of function. The
Goals of management in children with myelomeningo- chronic stage begins 2 years from the onset of the disease,
cele are to maintain a stable, plantigrade foot for weight and no further muscle recovery is expected. The paralysis is
bearing while minimizing the risk of ulceration of insensate associated with flaccid joints or active joints with muscle
areas of the weight-bearing areas of the foot. Neurogenic imbalance. Flaccid joints will develop contractures only if
Table 17.3 Common deformities of the lower extremity in poliomyelitis, and their surgical management
Deformity Surgical management
Quadriceps femoris paralysis Biceps femoris and semitendinosus transfer to patella
Flexion contracture of the knee Soft-tissue release or femoral osteotomy
Genu recurvatum Tibial osteotomy
Soft-tissue triple tenodesis
Flail knee Orthosis with drop-lock knee and arthrodesis
Equinus Achilles tenotomy
Cavus Plantar fascia release
Calcaneal osteotomy
Base of metatarsal osteotomies
Ankle valgus Medial distal tibial hemiepiphysiodesis
Supramalleolar osteotomy
Hindfoot (subtalar) valgus Calcaneal osteotomy (medial slide)
Extra-articular arthrodesis of the subtalar joint (Grice)
Triple arthrodesis
Convex pes valgus (rocker bottom foot) Open reduction with transfer of tibialis anterior and peroneus brevis and lengthening of the tendo-Achilles
left immobile in abnormal positions for long periods of time.

These joints are best treated with stretching exercises and Hereditary Motor Sensory Neuropathy
orthoses to maintain the joint in neutral position. Active
joints, on the other hand, are prone to develop fixed contrac- These are a group of hereditary neuropathies with sensory
tures over time and secondary bony deformities. These are and motor involvement of the peripheral nerves. These can
treated by dynamic braces to counteract the unopposed mus- usually be diagnosed by one or more of the following inves-
cle actions. Surgical treatment includes muscle-balancing tigations: blood tests for genetic testing, nerve conduction
procedures, such as tendon lengthening and transfers [125], studies, and nerve biopsies. The most common such neurop-
and corrective osteotomies to address fixed deformities and athy is hereditary sensory motor neuropathy (HSMN) type 1
to stabilize joints (Table 17.3). These are ideally done when or Charcot-Marie-Tooth (CMT) disease type 1. CMT type
the child is older, so that optimal growth potential has been 1A is autosomal dominant and associated with demyelin-
achieved [125–127]. ation of the peripheral nerves due to a duplication of periph-
eral myelin protein (PMP22) that resides on chromosome
Box 17.6. Principles of Tendon Transfer 17. The typical clinical presentation is usually not obvious
1. The joint of interest must be free of fixed defor- until the second decade characterized by progressive atro-
mity, with an acceptable range of passive motion. phy, muscle weakness, loss of balance, and contractures
2. Any deformity will require correction prior to involving the intrinsic muscles of the foot which are the first
transfer. nerves to become involved. Sensory loss occurs first in the
3. Muscle strength of tendon to be transferred must distal legs followed by the distal upper extremity. Sensory
be at least grade 4, as the transfer is associated and motor involvement gradually worsens. Nerve conduc-
with a decrease in strength by one grade. tion velocities are slowed in children even before the clinical
4. The line of excursion and strength of transferred onset of disease. Motor amplitudes decrease gradually.
muscle should be similar to the one being replaced. Nerve biopsy typically demonstrates age-related loss of
Linear line of action is preferable. myelinated axons and disability correlates with axonal loss
5. Synergistic muscle (in-phase transfer) is preferred [128]. There are numerous additional subtypes of CMT 1
over an antagonistic muscle (out-of-phase transfer). caused by different mutations resulting in demyelination of
6. Paratenon of the transferred tendon should be the peripheral nerves. In contrast, HSMN type 2 or CMT
preserved. type 2 (CMT2) is an axonal (non-demyelinating) peripheral
7. Neurovascular supply to the transferred muscle neuropathy which is characterized by distal muscle weak-
should be persevered. ness and atrophy, and mild sensory loss, clinically similar to
8. Preferable to run in one muscle compartment with CMT1 but less severe, and nerve conduction velocities are
a smooth gliding channel. typically normal. Peripheral nerves are not enlarged or
9. Transfer is applied under adequate tension. hypertrophic. There are many subtypes of CMT2 (15 genetic
10. Firm fixation to bone is preferable to tendon. mutations) which are clinically indistinguishable and differ-
entiated only by genetic testing [129].
292 U.G. Narayanan
Fig. 17.26 Cavovarus foot

deformity in Charcot-Marie-
Tooth disease
Foot Deformities (Fig. 17.26)
The earliest and most common deformities involve the intrin-

sic muscles of the foot leading to clawed toes. This is fol-
lowed by weakness of the peroneus brevis and tibialis
anterior, while the peroneus longus and tibialis posterior are
spared. This pattern of weakness results in cavus and hind-
foot varus, associated with weight bearing on the lateral bor-
der of the foot resulting in callosities and difficulties with
shoe wear. A high stepping gait facilitates clearance of a drop
foot. Examination reveals mild sensory disturbances and
absent reflexes [130].
Treatment is initially symptomatic and supportive, and
custom orthotics or AFOs can play a useful role in the man-
agement of flexible deformities. Surgery is indicated when
orthotics fail to manage symptoms. For cavus deformities Fig. 17.27 Older onset hip dysplasia in Charcot-Marie-Tooth disease
that are not fixed, an open plantar fascia release is done, but
usually in combination with an extension osteotomy of the
first metatarsal (or medial cuneiform) to address the
plantarflexed deformity of the first ray. Flexibility of the Hip Deformities
hindfoot varus is preoperatively assessed using the Coleman
block test and reveals the plantar flexion of the first ray that The onset of hip dysplasia occurs relatively late in HSMN,
is the driving force of the hindfoot varus, obviating the need most commonly seen in patients affected with CMT1, typi-
for a calcaneal osteotomy. A rigid cavus associated with fore- cally presenting first in adolescence [132, 133]. Since the
foot equinus might require extension osteotomies of addi- development of dysplasia is insidious and asymptomatic,
tional metatarsals. More severe cavus might require a annual radiographs are recommended to monitor the hips
midfoot or tarsal osteotomy. Rigid hindfoot varus may need once a diagnosis of CMT1 has been established. Radiographs
a calcaneal osteotomy (lateral based closing wedge). Claw usually reveal a dysplastic acetabulum characterized by
toe deformities can be addressed with transfers of the exten- smaller lateral and anterior center-edge angles; decreased
sor hallucis longus to the neck of the first metatarsal and the femoral head coverage; increased acetabular anteversion;
common extensor tendon to the lateral cuneiform along with and abnormal proximal femur characterized by increased
flexor tenotomies or transfer of the toe flexors to the extensor coxa valga [134] (Fig. 17.27). Pelvic osteotomies to correct
hood. Triple arthrodesis is ideally avoided because of longer the acetabular dysplasia and proximal femoral osteotomies
term risk of arthritis in the ankle but may be necessary for are indicated when hips become symptomatic or the acetabu-
severe fixed deformities [131]. lar dysplasia becomes progressive. Stable internal fixation is
recommended to avoid prolonged immobilization which can in management of these conditions, most effectively in the
cause profound weakness. Postoperative nerve palsy is context of a multidisciplinary approach along with physical
reported more commonly in HSMN [132]. therapists, developmental pediatricians, physiatrists, neurol-
ogists, orthotists, and other specialists. Although our inter-
ventions have been shown to make substantial differences,
Muscular Dystrophies much work remains to be done to define the effectiveness
and longevity of the outcomes, in terms that are relevant and
These are a group of more than 30 genetic diseases character- meaningful to these populations. These conditions are
ized by progressive skeletal muscle weakness. Duchenne chronic and permanent and currently have no cures. Future
muscular dystrophy (DMD), the most common form, is an advances will stem from basic research to understand the
X-linked recessive condition due to an absence of dystro- embryology and biology of the neural axis and the musculo-
phin, a protein involved in the stabilization and integrity of skeletal system, and the pathophysiology of these heteroge-
the cell membrane. Cell membrane becomes permeable to neous disorders; and epidemiologic research that will unravel
creatine kinase (CK), which is elevated in the blood, the risk factors will eventually provide the strategies to miti-
and muscle fibers degenerate and are replaced with fibrofatty gate or reverse the effects of these conditions, or prevent
tissue. Becker muscular dystrophy is associated with faulty or them from occurring in the first place.
decreased dystrophin and is similar to DMD but much less
severe. CK levels are elevated but are not as high as in DMD.
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Arthrogryposis
18
Reggie Hamdy and Noémi Dahan-Oliel
Arthrogryposis is a group of disorders characterized by

nonprogressive, multiple joint contractures and muscle Etiology
weakness, present at birth. The word “arthrogryposis” is
derived from the Greek language (arthro = joint and gryp = The main cause of arthrogryposis is diminished fetal
curved), referring to the multiple congenital contractures movements, resulting from either fetal or maternal abnor-
inherent to this category of disorders [1]. Most individuals malities. The decreased fetal movements ultimately lead to
with arthrogryposis have normal or above normal intelli- the development of multiple joint contractures, as connective
gence and normal sensation. Arthrogryposis can be seen in tissue around the joint limits joint mobility, leading to con-
isolation or in association with other congenital abnormali- tractures [1, 6]. During pregnancy, a limb will not achieve
ties as part of a syndrome with or without visceral and/or optimal growth when in utero movement is restricted for
central nervous system involvement [2]. There are over 300 several months [1]. There are numerous causes of dimin-
different syndromes associated with congenital contrac- ished fetal movements, including neuropathic, muscle or
tures [3], the most common being amyoplasia affecting all connective tissue abnormalities, space limitations of the
four limbs [4]. Contractures typically affect two or more fetus, intrauterine vascular compromise, and maternal dis-
areas of the body and limit children’s mobility, self-care, and eases [1, 6]. Prenatal diagnosis may be possible by using
daily activities. repeated ultrasound studies to evaluate fetal movements and
characteristic fetal positions.
Prevalence
Classification
The overall prevalence of arthrogryposis has been estimated
at 1 in every 3,000 live births [5]. Recently, the prevalence of Two main classification systems for arthrogryposis exist.
arthrogryposis was reported to range from 1 in 2,000 in The first classification system by Dr. Judith G. Hall was first
Leicester, UK [6], to 1 in 5,100 in Alberta, Canada [7]. published in 1997 and classifies arthrogryposis into three
main groups, according to the presence or absence of associ-
ated visceral and central nervous systems [2] (Table 18.1).
A more recent classification system by Dr. Bamshad and col-
leagues [4] divides arthrogryposis into three main entities,
R. Hamdy, MD (*) including amyoplasia, distal arthrogryposis, and syndromic
Division of Pediatric Orthopedics, Department of Orthopedics, (Fig. 18.1). The main difference among those two classifica-
Shriners Hospital for Children, McGill University, tion systems is the inclusion of different types of distal
1529 Cedar Avenue, Montreal, QC, Canada H3G 1A6 arthrogryposes together, as in the newer classification, or as
separate entities depending on involvement of body areas, as
N. Dahan-Oliel, PhD, OT in the Hall classification. In this chapter, the different disor-
Clinical Research/Rehabilitation, Shriners Hospital for Children,
1529 Cedar Avenue, Montreal, QC, Canada H3G 1A6 ders encompassed by the umbrella term arthrogryposis are
e-mail: [email protected] presented using Hall’s classification [2].

298 R. Hamdy and N. Dahan-Oliel
Group 1: Disorders Affecting Mainly of joint deformity is highly variable. The other types of distal
the Four Limbs arthrogryposes involve other body parts (e.g., eyes, mouth),
and hence are presented as part of group 2 arthrogryposes.
The most common disorder of this group is amyoplasia There are other types of arthrogryposis involving mainly
(a = no, myo = muscle, plasia = growth) meaning no muscle the limbs and which are included in group 1, such as contrac-
growth. It is the most common form of arthrogryposis, and tural arachnodactyly and symphalangism [1].
represents one third of all cases. Most affected individuals Both amyoplasia and distal arthrogryposis type I have
have all four limbs involved. It has not been observed to been found to respond well to physical therapy [10, 11] and
recur in siblings [1]. these disorders are usually seen by orthopedic surgeons.
Distal arthrogryposes (DA), as the name indicates, involve
the distal joints, and do not involve a primary neurological
and/or muscle disease. Distal arthrogryposes share a pattern Group 2: Disorders Affecting the Limbs
of hand and foot involvement, limited involvement of with Involvement of Other Parts of the Body
proximal joints, and variable expressivity [4]. These disor-
ders are inherited as autosomal dominant traits. Currently, Distal arthrogryposes (DA) other than type 1 are included in
distal arthrogryposes are subdivided into 10 types, depend- this group as in addition to contractures of the hands and
ing on the number and nature of additional features [4]. Of feet, they involve other body parts. Type 2 can be further
these, type 5 can be further divided into several subtypes separated into two subtypes, Freeman-Sheldon syndrome
based on additional phenotypic features [8, 9]. Type I (DA1) (FSS or DA2A) and Sheldon-Hall syndrome (SHS or DA2B)
is characterized by a typical positioning of the hands with [4]. The defining characteristic in FSS is that oropharyngeal
medially overlapping fingers, clenched fists, ulnar deviation abnormalities, scoliosis, and a very small oral orifice, puck-
of the fingers and camptodactyly, and clubfoot. The degree ered lips, and dimple in the chin, hence the name “whistling-
face syndrome” (Fig. 18.2). SHS has similar congenital
Table 18.1 Hall classification of arthrogryposis contractures present in DA1, with the addition of more
prominent nasolabial folds, palpebral fissures, and a small
Group Type of involvement
mouth [4]. DA3 and DA4 are very rare. DA5 is unique in that
Group 1 Mainly limb involvement (four limbs, lower
limbs or upper limbs) ocular abnormalities are present in addition to muscle con-
Group 2 Limbs are affected with involvement of other tractures. Other types of DA are very rare and have distin-
parts of the body guishing features, such as very short stature and cleft palate
Group 3 Limbs are affected with central nervous system in DA6, and inability to fully open the mouth and pseudo-
involvement camptodactyly in DA7 [4].
Fig. 18.1 Types of congenital

contractures [4]. Reproduced
with permission from Bamshad
M, Van Heest AE, Pleasure
D. Arthrogryposis: a review and
update. J Bone Joint Surg Am.
2009 Jul;91 Suppl 4:40–6
18 Arthrogryposis 299
In addition to DA, there are many different syndromes the most common of the multiple pterygium syndromes, but
involving muscle contractures and other body areas [2], such is very rare and affects 1 in 300,000 live births. It is inherited
as multiple pterygium syndromes, diastrophic dysplasia, and as autosomal dominant with a wide pattern of expression.
Larsen syndrome. Multiple pterygium syndromes involve a Key features include genitourinary, craniofacial, and extrem-
web or triangular membrane that forms across a joint [2]. ity malformations in association with popliteal webs that
Different pterygium syndromes have different forms of vary greatly in severity. The fixed knee and ankle deformities
inheritance and features. Popliteal pterygium syndrome is render ambulation difficult (Fig. 18.3). Diastrophic dysplasia
involves contractures of the shoulders, elbows, interphalan-
geal joints, and hips [1, 2]. This type of dwarfism has an
autosomal recessive inheritance. Other features include cys-
tic masses involving the external ear and cleft palate [1].
Mortality rate is increased during infancy, but life expec-
tancy is normal past infancy. Larsen syndrome involves a
distinctive facial appearance with flattening of the face,
prominent forehead, depressed nasal bridge, widely spaced
eyes, and ligamentous hyperlaxity, as well as flexed hips and
hyperextended knees [1]. Often, there are multiple joint dis-
locations (hips, knees, shoulder, and elbows), hand deformi-
ties with long cylindrical fingers, feet deformities in the form
of equinovarus or equinovalgus, and a high incidence of
spine anomalies, specifically cervical kyphosis caused by
marked hypoplasia of one or two vertebral bodies. Congenital
cardiac and respiratory anomalies may also be present. The
management of the orthopedic problems is usually surgical.
Group 3: Disorders Affecting the Limbs

with Involvement of the Central Nervous
System
Numerous syndromes with involvement of the central ner-

Fig. 18.2 Photograph of a toddler with Freeman-Sheldon syndrome vous system can have associated congenital contractures [2].
showing typical facies. Reproduced with permission from Bamshad M,
Van Heest AE, Pleasure D. Arthrogryposis: a review and update. J Bone These may be caused by developmental abnormalities of the
Joint Surg Am. 2009 Jul;91 Suppl 4:40–6 forebrain in utero, chromosomal deletions or rearrangements,
Fig. 18.3 (a, b) Eleven-year-old

boy with popliteal pterygium
syndrome
or genetic mutations. These disorders are the most common Genetic investigations should include both blood cells and
cause of severe arthrogryposis [4]. Nerve conduction studies skin fibroblasts as, in some cases, mosaicism may exist
and EMG may be indicated in the case of neuromuscular dis- (when chromosomal studies on blood cells are normal but an
orders associated with arthrogryposis [4]. abnormality is detected in the skin fibroblasts). The most
common form of arthrogryposis, amyoplasia, is not a heredi-
tary condition and no specific genetic diagnosis can be made
Genetic Aspects of Arthrogryposis in this condition.
The focus of this chapter is on lower limb deformities as
Multiple congenital contractures are present in a number of it pertains to amyoplasia, as it is the most common form of
genetic syndromes. Many specific types of arthrogryposis arthrogryposis.
have been mapped to loci in human chromosomes [1, 2]. The
disorder may be caused by a single gene, in which case the
disorder can be autosomal dominant, autosomal recessive, or Clinical Picture
X-linked. Rarely, disorders have a much higher risk of recur-
rence, such as mitochondrial inheritance. The presence of A child with amyoplasia presents with a typical clinical pic-
chromosomal abnormalities is prominent in cases of multi- ture. In the upper limbs, the shoulders are internally rotated
ple congenital contractures with intellectual disability. and adducted; the elbows are extended, the forearms are pro-
Recent studies have identified several genetic mutations nated, the wrist and fingers are flexed (Fig. 18.4). This pat-
responsible for different forms of arthrogryposis [12–15]. tern of upper extremity involvement is often described as a
Some types of arthrogryposis are sporadic, such as amyopla- policeman’s hand or a waiter’s tip hand (Fig. 18.5). In the
sia, in which genetics does not play a role in its inheritance. lower limbs, the foot is most commonly affected (about 90 %
Prenatal testing and genetic counseling are indicated for par- of cases), followed by knee deformities (about 70 % of cases)
ents with a higher risk of having another affected child. and then hip deformities in about 40 % of patients. Flexion,
abduction, and external rotation contractures of the hips are
typically observed (Fig. 18.6) (Box 18.1). Often, there is loss
Intellectual Skills
Intelligence is normal in many forms of arthrogryposis,

including amyoplasia, distal arthrogryposis type I, dia-
strophic dysplasia, and Larsen syndrome. Patients with cer-
tain forms of arthrogryposis associated with chromosomal
abnormalities or with CNS involvement may have decreased
cognitive function [1].
Workup of a Child with Arthrogryposis
Any infant or child seen with multiple contractures should

have a thorough clinical evaluation including a comprehen-
sive family history, physical examination, and if necessary,
further workup. In the physical examination, it should be
determined first if arthrogryposis involves only the limbs or
if it involves also other parts of the body, specifically the
central nervous system. Second, it is important to determine
which part of the limbs is involved, and to what extent. The
range of motion of each joint should be carefully recorded.
Radiographs of the spine, hips, and feet should be obtained.
Bone mineral density appears to be lower in children with
arthrogryposis compared to age-matched peers [16], and
should be investigated.
It is essential to determine if this condition is genetic or Fig. 18.4 Photograph of a 5-month-old baby with arthrogryposis
nongenetic, not only for management purposes but also for showing bilateral clubfeet, extension contractures of the knees, and
counseling the parents on the risk of future pregnancies. upper limb involvement with extended elbows and flexed wrists
Box 18.1. Diagnostic Approach for the Lower Limbs in a

Child with Amyoplasia
• The deformities are present at birth.
• All four limbs are usually affected.
• Sensation is normal.
• Muscular atrophy and weakness is present.
• Normal intelligence.
• The feet are most commonly affected.
• Knee flexion contractures are more common than
extension contractures.
• Hip flexion, external rotation, and abduction con-
tractures of the hip, hip subluxation, or dislocation
may be present.
of skin creases across the joints and dimpling at the sites of

the joints. The muscles are severely atrophied. Children with
amyoplasia have normal IQ and normal sensation [17].
Prognosis
Fig. 18.5 Photograph of a 5-year-old child with arthrogryposis show- The functional long-term prognosis is usually very good for
ing typical upper and lower limb deformities, including policeman tip most patients with amyoplasia, unlike other neuromuscular
position with flexed wrist and extended elbows in the upper limb, club-
feet and knee flexion contractures in the lower limb conditions. The contractures are usually most severe at birth
and then gradually improve with life. The overall good prog-
nosis should be clearly explained to the parents. Long-term
ambulatory status and functional outcome at skeletal matu-
rity are not necessarily correlated with the severity of arthro-
gryposis at birth, warranting early intensive treatment and
rehabilitation in these children [18, 19].
General Management
Early intervention, in the form of aggressive physiotherapy

including stretching, joint mobilization, and range of motion
exercises, as soon as possible after birth, can facilitate opti-
mal long-term functional outcome [4, 17–19]. Surgical
interventions may also be indicated during the first year of
life, as outlined below. Regular and frequent follow-up
(every few weeks) is recommended during the first year of
Fig. 18.6 Photograph of a 2-year-old child showing typical flexion life to monitor progression and plan surgical interventions.
abduction and external rotation contractures of the hips This is followed by regular visits, typically every 6 months.
Children with arthrogryposis should be followed in a multi- may facilitate the manipulations and may yield good results
disciplinary clinic. The overall goal in the management of with the Ponseti technique [24]. We recommend this
these children is to improve function and ambulation while approach as the initial treatment. In cases of failure of the
fostering optimal development. It is often recommended that Ponseti technique, a formal posteromedial release is recom-
the foot deformities be treated first, followed by the knee and mended [25] even though the recurrence rate is high
finally the hip deformities [17]. (Fig. 18.8). In cases where a posteromedial release is not suf-
ficient to obtain a plantigrade foot, then a talectomy is advis-
Lower Limb able [22, 23]. In cases of recurrent deformities, repeat soft
Involvement of the lower limb is common in arthrogryposis tissue releases could be dangerous as the anatomy is com-
[11], and presents a challenging problem to the pediatric pletely distorted and neurovascular damage may ensue.
orthopedist. Lack of joint motion may be due to inadequate Consideration should be given to correction through multiple
muscle development, lack of normal and mobile skin and tis- osteotomies. The use of gradual correction with external
sue, and contractures with fibrous connective tissue. These
issues must be considered when devising the treatment plan,
and include the affected joints (e.g., foot, knee, and hip)
(Box 18.2). Range of motion of the knee and hip may be
improved with treatment, thereby facilitating ambulation [20].
Foot
Clubfoot deformity or talipes equinovarus is the most com-
mon foot deformity in arthrogryposis and is present in over
90 % of infants with amyoplasia [21]. Talipes equinovarus is
much more common and is more rigid than the standard idio-
pathic clubfoot. It may be associated with knee contractures
and hip contractures, subluxation, or dislocation (Fig. 18.7).
The goal in the management of feet deformities is to
obtain a painfree, plantigrade foot. Treatment should start
immediately after birth with the Ponseti technique. Previous
studies have reported that clubfeet in children with arthro-
gryposis are usually stiff and resistant to standard treatment
and may require talectomy as a first-line treatment [22, 23].
However, more recently, it has been shown that tenotomy of
the tendo Achilles before the start of the Ponseti technique
Fig. 18.8 (a, b) Radiographs of a 4-year-old child with arthrogryposis

Fig. 18.7 Photograph of a 3-week-old infant with arthrogryposis showing left clubfoot. (c) Postoperative radiograph following radical
showing right knee extension contracture, left knee subluxation, and posteromedial release of the left foot after a failed Ponseti technique.
bilateral clubfeet (d) Three-month postoperative radiograph showing adequate correction
Fig. 18.9 Photographs (a, b) showing severe deformities of the right foot in a 3-year-old girl. The right foot is severely supinated at age 9 (c, d).
Treatment of deformity with Ilizarov external frame (e, f). (g, h) Satisfactory alignment of the right foot post-Ilizarov removal at age 11
fixators is another viable option (Fig. 18.9). A standard followed by prolonged splinting to prevent or minimize
Ilizarov circular fixator could be used [26] or a Taylor Spatial recurrence of deformities [24, 27]. It should be clearly
Frame with the foot program [27]. In patients near or at skel- explained to the family that while such surgical treatment
etal maturity presenting with residual or recurrent foot defor- will eventually correct the deformity, the foot remains stiff,
mities, triple arthrodesis may be the best option [28]. with a high likelihood of needing subsequent surgeries as the
Conservative treatment in the form of the Ponseti tech- recurrence rate is very high [22, 29].
nique, as described above, should be considered first. In case Congenital vertical talus is reported to occur in approxi-
of recurrence or substantial residual deformity, a radical pos- mately 2–12 % of patients with arthrogryposis [30]. Because
teromedial release may be necessary around the age of 1 year many of these patients have the potential to ambulate, the
congenital vertical talus should be treated early, before walk- Knee

ing age. Immediately after birth, the modified Ponseti tech- The knee is the second most commonly affected lower
nique should be initiated as described by Alaee, Boehm, and extremity joint in amyoplasia (38–90 % of patients) [33].
Dobbs [31]. This may be successful in the correction of the Knee contractures can be the most disabling deformity as they
deformity. However, if the congenital vertical talus is rigid, prevent motion and anatomical limb alignment [34]. Among
then more aggressive treatment in the form of a one-stage knee deformities, flexion contractures are the most common
release is usually recommended. A tibialis anterior transfer in arthrogryposis [35, 36] and are also more disabling than
to the talar neck with or without Grice subtalar fusion is also extension contractures [35]. The goal in the management of
recommended [32]. In very stiff feet and recurrent deformi- flexion deformities is to facilitate ambulation, sitting, and
ties, surgical options include excision of the navicular, talec- standing (Table 18.2). Knee deformities may be more difficult
tomy, multiple osteotomies, and gradual correction with an to treat than other deformities and, more importantly, present
external fixator (Fig. 18.10). If the patient is near skeletal some of the most challenging problems causing gait distur-
maturity and has severe deformities, then a triple arthrodesis bances and may render ambulation very difficult [33, 34].
may be considered [32]. Contrary to extension deformities that may not interfere with
walking, flexion contractures of more than 20–30° usually
interfere with ambulation and should be treated. In fact, the
presence of knee flexion contractures is the single most
important indicator of the potential for ambulation. We and
others have shown that correction of knee flexion deformities
has a direct positive impact on the ambulation potential of
these children [19, 36]. In our own series of patients where
knee flexion contractures were treated with supracondylar
osteotomies (Fig. 18.11) or with gradual correction using an
Ilizarov circular frame, we reported that ambulation gains
were maintained in most patients despite some loss in correc-
tion with time in many patients [19]. However, quadriceps
strength should also be taken into account in the management
of knee deformities as quadriceps weakness (noted in about
60 % of patients) [37] may preclude any gains in ambulation
despite correction of knee flexion deformity [33].
The fact that there are several options for the treatment of
knee flexion deformities emphasizes the challenge that these
deformities present [34]. Treatment of knee deformities
should start at birth with intensive stretching exercises and
bracing. If the flexion deformity exceeds 30° and is not
responsive to physiotherapy, serial casting or braces with
hinges may be used in order to gradually stretch the contrac-
ture. If these conservative measures fail to decrease the con-
tracture to 20° or less, more aggressive techniques should be
used such as soft tissue releases, femoral supracondylar oste-
otomy with or without shortening, anterior hemiepiphysio-
desis of the distal femur, and gradual correction of the
Table 18.2 Indications of management for knee contractures

Knee contracture
severity Degrees Management
Mild 0–20 • Physiotherapy
• Stretching
• Serial casting
Moderate 20–40 • Anterior distal femoral
hemiepiphysiodesis
Fig. 18.10 (a) Radiograph showing vertical talus in a 2-year-old child • Soft tissue release
with arthrogryposis. (b) Radiograph obtained after talectomy was per-
Severe 40–60 • Femoral extension osteotomy
formed shows good position of the calcaneus under the tibia. (c)
Radiograph obtained 4 years postoperatively Very severe 60+ • Gradual distraction
Fig. 18.11 (a) Preoperative radiograph showing a severe knee flexion Radiograph obtained 2 months postoperatively. (d) Radiograph
deformity in a 5-year-old child with arthrogryposis. (b) Postoperative obtained 1 year postoperatively showing remodeling
radiograph after supracondylar osteotomy was performed. (c)
deformity using external fixators. However, some of these Because of the rapid remodeling that occurs following these
techniques have a high rate of recurrence in the skeletally extension osteotomies (about 1° a month) [40], this tech-
immature patient and the natural history of knee flexion nique has a high recurrent rate of flexion deformities and it is
deformity is typically one of progression [38]. therefore preferable to reserve that technique for patients
Soft tissue releases of the hamstrings alone are usually closer to skeletal maturity [34].
not enough to correct the flexion deformity, and the release In severe knee flexion deformities of more than 60°, it
should include the posterior capsule of the knee joint. The may be safer to use gradual correction with external fixators
surgical dissection is not easy as much of the muscle is [36] (Fig. 18.12). This has the advantage of protecting the
replaced by fibrous tissues and it may be difficult to identify neurovascular structures from sudden stretching, unless a
the neurovascular structures. The posterior capsule has to be concomitant shortening is performed with the supracondylar
released in most cases as it is very thick and is believed to be osteotomy. Posterior soft tissue release could be performed
one of the main factors in recurrence of the deformity [33]. before the application of the external fixator [36].
Anterior hemiepiphysiodesis of the distal femur is another Furthermore, gradual distraction may lead to an increase in
excellent option that has been recently described [38]. This the arc of motion of the knee. Attention to details of place-
technique is minimally invasive and should be used as a first- ment of the hinges when standard Ilizarov circular fixator is
line treatment of flexion deformities in the skeletally imma- used is important in order to prevent subluxation of the knee
ture patient with mild to moderate deformities—less than or compression of the articular cartilage [33]. Gradual dis-
45° [33, 39]. Care should be taken in the placement of the traction may be associated with several complications,
tension-band plates so as not to interfere with the patello- including fractures, exacerbation of the osteoporosis already
femoral joint. Supracondylar extension osteotomy with dis- present in these patients, knee subluxation, pin tract infec-
tal femoral shortening is another excellent option in the tion, pain, and recurrence of the deformity [36].
treatment of knee flexion deformities as it avoids extension Knee extension contractures are less common [33] and
soft tissue dissection around the knee. The main problem may vary from full extension of the knee with no flexion,
with this technique is that it does not increase the range of knee recurvatum, and anterior knee subluxation to frank
motion of the knee—it only changes the arc of motion. knee dislocation [33]. Long-standing deformities may lead
Therefore, an increase in extension of the knee leads—at the to abnormal loading on the articular cartilage with subse-
same time—to a decrease in flexion, and this may interfere quent damage and deformities of the epiphysis. Treatment of
with the sitting position and getting in and out of a car [33]. these contractures and/or dislocations should also start
Hence, careful planning of the treatment intervention is nec- immediately after birth and include passive stretching, cast-
essary, as well as a discussion with the patient and family ing, and bracing. If knee extension contracture is associated
preoperatively regarding the expectations of the surgery. with a clubfoot deformity, then corrective casting could be
Fig. 18.12 (a) Photograph shows a severe knee flexion deformity in a ing with this degree of knee flexion contracture. (d) Photograph shows
10-year-old patient with arthrogryposis (scar shows previous soft tissue gradual correction with a circular external fixator. (e) Photograph shows
releases). (b, c) Photographs show that the patient had difficulty stand- full correction has been obtained
Box 18. 2. Management of the Lower Limb Deformities

– Bracing
in Amyoplasia
– Casting following posterior soft tissue releases
• Early aggressive physiotherapy – Anterior hemiepiphysiodesis of the distal femur
• Feet – Distal femoral extension osteotomies
– Early tenotomy of the tendo Achilles plus – Gradual soft tissue distraction
Ponseti technique • Knee extension contractures
– If Ponseti technique fails, consider posterome- – Stretching
dial release – Casting
– In severe and recurrent cases, talectomy or mul- – Quadricepsplasty
tiple osteotomies with acute or gradual distrac- • Hip flexion contractures
tion are indicated – Physiotherapy
• Knee flexion deformities – Soft tissue releases
– Stretching – Subtrochanteric extension osteotomy
• Hip instability
– Reduce unilateral hip dislocation
– In bilateral hip dislocation, surgical treatment
depends on mobility of the hip
• The potential for ambulation is not related to the
severity of deformities at birth; warranting early
intervention for optimal long-term function
performed on both the foot and knee simultaneously [33]. If

surgical correction of the foot is required, then sufficient
amount of knee flexion is necessary in order to maintain the
position of the foot, and hence knee surgery could be
performed at the same time as the foot surgery [33]. In cases
of failure of conservative treatment for knee extension con-
tractures, a quadricepsplasty is indicated when less than 35°
flexion is present despite nonoperative measures [41]. Most
authors suggest that surgery should be performed early,
between 3 and 6 months of age [41]. Numerous techniques
of quadricepsplasty have been described, including percuta-
neous, mini-incision, and open techniques [33]. Care should
be taken not to over lengthen the quadriceps tendon [33].
Knee dislocations usually require surgery and should be
reduced before 6 months of age [33].
Hip
Hip involvement is common in patients with amyoplasia and
has been reported to range from 50 to 90 % [18, 42].
Contractures may occur as isolated flexion or external rota- Fig. 18.13 (a) Radiograph shows a unilateral hip dislocation in a
tion contractures, or as a combination of flexion, external neonate with arthrogryposis. The contralateral hip shows an abduction
contracture. (b) Radiograph shows failed closed reduction of the right
rotation, and abduction contractures. Stretching exercises hip and the abduction contracture of the contralateral hip has been suc-
should start immediately after birth. It is difficult to apply cessfully treated with physiotherapy. (c) Postoperative radiograph
orthoses for hip flexion contractures. An increased lumbar obtained after open reduction showing avascular necrosis of the right hip
lordosis may compensate for a hip flexion deformity.
However, if there is persistent hip flexion contracture of more reduction of bilateral dislocations have a high rate of compli-
than 30° despite physiotherapy, surgical treatment is usually cations, including increased stiffness, avascular necrosis,
required as such contractures may interfere with walking and the possibility of redislocation and hence recommend no
[43]. Soft tissue release should be considered, although it is treatment. On the other hand, we and others favor reduction
very difficult to obtain complete correction of the contracture of bilateral dislocation as this may help function and ambula-
with soft tissue release only. Alternatively, subtrochanteric tion [25, 42].
extension femoral osteotomy may be considered, but the Closed reduction or the use of Pavlik harness is often
recurrence rate is high [44]. For these reasons, it is preferable unsuccessful [46, 47] and open reduction is usually neces-
to wait until skeletal maturity before performing a subtro- sary (Fig. 18.13). We prefer to use a Smith-Peterson approach,
chanteric extension osteotomy. External rotation contrac- although a medial approach is advocated by others [21].
tures may be beneficial as they widen the base of stance and A complete capsulotomy near the acetabular brim is neces-
in most of the cases do not need special treatment [17]. sary, together with a pelvic osteotomy, usually an acetabulo-
Hip dislocations occur in about 10–30 % of patients with plasty. A high rate of avascular necrosis, up to 70 % in some
amyoplasia [45] and are almost always teratologic. Almost series, has been reported [46]. Postoperatively, a hip spica
all authors agree that a unilateral hip dislocation needs to be should be kept for 8–12 weeks, followed by part-time brac-
treated in order to prevent pelvic obliquity, scoliosis, and ing for another 12 weeks [17]. Bilateral stiff hip dislocations
unequal sitting pressure. On the other hand, the management with limited abduction may interfere with toileting, and in
of bilateral hip dislocation in children with amyoplasia is such cases bilateral valgus osteotomies are recommended
controversial. Some authors believe that attempts at surgical (Fig. 18.14).
usually detected during the first few years of life. Early onset,
a paralytic curve pattern, and pelvic obliquity are poor prog-
nostic patterns for curve progression. Bracing is usually inef-
fective in controlling the curve and most children with severe
scoliosis will require surgical treatment. Combined anterior
and posterior fusion seems to give the best curve correction
with the least loss of correction after surgery. Children with
arthrogryposis should have a physical examination of the
spine early on with regular follow-ups, including upright
radiographs to avoid an undetected severe deformity that is
difficult to treat.
Orthopedic Management of Specific Conditions

Popliteal Pterygium Syndrome
Conservative treatment in the form of physiotherapy, trac-
tion, and casting is usually unsuccessful. Careful preopera-
tive planning with MRI examination is essential in order to
delineate the exact anatomical position of the sciatic nerve.
When the popliteal webbing is mild, multiple skin Z plasties,
excision of the posterior fibrotic band, hamstring lengthen-
ing, posterior capsulotomy, and tendo Achilles lengthening
are recommended. When the flexion deformity is severe,
Fig. 18.14 (a) Radiograph of a young girl with arthrogryposis show-
femoral shortening and extension osteotomy with soft tissue
ing a bilateral abduction contracture of the hips. (b) Radiograph show- release may be one option [51]. Another option that seems to
ing bilateral valgus osteotomies with increased abduction give good results in severe deformities is gradual correction
using a circular fixator, excision of the dense fibrotic band
Upper Limb and multiple Z plasties of the skin.
The classical upper limb deformities in arthrogryposis
include adduction and internal rotation of the shoulder, exten- Larsen Syndrome
sion of the elbow, flexion and ulnar deviation of the wrist, and Conservative treatment is typically unsuccessful in stabiliz-
stiff fingers with thumb-in-palm deformity. The goal in the ing the knees and surgery, including lengthening the quadri-
management of upper limb deformities is to maximize func- ceps is recommended. The treatment of bilateral hip
tional independence by positioning the limbs for optimum dislocations is controversial. Equinovarus deformities are
use. For a long time, it was believed that the best position of treated with posteromedial release. Conservative methods
the upper limbs should be one limb in full extension and one are recommended for the correction of hip dislocations, knee
in flexion. However, absence of a strong unilateral grasp ren- deformities, and clubfeet, and operative correction should be
ders the use of bimanual function necessary, and therefore a performed once the child’s general health is stable [52].
bimanual use pattern of the upper limbs is recommended. Because of the potential morbidity and mortality associated
Preservation of available elbow and wrist motion is essential with cervical kyphosis, early posterior spinal fusion is indi-
[48]. Early surgical treatment is warranted if bony changes of cated for patients with mild and flexible cervical kyphosis.
normal elbow joints occur early on, and if development and Children with Larsen syndrome should be screened with
independence in daily activities may be promoted through radiographs at their first visit for early detection of cervical
correction of the upper limbs [49]. Ideally, the upper limbs kyphosis to enable timely surgical management with poste-
should be positioned at tabletop level in order to facilitate rior fusion [53].
self-care, self-feeding, and the use of computers.
Scoliosis Timing for the Management of Lower

The reported incidence of scoliosis in arthrogryposis is Limb Deformities
20–30 % [50]. About one third of children with amyoplasia
have scoliosis. There is no single curve that is typical in these Many patients will have involvement of the feet, knees, and hip
patients; congenital, paralytic, and idiopathic-like curves joints. The treatment should be individualized. However, early
have been reported, and spinal deformity varies from minimal aggressive treatment, starting with the feet, then progressing
to severe [50]. Scoliosis is typically not present at birth but is proximally to the knees and then the hips is recommended.
Intensive physiotherapy throughout the treatment process is

strongly emphasized. We do not recommend simultaneous Rehabilitation
correction of multiple joint deformities at the same time
because of the lengthy surgical intervention involved with Multiple joint contractures usually affect the child’s capacity
such a procedure and the different rehabilitation programs for of performing daily activities, such as feeding, dressing,
each joint. grooming, toileting, mobility, and transfers. Decreased func-
tion in daily activities had a linear relationship with ambula-
tory level in 30 children aged 5–18 years of age with
Ambulation in Children with Amyoplasia amyoplasia or nonsyndromic arthrogryposis, but normal
scores in happiness and pain/comfort were reported [16].
Most of the lower limb deformities are worst at birth and Participation in sports and physical activity was found to be
several studies have shown that many children with amyo- reduced in children with arthrogryposis compared to peers
plasia are able to ambulate, despite having had numerous [16, 54, 55], demonstrating the need for rehabilitation in
surgeries [18, 43]. As previously mentioned, correction of these children. The main objective of rehabilitation profes-
severe knee flexion deformities facilitates ambulation. sionals is to maximize the child’s potential and autonomy at
Muscle strength, specifically that of the quadriceps, may home, at school and in the community. Stretching and brac-
affect ambulation to a greater extent that the severity of the ing starting early in infancy is critical to optimize range of
deformities [43] and hence the importance of muscle motion. Physical therapists focus on improving gross motor
strengthening exercises. The importance of adequate bracing skills and mobility. Assistive devices (e.g., cane, walker, and
and its role in helping children ambulate cannot be overem- wheelchair) are recommended depending on the severity of
phasized (Fig. 18.15). Other factors that may affect ambula- contractures and involvement of joints in order to promote
tion include upper limb function and the child’s and independent mobility. Technical aids and other customized
caretakers’ motivation. Although amyoplasia is not a pro- adaptations such as adapted utensils, raised working surface
gressive disorder, there is a tendency for recurrence of the and adapted toys, and access to computers are provided to
deformities after correction. compensate for decreased function and facilitate autonomy
in activities of daily living (e.g., feeding, writing, play, com-
puter use). Occupational therapists focus on fine motor skills
and activities of daily living. Splints or braces may be indi-
cated to improve or maintain range of motion and joint align-
ment, to prevent contractures, and to maximize function in
everyday activities such as eating, writing, or walking.
Speech therapy, nutrition, and other rehabilitation areas may
be warranted depending on the child’s underlying needs. An
individualized treatment plan delivered throughout the lifes-
pan of the affected child with arthrogryposis via a multidis-
ciplinary team is essential to promote each child’s full
potential during infancy, pre-school, school-age, and adoles-
cence (Table 18.3). Adequate transitioning to adult health
care services and preparation for independent living is rec-
ommended when the child is nearing discharge from the
pediatric hospital center.
Summary
Arthrogryposis is a general term that encompasses a multi-

tude of different disorders with the commonality of multiple
congenital contractures. The lower limbs are often affected,
requiring surgical interventions and intense and long-term
rehabilitation. Clubfoot deformity or talipes equinovarus is
Fig. 18.15 Bracing and the use of canes to facilitate functional ambu- the most common foot deformity in arthrogryposis. The knee
lation as well as transfers from a wheelchair is the second joint most commonly affected. Knee contractures
Table 18.3 Goals in rehabilitation during the lifespan

Lifespan years Rehabilitation goals Frequency of therapy
Infancy • Increase range of motion and maintain gains Weekly
• Positioning
• Educate and reassure parents and family about handling and caring for their child
Pre-school and • Maintaining range of motion Weekly or monthly for
school-age • Strength training follow-up
• Promoting independence in functional activities
• Assistive devices to assist mobility
• Technical aids and customized adaptations to maximize function in self-care,
handwriting, computer, and leisure activities
Adolescence • Therapy following surgery to improve range of motion and strength Intensive therapy postsurgery
• Maximize independence in school and community Consultation and monitoring
• Transitioning to adult health care as needed (e.g., driving)
• Preparation for independent living
can be the most disabling deformity as they prevent motion 10. Hall JG, Reed SD, Greene G. The distal arthrogryposes: delineation
of new entities – review and nosologic discussion. Am J Med
and anatomical limb alignment. Treatment must be individu-
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Limb Lengthening and Deformity
Correction in Patients with Skeletal 19
Dysplasias
Mihir M. Thacker, Ellen Dean Davis, Colleen P. Ditro,

and William Mackenzie
Skeletal abnormalities in patients with skeletal dysplasias

Introduction can be a source of considerable morbidity and often need
surgical correction [5, 10]. These may result from:
Skeletal dysplasias include a wide and varied array of disor-
ders, all characterized by an abnormality in the formation of 1. Shortening of specific limb segments (rhizomelic,
bone and cartilage. There are many (>400 types) skeletal mesomelic, and acromelic) characteristic of many dispro-
dysplasias and most are caused by specific mutations in portionate skeletal dysplasias with short stature.
genes involved in skeletal development [1] (Table 19.1). 2. Unequal growth rates of paired long bones in the forearm
These disorders are often associated with short stature (pro- and leg (e.g., relatively long fibula in achondroplasia asso-
portionate or disproportionate), angular deformities of long ciated with genu varum; and relatively short fibula in chon-
bones, joint surface irregularity, and ligamentous laxity/ dro-ectodermal dysplasia associated with genu valgum).
instability [2–5]. Patients with skeletal dysplasias may have 3. Joint contractures, deformities, and instability: Joint contrac-
spinal deformities and/or instability which can be a signifi- tures result from angular deformation centered at the joints
cant cause of morbidity. In addition to skeletal manifesta- rather than in the adjacent physeal and metaphyseal regions
tions, they may also have significant systemic involvement, of the developing bones. These are almost always in flexion
which often is the cause of premature mortality [2, 5] and are particularly characteristic of diastrophic dysplasia.
(Table 19.2). Extremity involvement can be quite disabling Joint instability can result from a combination of generalized
and affect quality of life. Treatment of these children must ligamentous laxity and structural epiphyseal abnormality
involve a multidisciplinary team familiar with both skeletal (e.g., spondyloepiphyseal dysplasia, Morquio syndrome).
and extraskeletal concerns. There is a paucity of literature
regarding the natural history of many of these disorders, but
there has been a recent surge in interest in many of these Lower Extremity Deformity in Skeletal
disorders, particularly mucopolysaccharidoses [6–9]. With Dysplasia
improved understanding of the mechanisms involved in the
genesis of these disorders, better medical treatments are Individuals with skeletal dysplasias are not only shorter in
being formulated. stature than the average population, but each dysplasia has
its own set of unique skeletal abnormalities (Box 19.1 ).
In addition, the natural history of these dysplasias is highly
variable and influences decision making. Characteristic
deformities at various bones and joints are seen in skeletal
dysplasias (Table 19.3).
M.M. Thacker, MD (*) • C.P. Ditro, DNP • W. Mackenzie
Department of orthopedic Surgery, Nemours Alfred I. duPont The challenges of deformity correction in skeletal dyspla-
Hospital for Children, 1600 Rockland Road, Wilmington, sia patients include:
DE 19803, USA
e-mail: [email protected]; [email protected];
• Multifocal, and often multiplanar deformities (sagittal,
[email protected]
coronal, rotational, and translational).
E.D. Davis, MD
• The abnormalities in ossification can impair visualiza-
Department of orthopedics, St. Joseph’s Regional Medical Center,
246 Hamburg Turnpike, Suite 305, Wayne, NJ 07470, USA tion of the chondro-osseous structures and joint lines on
e-mail: [email protected] plain radiographs, adding another level of difficulty to

314 M.M. Thacker et al.
Table 19.1 Site of genetic mutation and mode of inheritance of certain Table 19.3 Characteristic deformities of various skeletal dysplasias
bone dysplasias
Deformity Skeletal dysplasia
Dysplasia Gene defect Mode of inheritance Acetabular dysplasia Diastrophic dysplasia
Diastrophic dysplasia DTDST gene AD MED
(5q32-q33.1) Morquio syndrome
Multiple epiphyseal COMP (Chr19) AD, less commonly Coxa vara Cleidocranial dysplasia
dysplasia (MED) COL9A2 AR MED
Type I Pseudoachondroplasia
Type II SED—congenita
Pseudoachondroplasia COMP (chromosome AD Schmid metaphyseal chondrodysplasia
19q13) Coxa valga Morquio syndrome
Ellis-van Creveld EVC gene AR Hip degenerative disease Pseudoachondroplasia
syndrome (chromosome 4p16) MED
Achondroplasia FGFR3 80 % Sporadic SED
mutation Genu varum Achondroplasia
20 % AD McKusick metaphyseal
Hypochondroplasia FGFR3/IGF-1 Spontaneous chondrodysplasia (mild)
mutation, less Pseudoachondroplasia
commonly AD Schmid metaphyseal chondrodysplasia
Spondyloepiphyseal Type II collagen Congenita: AD Genu valgum Diastrophic dysplasia
dysplasia (SED) Tarda: x-linked Ellis-van Creveld syndrome
recessive MED
Kniest syndrome Type II collagen AD Morquio syndrome
Pseudoachondroplasia
Metaphyseal Collagen type X A1 AD
SED - congenita
chondrodysplasias chain deletion AD
Schmid type PTHRP (chromosome AR Internal tibial torsion Achondroplasia
Jansen type 3p22-p21.1) Schmid metaphyseal chondrodysplasia
McKusick RMRP (chromosome 9) External tibial torsion
Cleidocranial RUNX2/CFBA1gene AD Ankle varus Achondroplasia
dysostosis (chromosome 6) McKusick metaphyseal
Morquio syndrome GALNS/GLB1 gene AR chondrodysplasia (mild)
(type IV Mucopoly- defect Schmid metaphyseal chondrodysplasia
saccharidosis) Talipes equinovarus SED (supple)
Diastrophic dysplasia (rigid)
AD autosomal dominant, AR autosomal recessive, DTDST diastrophic
dysplasia sulfate transporter, COMP cartilage oligomeric protein, COL MED multiple epiphyseal dysplasia, SED spondyloepiphyseal
collagen, EVC Ellis-van Creveld, FGFR3 fibroblast growth factor 3, dysplasia
IGF1 insulin like growth factor 1, PTHRP parathyroid hormone related
peptide, RMRP RNA component of mitochondrial RNA processing
endoribonuclease, GALNS N-acetylgalactosamine-6-sulfatase, GLB
beta galactosidase, RUNX2 runt related transcription factor 2
Table 19.2 Selected systemic abnormalities in patients with skeletal

dysplasias planning the deformity correction. This may necessitate
additional imaging studies such as MRIs (often needing
System
involved Pathologic condition Skeletal dysplasia sedation or anesthesia, which is not without its difficul-
Head Hydrocephalus Achondroplasia ties) and CT scans as well as liberal use of intraoperative
Eye Cataracts Morquio syndrome arthrograms.
Retinal detachment SED congenital, Kneist • Deformity at one site (and its correction) may influence
Ear Recurrent otitis media Achondroplasia, Kneist the development and/or progression of deformities at
Hearing loss Kneist adjacent sites. Other challenges include deciding which
Cardiac Congenital heart defects Ellis-van Creveld deformities need to be managed surgically and which can
Acquired dysplasia
(cardiomyopathy, Morquio syndrome be accommodated by adjacent correction.
valvular disease)
Respiratory Laryngotracheomalacia Campomelic dysplasia, Skeletal dysplasias can affect the quality of bone and carti-
Central +/− obstructive diastrophic dysplasia lage. Therefore, it is imperative to have an understanding of the
sleep apnea Achondroplasia
pathophysiology and natural history of these conditions. These
Restrictive lung disease Metatropic dysplasia,
thanatophoric dysplasia, differences can affect the types of correction and fixation used.
Ellis-van Creveld dysplasia The size and quality of bone often times may influence implant
Immunologic T-cell impairment, McKusick metaphyseal selection and necessitate creativity in the operating room in
neutropenia, anemia chondrodysplasia order to accomplish the desired surgical goals.
19 Limb Lengthening and Deformity Correction in Patients with Skeletal Dysplasias 315
with back or radicular pain, neurogenic claudication, incon-

Box 19.1 tinence, or objective neurologic findings (such as hypertonia,
• Each skeletal dysplasia is unique with specific skel- brisk reflexes, clonus, motor weakness) should be evaluated
etal and systemic manifestations. Evaluation by an with an MRI [12]. The child’s gait is assessed by observing
experienced multidisciplinary team is essential. the child walk, run, and play [13]. Gait analysis can be help-
• Deformities in skeletal dysplasias are usually multi- ful in these patients. Discussion with the family is critical to
focal and multiplanar. establish the goals of treatment as well as to manage expec-
• Deformities may arise from bony, articular, as well tations appropriately.
as soft tissue abnormalities. These may need to be
addressed individually for appropriate correction.
Imaging
Patients with certain skeletal dysplasias have upper cervi- A skeletal survey is often performed at the initial visit to
cal instability and need appropriate evaluation. identify the skeletal features that aid in diagnosis. At our
institution, skeletal survey for skeletal dysplasia typically
includes anteroposterior (AP) and lateral radiographs of the
Surgical Considerations cervical, thoracic, and lumbar spine and AP view of the long
bones of the upper and lower extremities as well as of the
Preoperative Evaluation pelvis, hands, and feet. Cervical flexion-extension views are
obtained in most dysplasias (except for achondroplasia). We
Evaluation of a child with skeletal dysplasia is best per- add further radiographs as clinically indicated. AP and lateral
formed by a multidisciplinary team (including orthopedic full-length radiographs of the lower extremities (preferably
surgery, genetics, physical and occupational therapists, and weight-bearing views) are mandatory prior to surgical inter-
other necessary specialists such as otorhinolaryngology, vention. These allow for an evaluation of the overall align-
neurosurgery, ophthalmology, and dentistry) familiar with ment and location of deformities. It is also imperative to
these conditions [5, 11]. A thorough history, including ques- obtain dedicated weight-bearing radiographs of the specific
tions regarding pain, endurance, gait abnormalities, activi- joints to allow for appropriate deformity analysis (please
ties of daily living, and recreation, provides clues to refer to Chap. 2). The full-length radiographs alone will not
functional limitations. A detailed systemic evaluation is also always allow for an accurate quantitative determination of
indicated and may identify major, sometimes life-threaten- the magnitude of deformity. For example, the magnitude of
ing, issues (see Table 19.2). The extremities should be exam- ankle deformity can be misjudged based on full-length radio-
ined for range of motion, ligamentous laxity, joint graphs centered at the knee because of the parallax of the
contractures, and malalignment (in all planes). Examination X-ray beam. Additional three-dimensional studies such as
of the spine for sagittal and coronal plane deformities and a computed tomography and/or magnetic resonance imaging
thorough neurological examination are needed because of may be performed for detailed evaluation of bone and carti-
the frequent incidence of spinal deformities, instability, and lage anatomy, measurement of torsional abnormalities,
neurological compromise in certain dysplasias. Cervical assessment of joint condition, etc. However, these are static
spine instability is common in mucopolysaccharidoses like studies and may not always reflect the alignment of the limb
Morquio syndrome, spondyloepiphyseal dysplasia (SED), in conditions of dynamic loading (gait). We often perform a
diastrophic dysplasia, pseudoachondroplasia, and chondro- three-dimensional gait analysis in these patients as an adjunct
dysplasia punctata. Flexion-extension radiographs are useful to X-rays to aid in preoperative decision making. Such
in evaluating such patients who may be at risk for cervical dynamic evaluation helps to quantify the forces across the
instability, especially if they have symptoms of neck pain various joints and also helps us compare pre- and postopera-
and long track signs. We use flexion-extension radiographs tive function.
in most dysplasias, except for achondroplasia, at presenta-
tion and also intermittently thereafter, especially in dyspla-
sias such as SED or Morquio syndrome, where instability Anesthesia
may develop over time. These, however, may be somewhat
difficult to interpret in this population given the delayed ossi- Preparation of the patient for the surgical procedure is at least
fication, odontoid hypoplasia, limited neck motion, and as important as the surgical procedure itself (Box 19.2). Given
abnormal anatomy seen in many of these patients. the fact that many of these patients have small and “difficult”
Additionally, sometimes the bony landmarks used for mea- airways, preoperative anesthesia consults and discussion of
surement may be obscured by overlapping shadows cast by the various issues specific to that patient are very useful. There
the mandible, occiput, and foramen magnum. Any patient may be minor surgical procedures but there is no “minor
anesthesia” for these patients. Anesthesia should be performed Implant Size and Design
by an anesthesiologist familiar with various medical comor-
bidities associated with this patient population. Our institu- Implant size and design needs to be considered in preopera-
tion has described a “difficult” airway management in these tive planning. Some of these patients are extremely small
patients as one which requires two anesthesia providers when (primordial dwarfism) and appropriately sized implants are
ventilating with a facemask. One provider applies the face critical to the successful execution of the preoperative plan.
mask while maintaining anterior mandibular displacement The popular commercially available precontoured implants
and cervical stabilization while the other ventilates with posi- will rarely accommodate the morphology of bones in patients
tive pressure [14]. Care must be taken to protect the neck in with skeletal dysplasias. Given the poor bone quality in some
patients with cervical instability by using in-line cervical trac- of the patients, locking plate and screw constructs may be
tion [15]. In addition, specialized tools such as video laryngo- useful when performing acute corrections. Even while plan-
scope and fiberoptic intubation should be readily available in ning deformity correction with external fixators, implant
these situations. Given the fact that each anesthetic session selection should be kept in mind. For example, the appropri-
poses a certain risk to the patient, we prefer to combine as ate fit of a Taylor Spatial Frame (Smith & Nephew; Memphis,
many procedures as feasible under the same anesthetic. TN, USA) may necessitate use of extra-extra short struts
Regional anesthesia is often used for pain control and to (which may need to be ordered preoperatively). Frequently,
reduce the amount of narcotic needed during and after sur- given the multifocal nature of the deformities, more than one
gery. Caudal epidural analgesia is preferred over lumbar epi- osteotomy and therefore fixation options may be needed in
durals in patients with known vertebral anomalies, prior the same bone. Having an appropriate inventory of instru-
fusions, and thoracolumbar kyphosis that can co-exist with ments and implants saves the surgeon from frustration and
these conditions. Typically, caudal epidural may be adminis- improves patient outcome.
tered to those patients 10 years of age and younger who
undergo lower extremity osteotomies. Careful dosing of the
epidural medications and regular neurovascular checks are Methods of Deformity Correction
essential to avoid missing the diagnosis of an early compart-
ment syndrome. The principles of deformity correction in the dysplasia popu-
lation are no different from those without dysplasias.
However, the multifocal and multiplanar nature of the defor-
Positioning mities, issues with size and quality of the bone, ligamentous
laxity, and growth potential make deformity correction
Positioning these patients is also critical as patients with extremely challenging in these patients. Many of these
severe kyphosis are at risk for developing paraparesis/para- patients have substantial delay in ossification, making
plegia with prolonged supine position. The pathogenesis of planning and correction of deformities even more challeng-
this phenomenon is poorly understood at this time. ing. It has been demonstrated that the choice of osteotomy
Neuromonitoring should be considered in long procedures in often times changes based on the results of intraoperative
such high-risk patients undergoing lower extremity surgery. arthrography, particularly in patients under 8 years of age
[16]. We use arthrography liberally during surgery to miti-
gate some of these issues. This helps us not only to visualize
Box 19.2 the cartilaginous anatomy but also to evaluate the dynamic
• Careful anesthetic technique, protecting the cervi- stability of the joint with stress views in the operating room.
cal spine and minimizing the number of anesthetic Deformity correction in these patients can be accom-
episodes, is important. plished via acute or gradual methods.
• Consider neuromonitoring during nonspinal proce-
dures in the supine position in patients with severe
kyphosis to minimize risk of adverse neurologic Acute Correction
events.
• Careful preoperative planning and attention to Acute methods of deformity correction include use of oste-
addressing the bony and soft tissue deformities is otomies with immediate correction and fixation via various
key to improving outcomes. methods. In general, these techniques follow the same prin-
• Sequence of correction of deformities may vary in ciples as those patients without skeletal dysplasias. Care
individual circumstances, but is usually proximal to should be taken to be familiar with the various deformities
distal. associated with particular conditions (see Table 19.3). For
example, genu varum is present in the majority of achondro-
plastic children by 5 years of age and 90 % of achondroplastic the physis after staple removal is unpredictable, necessitat-
adults [17]. The genu varum can be secondary to distal femo- ing avoidance of excessive overcorrection and close moni-
ral varus, lateral joint line opening, proximal and distal tibial toring until skeletal maturity [21]. There are some skeletal
varus and is nearly always associated with internal tibial tor- dysplasias that are associated with extremely slow growth or
sion. Progressive varus, symptomatic gait abnormalities, lat- have significant joint laxity, which can affect correction.
eral thrust, and occasionally cosmesis are our typical Slow growth and a significant deformity is a combination
indications of genu varum correction. One or all of these that will likely lead to failure of correction when using
deformities may be present, so careful preop planning must guided growth treatment in such patients (Box 19.3).
be done while planning the correction. The deformity in
young children usually is due to proximal tibial varus, lateral
joint line opening, and internal tibial torsion. These children Gradual Correction with External Fixation
can be successfully treated with a proximal tibial osteotomy
with fibular shortening osteotomy via acute correction. Care Gradual deformity correction can also be accomplished
must be taken to displace the proximal tibia in order to main- using external fixators [22, 23]. This approach has two main
tain a neutral mechanical axis and correct the typical internal advantages. Primarily, the multifocal and multiplanar defor-
tibial torsion. With this method, acceptable correction can be mities that are associated with skeletal dysplasias can be
attained [18]. Recurrence is common in very young children, addressed at one surgery. Some of the gradual correction
especially if there is undercorrection. It has been suggested devices also allow for simultaneous multiplanar correction,
that the preferred treatment for skeletally mature individuals addressing coronal, sagittal, and axial deformities without
with achondroplasia and distal tibial varus is an acute open- the need for complex osteotomies that are prone to error.
ing distal tibial osteotomy combined with a shortening distal Secondly, this method also allows for concurrent limb
fibula. Displacement usually is required to correct the lengthening in this patient population with varying degrees
mechanical axis [18]. The authors have typically used a of short stature. Patients must be counseled about their care
shortening, derotational osteotomy to manage this deformity. and management preoperatively as limb lengthening can be
With acute deformity correction, one can re-establish normal associated with several complications [24, 25]. However,
mechanical alignment, but not perform additional limb with appropriate instruction and care, external fixators are
lengthening. In addition, acute corrections, particularly of extremely useful for correction of most complex deformities.
the proximal tibia, have been associated with complications Currently, we prefer to use the Taylor Spatial Frame (Smith
including peroneal nerve palsies, vascular injuries, and com- & Nephew; Memphis, Tennessee) for more complex defor-
partment syndromes [19]. mities and a uniplanar external fixator for some of the less
complex deformities.
Gradual Correction with Guided Growth

Box 19.3
Gradual deformity correction is a powerful tool for angular • Growth modulation around the knee is a useful
realignment and/or lengthening in the skeletal dysplasia pop- technique for deformity correction in patients with
ulation. This can be done by way of guided growth tech- skeletal dysplasia, but the results are dependent on
niques or external fixation methods. There is a paucity of the growth potential and degree of associated liga-
literature that addresses the application of guided growth mentous laxity.
techniques in this unique population. However, we have • Special inventory of implants of appropriate size is
demonstrated that growth modulation using a tension band essential.
plate and screw system or staples is an effective way to pro- • Plan procedures keeping in mind the natural history
vide deformity correction in a variety of skeletal dysplasias of the underlying skeletal dysplasia.
[20]. It is a relatively simple surgery that has a low risk of
damage to the physis or mechanical failure. We have also
found that despite an abnormal epiphysis and metaphysis,
the screw purchase has been reliable. An exception is pseu-
doachondroplasia where it can be difficult to achieve epiphy- Controversies in Lengthening for Stature
seal fixation. This technique can be used in very young
patients [20]. Guided growth via hemiepiphyseal stapling in Limb lengthening (without the correction of angular defor-
patients with multiple epiphyseal dysplasia (MED) has also mities) in short-statured individuals has been a subject of
been studied. In these children, stapling has been effective debate [24–29]. The achondroplastic population has been the
for angular deformity correction. However, the response of most studied to date. The motivation to lengthen limbs in the
skeletal dysplasia population has mainly been to improve There is a role for stature lengthening in the carefully
functional disabilities associated with difficulty in locomo- selected patient who is well aware of the long and arduous
tion [30]. Other concerns, particularly in the idiopathic short- nature of the process and the potential for significant compli-
statured patient, have been related to aesthetic, social, and cations. However, the patient must be counseled that the gain
psychological issues. in height may not correlate to an improvement in quality of
Medical and surgical treatments to address short stature in life [25].
achondroplasia have been studied. Growth hormone (GH)
administration has led to variable results [31]. In trials, early
gains in height after commencement of GH treatment have Postoperative Considerations
been seen to varying degrees. However, the effects have not
been as promising with continued administration. There are There are several aspects of the postoperative course specific
also concerns of aggravating the disproportionate nature of to the short-statured patients that should be taken into con-
limb segment shortening. Surgical techniques for stature sideration. This is particularly true when utilizing external
lengthening in achondroplasia have addressed the femora, fixation for deformity correction. These children typically
tibiae, and humeri via distraction osteogenesis. This method require more support because of their limited upper extrem-
has reliably been able to provide increases in height, but at a ity reach. It is important to work with the physical and occu-
cost [24, 25, 27, 29]. pational therapists to provide appropriate assistive devices
The average gain in height through surgical limb length- and strategies for activities of daily living.
ening in achondroplasia has been reported to be 18–23 cm, The psychosocial aspect of deformity correction should
resulting in an average total patient height of 140 cm [24, 30, not be overlooked in patients with skeletal dysplasias. These
32]. This does not completely ameliorate the disproportion- children may have preexisting self-image concerns because
ate trunk height. There are several drawbacks to limb length- of their underlying conditions. With external fixators in
ening purely for increased stature, including prolonged place, these self-conscious feelings can be amplified. It may
treatment time and high complication rate (reported up to be useful to involve mental health professionals in the preop-
100 %). It is unclear as to whether or not such bilateral limb erative evaluation and postoperative care of these patients.
lengthening improves the patient’s quality of life. Kim et al. The skeletal dysplasia population often travels to referral
showed that there was an improvement in self-esteem (using centers for these complicated deformity corrections. We pre-
the Rosenberg self-esteem questionnaire), but there was no fer to keep these patients locally during the timeframe of dis-
difference in physical or functional scores (using the SF-36 traction and/or correction. This allows us to follow them in
and AAOS lower extremity scores) when comparing achon- clinic on a weekly or biweekly basis and to appropriately
droplastic patients who underwent lengthening versus those address any complications that may arise. This oftentimes
who did not undergo surgical treatment [24]. They studied entails working with local or national charities, such as the
22 patients with achondroplasia who had tibial and femoral Ronald McDonald House, for lodging and other necessities.
lengthening and compared them to a matched cohort with In addition, the caregivers of these children need ample time
achondroplasia who did not undergo lengthening. They had to plan for the required postoperative stay if they are not
123 complications in 88 lengthened segments but reported locally based.
that may of these were minor and did not need intervention.
They felt that lengthening was a viable option given the
improved self-esteem. The same group also looked at Planning for the Future
humeral lengthening in the achondroplasia population and
reported that humeri tend to consolidate faster and have less When planning these corrections, care must be taken to con-
fractures and angular malalignment compared to femoral sider the long-term effects of such surgical interventions.
lengthening. Extensive (more than 50 % of the length of the Many individuals with skeletal dysplasias, such as multiple
femur) lengthening of the femur results in higher incidence epiphyseal dysplasia (MED), spondyloepiphyseal dysplasia
of fractures and joint issues. Extensive lengthening is also (SED), pseudoachondroplasia, diastrophic dysplasia, and
associated with physeal growth retardation, both femoral and Morquio syndrome (MPS IVA), are predisposed to prema-
tibial. Surgical strategies include bilateral tibial lengthening, ture osteoarthritis. These patients often need total joint
lengthening of the femur and tibia on one side, crossed femur arthroplasty (TJA) at a relatively young age. This must be
and tibia or all 4 segments simultaneously. Time in external taken into account when performing osteotomies that may
fixators may be shortened by lengthening over an intramed- make TJA more difficult. The surgeon needs to consider var-
ullary nail or use of intramedullary lengthening techniques. ious obstacles when performing TJA in these patients. For
The authors rarely perform stature lengthening. instance, in total knee arthroplasty, customized implants and
constrained designs are often needed because of patient’s Several techniques for correction of genu varum in
short size and a lack of ligamentous constraints associated achondroplastic patients have been described previously.
with the underlying pathologies [33, 34]. When considering Fibular epiphysiodesis or shortening may be useful in young
total hip arthroplasty, the surgeon needs to evaluate the need patients (5–8 years old) with mild deformity [18, 40].
for potential acetabular augmentation secondary to bony However, long-term data on this technique is lacking. Acute
deficiency, the possibility of extensive soft tissue releases correction in young children with an oblique proximal tibial
because of contractures, presence of tortous and narrow osteotomy (Fig. 19.1a–k), simultaneously correcting the tib-
proximal femoral intramedullary canals, and the potential for ial varus and internal rotation, has achieved reliable results
needing custom implants due to small stature [35]. When with a low recurrence rate as long as the joint line and
proper preoperative planning is executed, good results can mechanical axis are brought to a neutral position [41, 42].
be achieved with total joint arthroplasty in patients with skel- In older children and adolescents, we prefer gradual cor-
etal dysplasia, though complication rates can be high [33, 35]. rection of the proximal tibia with a circular fixator for severe
deformities. Concurrent varus deformity in the distal femur
and distal tibia is corrected as needed. The authors tend to
Specific Skeletal Dysplasias and Their use acute correction of these associated deformities. This
Associated Deformities approach allows for simultaneous correction of multiple
sites of deformity as well as multiple planes of deformity
Achondroplasia [22, 43]. Gradual tensioning of loose lateral collateral knee
ligaments has been described but the authors do not use this
Achondroplasia is the most frequently encountered form of technique.
skeletal dysplasia with an incidence of approximately 1 in When considering isolated distal tibial varus correction,
10,000 live births [36]. This results from a mutation in the we do a very comprehensive preoperative analysis to confirm
FGFR3 gene. This over-activity of FGFR3 results in abnor- that there is no significant proximal tibial deformity.
malities in endochondral ossification, whereas intramembra- Concurrent untreated proximal tibial varus, even if mild, will
nous ossification is not affected. This therefore results in result in poor patient satisfaction.
involvement of the base of the skull (resulting in foramen Bifocal tibial osteotomies (Fig. 19.2a–d) can be utilized
magnum stenosis) and the extremities. There are predictable to correct both the proximal and distal tibial varus deformi-
patterns of deformity in this population. In the upper extrem- ties and internal rotation and also simultaneously gain length
ity elbow flexion deformities are common and often associ- with an acceptable complication risk (mostly equinus con-
ated with distal humeral recurvatum. The severe rhizomelia tractures with more than 15 % lengthening through the distal
seen in these individuals can result in marked limitation in tibial osteotomy) [44].
reach and humeral lengthening can improve function in these
patients. In the lower extremity, hip flexion and knee defor-
mity are often noted. The most commonly addressed defor- Spondyloepiphyseal Dysplasia
mity is genu varum, which is often associated with internal Congenita (SEDC)
tibial torsion and occasionally genu recurvatum. A common
misconception is that uncorrected genu varum in children Spondyloepiphyseal dysplasia congenital (SEDC) results in
with achondroplasia results in knee osteoarthritis later in life. a short trunk type of disproportionate dwarfism (Fig. 19.3).
In fact, osteoarthritis is rare and the knee joint is usually not As the name suggests, both the axial and appendicular skel-
a source of functional limitation in these patients. Spine eton are involved. The genetic cause for this condition
problems such as central stenosis of the lumbar segments involves an abnormality in type II collagen [45]. Lower
often result in limited function. The etiology of genu varum extremity problems include coxa vara, femoral retroversion,
is unclear. Several mechanisms have been proposed, includ- hip flexion contracture, genu valgum, and foot deformities,
ing ligamentous laxity, obesity, asymmetric growth of the such as equinovarus and planovalgus. As the genetic defect
proximal tibial physis, and asymmetric growth of the fibula is in type II collagen, articular cartilage is affected. This
relative to the tibia [37–39]. As previously mentioned the results in premature osteoarthritis that manifests in the sec-
varus deformity can exist in the distal femur, the knee joint, ond to third decade of life [46].
and the proximal and distal tibia. Indications for surgical cor- Severe coxa vara with neck-shaft angles less than 90° is
rection of genu varum and internal tibial torsion in these often present in patients with SEDC. This deformity can be
children include knee pain, progressive deformity, lateral difficult to judge radiographically because of delayed ossifi-
thrust during gait, and rarely, cosmesis. Genu valgum, cation of the capital femoral epiphysis [47]. We have
though uncommon, may also be seen in some patients. described the use of the Hilgenreiner-Trochanteric angle for
However, the valgus deformity is rarely severe enough to assessment of coxa vara in these children [48]. The epiphy-
warrant surgical correction. seal deformity of the hip can result in femoral head flattening,
Fig. 19.1 Achondroplasia. (a, b) Clinical photographs of a patient parallel to the joint surface. (g) Oblique plane osteotomy of the proxi-
with achondroplasia with bilateral genu varum and internal tibial tor- mal tibia. (h) Intraoperative fluoroscopy, confirming adequate correc-
sion. (c) Anteroposterior (AP) radiograph of bilateral lower extremities, tion using the bovie cord technique. (i) Postoperative radiographs after
showing characteristic skeletal changes with genu varum, long fibula. healing and cast removal demonstrate excellent alignment. (j, k)
(d) Acute correction of proximal tibial varus and internal rotation. The Clinical photographs of the patient in (a, b) after correction of genu
first step is a fibular shortening osteotomy. (e, f) The next step is an varum deformity
arthrogram to delineate the joint surface and placement of a K-wire
Fig. 19.2 (a) Thirteen-year-old boy with achondroplasia. There is mal tibial deformity and acute correction of the distal tibial deformity.
varus in both the proximal and distal tibia. (b) Bilevel tibial osteotomies (c, d) Anteroposterior radiograph of the lower extremities showing cor-
with application of a circular frame for gradual correction of the proxi- rection of the deformities bilaterally, before and after frame removal
Fig. 19.3 (a, b) Clinical

photographs of a girl with
spondyloepiphyseal dysplasia
congenita (SEDC) showing
characteristic changes such as
kyphoscoliosis, lumbar
hyperlordosis, pectus carinatum,
genu valgum
hinge abduction, and lateral subluxation [47]. Indications for angle greater than 60°, and Hilgenreiner-Trochanteric (H-T)
correction of coxa vara include hip pain and reduced func- angle of less than 0° [48–50]. Correction is performed
tion, Trendelenburg gait, a neck-shaft angle of less than through a proximal femoral valgus osteotomy. Femoral ret-
100°, progressive varus, Hilgenreiner-epiphyseal (H-E) roversion and hip flexion contracture (which are almost
Fig. 19.4 (a, b) AP and frog leg lateral views of the pelvis in a patient is placed through the trochanter to shut down the trochanteric physis.
with SEDC show delayed ossification of the head and neck of the (d) The degree of correction based on the maximum adduction view. (e)
femur. The high riding trochanters result in decreased abduction. The appearance after the proximal femoral osteotomy and fixation with
(c) Intraoperative radiographs (arthrogram) demonstrate severe coxa a blade plate. (f, g) Preoperative and postoperative long leg radiographs
vara. The contrast outlines the cartilaginous femoral head. Guide wire after correction of hip deformities and removal of hardware
always present) can be addressed concurrently through this femoral deformity may result in an aggravated appearance of
osteotomy. Fixation can be achieved with a blade plate or genu valgum [51].
locking plate system (Fig. 19.4a–g). Ideally the fixation is Genu valgum is another common deformity seen in
placed across the trochanteric apophysis to achieve physeal SEDC. The valgus can be attributed to the distal femur, prox-
closure. If there is a triangular metaphyseal fragment, we try imal tibia, or both segments. The magnitude of genu valgum
to achieve fixation of that fragment. Radiographic measure- is often related to the severity of coxa vara. This deformity
ments associated with low recurrence of coxa vara after can often be corrected by distal medial femoral and/or proxi-
proximal femoral valgus osteotomy are H-E angle less than mal medial tibial hemiepiphysiodesis. If inadequate growth
38° and H-T angle greater than 13° [48] and progressive is remaining to achieve correction through growth modula-
proximal femoral epiphyseal ossification. Open reduction of tion, then realignment osteotomy at the appropriate level can
the hip may be necessary at the same surgical procedure, but be performed (Fig. 19.5a–d). This can also address any knee
this is rare. Correction of the hip deformity should precede flexion and/or tibial torsion at the same time. Note that the
correction of knee deformity. It is important for patients and proximal femoral alignment has been corrected prior to final
their parents to be aware that correction of this proximal correction of the knee deformity (see Fig. 19.5d).
Fig. 19.5 (a) Patient with SED with bilateral genu valgum. ate translation during acute distal femoral valgus correction. This
(b) Correction of the genu valgum on the left with growth modulation. patient has also undergone proximal femoral varus correction
(c) Residual genu valgum on the right side, despite distal medial femo- bilaterally
ral hemi-epiphysiodesis. (d) Use of a locking plate to achieve appropri-
Symptomatic valgus deformities of the ankle and hindfoot The deformity at the knee is usually genu valgum [59].
can be treated with supramalleolar tibial and fibular osteoto- Prior to skeletal maturity, this can be addressed effectively
mies and hindfoot procedures as needed. The symptomatic, with guided growth techniques (Fig. 19.6a–e). After or near-
flexible foot deformities in patients with SEDC are typically ing skeletal maturity, osteotomy at the site of deformity (dis-
managed with orthotics. Stiff equinovarus feet are managed tal femur and/or proximal tibia) should be undertaken. Ankle
by techniques similar to idiopathic clubfeet. valgus can be managed with guided growth as well [20].
Multiple Epiphyseal Dysplasia (MED) Diastrophic Dysplasia
Multiple epiphyseal dysplasia is a skeletal dysplasia with Diastrophic dysplasia is an autosomal recessive condition
wide variations in clinical presentation and skeletal manifes- caused by a mutation in DTDST gene which encodes for a
tations [52, 53]. It is caused by mutations in multiple genes sulfate transporter protein [60, 61]. There is a wide variation in
including those for cartilage oligomeric protein (COMP), the phenotype of this condition. Common lower extremity
type IX collagen, and matrilin-3 and results in symmetric bony deformities include hip dysplasia with or without hip
changes of the epiphyses of long bones [52]. Spine involve- dislocation, genu valgum, and rigid foot deformities
ment is absent in these patients. They have mild short stature (Fig. 19.7a, b). Soft tissue problems include hip and knee flex-
and often present with lower extremity joint pain, angular ion contractures as well as lateral patellar dislocation [5, 62].
deformities, gait deformities, or stiffness [54, 55]. The Hips in these patients are notoriously difficult to reduce
epiphyses in these patients are often small and fragmented. and reconstruct. They are also difficult to assess radiographi-
Avascular necrosis may complicate fragmentation of the cally because of a marked delay in ossification of the proxi-
femoral head in up to 50 % of patients with MED [56, 57]. mal femur. The severity, deformity, and degree of hip flexion
Individuals with MED are predisposed to premature osteoar- contracture increase with age [63]. The deformities present
thritis, particularly in those with severe involvement of the can include severe acetabular dysplasia, a shortened femoral
proximal femoral epiphyses [58]. Hip deformity usually neck, a wide, irregular femoral head, and (relative) trochan-
mimics that seen in Legg-Calve-Perthes disease. Treatment teric overgrowth. These deformities often lead to painful and
of these deformities is similar to that for Perthes disease [47]. restricted motion along with hinge abduction. Premature
Joint pain and stiffness is very common in these children. arthritis requiring total joint replacement in young adulthood-
Anti-inflammatory medications, exercise in warm water, middle age can be expected.
stretching, and physical therapy can help to improve these Open reduction of these hips is technically difficult
symptoms. because of the severe acetabular dysplasia and short femoral
Fig. 19.6 (a, b) Progressive genu valgum, over 6 years, in a patient MED). This may be a source of symptoms if there is instability between
with multiple epiphyseal dysplasia. (c) Double layered patella is typi- the two fragments. (d) Anterior distal femoral epiphysiodesis for cor-
cally associated with patients with multiple epiphyseal dysplasia who rection of distal femoral procurvatum deformity. (e) Genu valgum cor-
have a homozygous mutation of the diastrophic dysplasia sulfate trans- rected after proximal medial tibial hemiepiphysiodesis
porter gene (one of the less common, autosomal recessive, variants of
neck. Function with hip dislocation is usually satisfactory in feet are not similar to idiopathic clubfeet. The main differ-
the short term but osteoarthritis and joint replacement is ence is that the navicular is displaced laterally [64]. Serial
inevitable. Surgical hip dislocation and femoral head reduc- manipulation and casting using the Ponseti technique can
tion have been successful in the short term for painful irregu- be effective in infancy but these feet often need surgical
lar movement in the authors’ experience. correction which must be customized to the deformity [65].
Knee problems in children with diastrophic dysplasia are Recurrent equinus is the most common long-term compli-
universal. Genu valgum is present, oftentimes with a later- cation. The feet are often scarred and further soft tissue
ally dislocated patella (25 % of patients) [5] (Fig. 19.8a, b). release doomed to failure. The authors have been managing
Progressive knee flexion contracture is often a clue as to the equinus with supramalleolar extension tibial and fibular
presence of abnormal patellar mechanics. The authors prefer osteotomies (Fig. 19.10a–c). Recurrence in the growing
to reduce the patella at a very young age to allow for remod- child is common.
eling and to alleviate valgus force at the knee. Patellar
realignment procedures that we utilize include an extensive
lateral release with lengthening of the iliotibial band and Morquio Syndrome
biceps femoris with medial plication and a vastus medialis
obliquus (VMO) advancement (Fig. 19.9a–f). Medialization Mucopolysaccharidosis type 4, also known as Morquio syn-
of the insertion of the patellar tendon insertion is often drome, is an autosomal recessive condition attributable to an
required. Anatomical variations include a shallow, irregular abnormality of the N-acetylgalactosamine-6-sulfatase
intercondylar grove and the medial femoral condyle can be (GALNS) gene or the B-galactosidase (GLB1) gene [66–
covered with synovium. 68]. Deficiency of these enzymes leads to accumulation of
Rigid foot deformities are typically seen in diastrophic keratin sulfate and chondroitin sulfate in lysosomes. Onset
dysplasia [64]. Deformities described in these children of clinical symptoms is between 1 and 3 years of age, with
vary from tarsal valgus and metatarsus adductovarus in severe early growth limitation and genu valgum being the
43 %, equinovarus and adductus in 29 %, equinus in 8 %, most apparent features. A waddling gait and flat feet are seen
metatarsus adductovarus in 13%, and normal in 7 %. These around 3 years of age (Fig. 19.11).
The hips have incomplete ossification of the proximal

femoral epiphyses and acetabular dysplasia. This leads to
progressive hip subluxation and femoral head deformation
(coxa valga, coxa magna, and coxa brevis) [69, 70]. The end
result of their untreated hip pathology is oftentimes prema-
ture osteoarthritis in early adulthood [71].
In order to adequately assess the complex deformities of
the hip joint that may warrant operative reconstruction, pre-
operative computed tomography scanning [6] and intraoper-
ative arthrography are very useful. Isolated proximal femoral
varus osteotomies or redirectional acetabular osteotomies
have not prevented progressive dysplasia and subluxation.
The acetabular bony roof is short with a large limbus. The
limbus does not ossify normally, likely secondary to the
accumulation of proteoglycans. Realignment procedures
include a proximal femoral osteotomy with an acetabular
procedure to improve coverage [7, 70, 72]. The authors use a
large shelf harvested from the inner wall of the ilium
(Fig. 19.12a–d).
Bilateral genu valgum is a prominent feature of Morquio
syndrome. This is a result of progressive deformation due to
incomplete ossification of the distal femoral and proximal
tibial epiphyses, in combination with generalized ligamen-
tous laxity [7]. Intraoperative knee arthrography is very help-
ful in planning corrective surgery about the knee. A distal
femoral and/or proximal tibial realignment osteotomy has
Fig. 19.7 (a) Child with diastrophic dysplasia with hitch hiker thumbs. been advocated [3, 70]. The authors have found success
(b) Rigid clubfeet and hallux varus in a patient with diastrophic
using guided growth techniques in the young child (preado-
dysplasia
lescent) (Fig. 19.13a, b) [20].
Fig. 19.8 (a, b) Flexed knees

secondary to dislocated patellae
in diastrophic dysplasia
Fig. 19.9 Operative correction

of patellar dislocation in a patient
with diastrophic dysplasia. (a)
Patella dislocated laterally with
the vastus medialis draped over
the anterior surface of the knee.
(b) Lateral release. Freer elevator
pointing to thickened, tight
iliotibial band. (c) Patella
relocated after extensive lateral
release. (d) Patella stabilized
with vastus medialis obliquus
advancement. (e, f) Postoperative
radiographs demonstrating
correction of genu valgum with
growth modulation and the
patella is appropriately located
on both views
Fig. 19.10 (a) Rigid equinovarus foot in a patient with diatrophic dys- shortening and posterior translation of the distal fragment. (c) Healing
plasia. A previous attempt at anterior hemiepiphysiodesis had been in plantigrade position after acute correction. These patients are at a
made at an outside facility, without success. (b) Patient was treated with high risk of recurrence
supramalleolar osteotomies with acute correction. Note the fibular
photographs of a boy with
Morquio syndrome showing the
characteristic pectus carinatum,
thoracolumbar kyphosis, genu
valgum, planovalgus feet
Fig. 19.12 (a) AP pelvis radiograph in a patient with Morquio syn- makes a Pemberton osteotomy less appealing.) (c) Improved coverage
drome, demonstrating coxa valga, subluxated hips, small epiphyses, with shelf acetabuloplasty. (d) Postoperative AP pelvis radiograph
and dysplastic acetabule. (b) Arthrogram shows residual acetabular demonstrates good femoral head coverage bilaterally with a shelf on the
dysplasia and femoral head uncoverage after proximal femoral varus right and a Pemberton osteotomy on the left
osteotomy. (Large unossified cartilaginous labrum/acetabular anlage
Fig. 19.13 (a, b) Genu valgum

correction in a patient with
Morquio syndrome using growth
modulation. Recurrence may be
seen in younger patients
Fig. 19.14 (a–c) Lower

extremity deformities in a patient
with Jansen metaphyseal
dysplasia. Characteristic severe
widening of the physes and
bowing of long bones are seen
The pes planovalgus feet seen universally in those chil- • Schmid type is the most common type of metaphyseal
dren with Morquio syndrome can usually be managed very chondrodysplasia. It is an autosomal dominant condition
well using functional foot orthoses. If conservative treatment which results from a heterozygous mutation of the
with these orthoses fails, lateral column lengthening or sub- COL10A1 gene on chromosome 6q21-q22.3. This causes
talar fusion in combination with medial column reconstruc- a defect in the α1 chain of type X collagen (which is
tion is an option [7]. However, long-term results of these essential for calcification of the hypertrophic zone of
procedures are not known. chondrocytes) [73].
• McKusick type of chondrodysplasia (also known as carti-
lage hair hypoplasia) is an autosomal recessive disorder
Metaphyseal Chondrodysplasia due to a defect in the RMRP gene on chromosome 9p13.3.
This results in milder genu varum and coxa vara and is
These are a group of dysplasias resulting from failure of uni- also associated with decreased immunity, varying degree
form ossification of the cartilage columns, with persistence of of anemia, and characteristic cone-shaped epiphyses in
cartilage islands, underdevelopment of the cartilage, resulting the phalanges [74, 75].
in deformities of the affected bones. The epiphyses are nor- • Jansen type (Fig. 19.14a–c) is a rare but severe, autoso-
mally preserved and the metaphyses are the most severely mal dominant, form of chondrodysplasia resulting from a
affected. The differential diagnoses include rickets and hypo- mutation (at 3p21.31) resulting in ligand independent
phosphatasia. These are usually associated with genu varum, activation of parathyroid hormone related protein recep-
ankle varus, and coxa vara of variable severity. There are tor I (PTHRP1). This results in hypercalcemia and hypo-
three major types of metaphyseal chondrodysplasia. phosphatemia [76, 77].
Fig. 19.15 Ellis-van Creveld syndrome (EvC). (a) Clinical photograph tion of the lateral tibial plateau. (i, j) Clinical photographs after applica-
of a girl with EvC showing severe genu valgum. (b) Characteristic dys- tion of a Taylor Spatial Frame™ and then after correction of distal
trophic nails seen in EvC, also called chondro-ectodermal dysplasia. (c) femoral valgus as well as a lateral release for correction of patellar
AP radiograph of the right lower extremity demonstrates multifocal instability. (k, l) Postoperative AP and lateral radiographs demonstrat-
valgus in the lower extremity. (d) AP radiograph of both knees demon- ing restoration of the mechanical axis after distal femoral and proximal
strates characteristic intra-articular deformity with depression of the tibial correction. (m, n) Clinical photographs after correction demon-
lateral tibial plateau. (e–h) CT scans (axial and coronal views as well as strating improved alignment as well as function
3D reconstructions) highlight the depression of the antero-central por-
Treatment of deformities in these patients varies by type Ellis-van Creveld Syndrome (EvC) or Chondro-
and severity. Patients with Schmid type dysplasia may show ectodermal Dysplasia
some spontaneous improvement in the first decade but per-
sistent or severe deformities may need correction using Ellis-van Creveld (EvC) syndrome is a rare autosomal
guided growth or osteotomies. Patients with McKusick type recessive skeletal dysplasia that is characterized by short
usually have mild deformities and rarely need treatment. ribs and disproportionate short stature, ectodermal dysplasia
Patients with Jansen type dysplasia often have severe defor- (including characteristic nail and tooth changes), postaxial
mities and need earlier and more aggressive treatment and polydactyly, and congenital cardiac defects (most com-
also have the greatest risk of recurrence. monly atrial septal defects) (Fig. 19.15a–n). This results
from a homozygous mutation of the EvC gene located on References

chromosome 4p16. Orthopedic issues include postaxial
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Lower Extremity Benign Bone Lesions
and Related Conditions 20
Lori Karol
They are most often solitary. They occur most frequently in

Introduction the long bones, with the most common site being the medial
aspect of the distal femur.
Benign bone lesions comprise a group of diseases where the Biopsy is often not needed since the radiographic appear-
presence of abnormal tissue within or on the surface of the ance is characteristic and does not resemble malignancy.
growing bone may create skeletal deformities of the lower Specimens from nonossifying fibromas show sheets of bland
limb. In conditions such as fibrous dysplasia, loss of mechan- fibroblasts with small nuclei without pleomorphism.
ical strength of the bone can lead to angular deformity or Treatment of nonossifying fibromas is nonsurgical in the
pathologic fracture. In the growing child, angular deformity vast majority of patients. Size greater than 50 % of the width
may also result from abnormal physeal growth as seen in of the bone and greater than 3.3 cm in length has been shown
multiple hereditary exostoses. Limb length discrepancy may to be predictive of pathologic fracture, but smaller lesions have
result from disturbances in physeal function as seen in such been noted to lead to stress fractures too, albeit rare [4, 5].
conditions as enchondromatosis. This chapter will discuss Pathologic fractures heal with immobilization and may lead to
the most common pathologic bony lesions seen in children resolution of the lesion. If the nonossifying fibroma is in a
that are associated with lower limb deformity. weight-bearing bone, is sufficiently large, and has undergone
pathologic fracture, curettage and bone grafting can eradicate
the lesion and allow for full athletic participation [6, 7]
Nonossifying Fibroma (see Fig. 20.1b) The natural history of nonossifying fibromas
is that they regress during adulthood.
A nonossifying fibroma is the most common benign bone
lesion found in children. It is a solitary lytic lesion which is
characterized by replacement of the normal bone with
Box 20.1. Nonossifying Fibroma
bland fibrous tissue. The lesions are usually asymptomatic
Age at presentation: 1st or 2nd decade.
and are diagnosed either coincidentally on a radiograph
Location: Eccentric metaphyis.
obtained following an injury or at the time of pathologic
Number: solitary.
fracture [1, 2]. They are usually diagnosed in the first two
Presenting symptoms: usually none, occasional fracture.
decades of life [3].
Rx: none unless large.
Nonossifying fibromas appear as lytic lesions that are
Malignant degeneration: none.
eccentric in the metaphyseal regions of growing bones
(Fig. 20.1a). They may appear lobulated, have distinct scle-
rotic margins, and do not create periosteal reaction. They are
contained within the bone and do not typically expand the
bone but do erode the cortex from within. The appearance Fibrous Dysplasia
within the lesion is lytic, without spicules of calcification.
Fibrous dysplasia is a noninherited condition defined by the
replacement of normal trabecular bone by fibrous tissue and
L. Karol, MD (*)
Department of orthopedic Surgery, Texas Scottish Rite Hospital,
immature disorganized osteoid [8]. When monostotic, the
2222 Welborn, Dallas, TX 75219, USA condition shows no gender predilection. Fibrous dysplasia
e-mail: [email protected] may be diagnosed in childhood, adolescence, or adulthood.

334 L. Karol
Fig. 20.1 (a) Radiograph of the

ankle of a 7-year-old boy with
ankle pain. Note the eccentric
lobulated contained lesion which
thins the surrounding cortex.
Biopsy at the time of surgery
confirmed the diagnosis of a
nonossifying fibroma. (b)
Radiograph 8 years following
curettage and bone graft shows
resolution of the lesion. The boy
is asymptomatic and participates
in contact athletics
Lesions may be present in one bone, known as monostotic

fibrous dysplasia, or in many bones, known as polyostotic
fibrous dysplasia. When associated with endocrine abnor-
malities such as precocious puberty, the diagnosis of McCune
Albright syndrome is made. Mazabraud’s syndrome is the
association of fibrous dysplasia with muscular myxomas and
is even more rare. Polyostotic fibrous dysplasia is more com-
mon in girls than in boys, especially when associated with
endocrinopathy.
Fibrous dysplasia and McCune Albright syndrome have
been found to result from a somatic mutation in the guanine
nucleotide-binding protein, Alpha-stimulating activity poly-
peptide gene, known more succinctly as the Gnas pathway
[9, 10]. Because this is a somatic mutation, the disease is not
hereditary. This mutation is specific to fibrous dysplasia and
is not seen in other lytic processes of bone.
Presenting symptoms of fibrous dysplasia include limp,
pathologic fracture, or bone pain [11]. Patients may present
with angular deformity less frequently. Those children with
McCune Albright syndrome present with precocious puberty,
which usually manifests as painless vaginal bleeding in young
girls. They also have characteristic café-au-lait spots on their
skin, present from birth or infancy, with irregular borders
described as “coast of Maine” (Fig. 20.2). Other endocrinop-
athies associated with McCune Albright syndrome include Fig. 20.2 Clinical photograph of a girl with McCune Albright syn-
hyperthyroidism, growth hormone excess, and Cushing’s drome. Note the skin macule which is hyperpigmented and has irregular
disease [12, 13]. borders. The child has spinal involvement and scoliosis
The radiographic appearance of fibrous dysplasia is one of
one or more ill-defined lytic lesions. The disease may be pres-
ent in the metaphysis or diaphysis of the bone, but is typically within the lesions is blurred, opaque, and gray on radiographs.
not epiphyseal. The margins of the lesions are somewhat This has been termed “ground glass” appearance. The lesions
indistinct, lacking the sclerotic rim frequently present in can be expansile and may thin the cortices of the native bone.
patients with simple bone cysts. Owing to the lack of mar- They do not create a periosteal reaction (Fig. 20.3). Large
gins, the lesions of fibrous dysplasia are described as “flame- lesions in weight-bearing bones may result in bowing of the
shaped,” tapering off into the normal bone. As the bony bone, with coxa vara of the femur and anterior bowing of
trabeculae are replaced by fibrous tissue, the appearance the tibia being most common.
20 Lower Extremity Benign Bone Lesions and Related Conditions 335
with limited cellularity. Islands of immature osteoid are

present, but do not form mature trabeculae. These spicules of
immature bone take on abnormal irregular shapes, which
have been termed “Chinese Letters.” The islands of osteoid
are not rimmed in osteoblasts, in distinction to osteofibrous
dysplasia. Giant cells and osteoclasts may be scattered in the
microscopic field.
The orthopedic management of fibrous dysplasia is aimed
towards correcting deformity and the treatment of pathologic
fractures. The natural history of fibrous dysplasia in the skel-
etally immature patient is that of slow expansion of the bony
lesions. Deformity of weight-bearing bones appears as the
mechanical strength of the bone is lost due to progressive
replacement with fibrous tissue. In polyostotic disease, gait
becomes more difficult, with the median age at which
patients require assistive devices to walk being 7 years [14].
Characteristic deformities include coxa vara due to the
“shepherd’s crook” deformity of the femur, angular defor-
mity at the knee, and procurvatum of the tibiae. Pain in the
Fig. 20.3 Anteroposterior radiograph of a 3-year-old female with extremity may occur as the bone weakens and deforms sec-
polyostotic fibrous dysplasia. Poorly defined lytic areas are present ondary to microfractures that occur due to the stress of
throughout both femora and the right hemipelvis. The right femoral weight bearing. Limb length discrepancy may develop.
neck is expanded, and an early shepherd’s crook deformity is present.
Bony lesions do not require operative intervention unless
Intramedullary fixation is present due to previous pathologic fracture
associated with pathologic fracture or deformity. Curettage
and bone grafting of fibrous dysplasia leads to recurrence of
The most common sites for fibrous dysplasia are within the lesions, and the bone graft (allograft or autograft) is even-
the long bones, but any bone may be involved. Vertebral tually replaced again by the fibrous tissue characteristic of
involvement is seen less frequently. The skull may be fibrous dysplasia. Cortical bone grafts have been used, with
affected, which can lead to disfigurement over time. hopes of less resorption and greater improvement of mechan-
The differential diagnosis in patients with monostotic ical strength of the bone [15]. Unfortunately, these grafts
fibrous dysplasia includes simple and aneurysmal bone cysts, also resorb, although in a more delayed fashion compared to
eosinophilic granuloma, and when the tibia is involved, morselized bone graft.
osteofibrous dysplasia. Lesions due to fibrous dysplasia can Pathological fractures occur most frequently in the first
usually be differentiated from bone cysts as the margins of decade of life between the ages of 6 and 10 years [16]. The
the lesion are less distinct, there is no fallen leaf sign (seen in femur is the most common bone to sustain a fracture.
patients with simple cysts following pathologic fractures), Fractures occurring through dysplastic bone may be treated
and the matrix radiographically is more opaque in fibrous nonoperatively in the upper extremity, but usually benefit
dysplasia. Upon presentation, it has been recommended to from surgical treatment in the lower extremities. Load-
obtain a technetium bone scan to identify the presence of sharing intramedullary implants allow earlier weight bear-
polyostotic disease. By the age of 6 years, it has been found ing, which is important due to the underlying bone fragility
that all affected sites can be identified on bone scan [11]. Due (Fig. 20.4) [17–19]. Deformity should be fully corrected, as
to the lack of need for treatment of asymptomatic lesions, it residual angulation will predispose to future fractures.
has not been the practice of my institution to obtain a bone The shepherd’s crook deformity of the proximal femur is
scan for identification of all bony lesions. particularly challenging to treat [15, 20]. Progressive coxa
The diagnosis is usually made both clinically and radio- vara leads to a waddling gait, hip pain, and eventual patho-
graphically (especially in the polyostotic form); therefore logic stress fractures when the neck-shaft angle is less than
biopsy is only rarely required. When biopsy tissue is 120°. Proximal femoral valgus osteotomy can correct the
obtained, firstly, the lesion in fibrous dysplasia is filled with deformity, but fixation in the dysplastic bone is extremely
tissue rather than fluid on aspiration, differentiating it from challenging. Plate fixation frequently leads to pathologic
bone cysts. On gross examination, tissue from fibrous dys- fracture at the ends of the plate where stress concentration
plasia lesions appears firm and gray. There may be areas that occurs or to loss of fixation due to screw pullout. Hip screw
appear cystic. The specimen will feel gritty to palpation. and side plate fixation is typically performed; however the
Microscopic examination shows the abundant fibrous tissue screw over time may migrate out of the femoral neck as the
336 L. Karol
Fig. 20.5 Pelvis radiograph of a 21-year-old male who has undergone

multiple surgeries for treatment of right femoral fractures and coxa
vara. Note the expansion of the femoral neck, the lytic appearance of
the femoral neck and head, and the superior migration of the implant in
the femoral neck. There is shortening of the length of the limb on the
right
recurrent angulation, and increasing bone pain due to bone

Fig. 20.4 Anteroposterior radiograph of the lower extremities of an fragility (Fig. 20.6a, b).
11-year-old girl with McCune Albright syndrome. She has undergone Nonunion is infrequent in fibrous dysplasia. Bony callous
intramedullary fixation of the femora and tibiae as treatment of repeti-
tive pathologic fractures. Note the proximal femoral fixation for treat- can develop, but the bone softens with time due to replace-
ment of coxa vara and shepherd’s crook deformities. There is also a leg ment with fibrous tissue.
length discrepancy Limb length discrepancy is seen most frequently in chil-
dren with severe polyostotic disease. Lengthening is usually
fraught with complications due to the poor mechanical prop-
dysplastic bone remodels and the deformity recurs. Our pref- erties of the dysplastic bone. Contralateral epiphysiodesis
erence is to overlap a hip screw and side plate device with an may be offered, but is often rejected by some families due to
intramedullary flexible nail, with the goal of achieving rea- the patient’s underlying short stature.
sonable proximal femoral fixation to allow the osteotomy to Scoliosis is seen with greater frequency in patients with
heal, yet providing load-sharing distal to the device by virtue fibrous dysplasia [21, 22]. Vertebral involvement with scoli-
of the intramedullary nail. Customized proximal femoral osis has been found in one-third to over 50 % of patients with
nail-screw devices have been used as well, but again the polyostotic disease. As would be expected, spinal fusion sur-
proximal femur typically remodels despite the presence of gery may be difficult due to the bony fragility and excessive
the implant (Fig. 20.5). bleeding. Yet successful results of posterior spinal fusion
Osteotomies for deformity correction require meticulous have been published [22]. The efficacy of scoliosis bracing
preoperative planning. The deformities are typically multi- in fibrous dysplasia has not been proven, and the concurrent
planar, and more than one osteotomy may be required for presence of rib lesions precludes its use in most patients.
passage of intramedullary devices. There is frequently no Medical treatment with bisphosphonate agents has been
patent intramedullary canal in the dysplastic bone, so inser- used in patients with polyostotic fibrous dysplasia [23–26].
tion of flexible or rigid nails must be undertaken carefully to Bisphosphonate use has not been shown to affect the bony
avoid penetration with the nail or further pathologic fracture. lesions in fibrous dysplasia, but has been found to decrease
The intramedullary canal may require drilling to allow nail the pain patients experience. New clinical studies using
passage. Additionally, large amount of blood loss should be receptor activator of nuclear factor kappa-B ligand (RANKL)
anticipated and prepared for, as these lesions are hypervascu- inhibitors have shown promise in influencing the progression
lar. Unfortunately, skeletal growth leads to recurrent defor- of bone disease [27]. Patients with McCune Albright syndrome
mity, and revision surgery should be expected. Progression require referral at the time of diagnosis to pediatric endocrino-
of the fibrous dysplasia can lead to enlargement of the bone, logists for medical management [28, 29]. Those children with
Fig. 20.6 (a) AP radiograph of

the lower extremities of a
9-year-old girl with polyostotic
fibrous dysplasia. She has
undergone a right proximal
femoral allograft replacement
due to marked bony enlargement
suspicious for malignancy, a left
proximal femoral osteotomy, and
right tibial osteotomy with
intramedullary fixation. (b) Two
years later, the tibia has
undergone great expansion and
angular deformity has worsened
with recurrent bone pain with
weight bearing
skull involvement should be referred as well to craniofacial

specialists, as bony enlargement and overgrowth can lead to Osteofibrous Dysplasia
cosmetic disfigurement and optic foraminal sclerosis may
progress to blindness. Osteofibrous dysplasia, also known as Campanacci’s disease,
The development of cystic lesions most closely resem- is a peculiar bone dysplasia which is typified by both sclerotic
bling aneurysmal bone cysts may occur within the dysplastic and lytic lesions most commonly present in the tibia in chil-
bone. Rapid enlargement of the lesions coupled with increas- dren and adolescents [32, 33]. It is usually not genetically
ing pain is usually present. Malignant degeneration has been transmitted. Osteofibrous dysplasia is bimodal in presenta-
documented in rare patients with fibrous dysplasia [30, 31]. tion. It can present in early infancy with apparent anterior
Malignant degeneration is most common in adulthood and bowing in the tibia [34, 35]. In such cases, it must be distin-
has been associated with previous irradiation, which is now guished from anterolateral bow of the tibia as associated with
contraindicated in patients with fibrous dysplasia. Painful congenital pseudarthrosis of the tibia and neurofibromatosis.
enlargement of the dysplastic bone may also be present. The fibula in patients with infantile osteofibrous dysplasia
In such instances, evaluation by an orthopedic oncologist is usually uninvolved, and pathologic fracture is rare. The
should be sought. condition is nearly always unilateral and does not involve the
rest of the skeleton.
The second form of osteofibrous dysplasia presents in late
Box 20.2. Fibrous Dysplasia childhood or adolescence. It also is most common in the tibia
Age at presentation: 1st or 2nd decade. and involves only one bone. It has been genetically linked to
Location: Long bones most frequent, metaphyis or adamantinoma.
diaphysis. Osteofibrous dysplasia is not typically genetically trans-
Number: solitary or polyostotic. mitted, but a rare form has been published in which several
Presenting symptoms: limp, pathologic fracture, bone family members were affected [36, 37].
pain. Radiographs of osteofibrous dysplasia show bubbly lytic
Rx: intramedullary fixation of fractures, deformity lesions scattered throughout the anterior diaphysis of the tibia
correction, bisphosphonates for pain. [38]. There is usually anterior bowing, and the limb may be
Malignant degeneration: rare in adulthood. somewhat shorter than the contralateral leg. The cortex may
appear thickened in areas (Fig. 20.7).
338 L. Karol
shown to lead to recurrence and is not recommended. Those

surgeons who have concern that osteofibrous dysplasia may
evolve into malignant adamantinoma have performed
aggressive resection of the affected tibial area, with recon-
struction as needed. Bone segment transport with proximal
corticotomy and external fixation and treatment with free
vascularized fibular graft have met with success in very rare
instances [45, 46].
Box 20.3. Osteofibrous Dysplasia

Age at presentation: infantile or 1st or 2nd decade.
Location: anterior tibial diaphysis.
Number: solitary.
Presenting symptoms: bowing, enlargement, pain.
Rx: controversial, possible en bloc excision.
Malignant degeneration: associated with adamantinoma.
Enchondromatosis
Fig. 20.7 AP and lateral radiograph of a 7-year-old female with pro- Enchondromas are benign hyaline cartilaginous lesions
gressive anterior bowing of the tibia. The lateral view shows loculated present within the metaphysis. Enchondromas may be soli-
lytic lesions in the anterior cortex of the tibia typical for osteofibrous tary or multiple, in which case the condition is named Ollier’s
dysplasia
disease. Solitary enchondromas are common and rarely
symptomatic, where Ollier’s disease is rare. Maffucci’s syn-
The differential diagnosis of osteofibrous dysplasia drome is defined as the presence of multiple enchondromas
includes monostotic fibrous dysplasia, congenital pseudar- with soft tissue hemangiomatosis and carries a much bleaker
throsis of the tibia, and adamantinoma. prognosis. Enchondromatosis is not genetically transmitted
Pathological examination of tissue from osteofibrous dys- but may be the result of a somatic mosaic mutation in isoci-
plasia shows fibrous tissue with areas of immature osteoid, trate dehydrogenase 1 or 2 [47]. The incidence of Ollier’s
as is seen in fibrous dysplasia. The histological distinction disease is estimated at 1 per 100,000.
from fibrous dysplasia is that the osteoid is rimmed in osteo- Presenting complaints in multiple enchondromatosis are
blasts in osteofibrous dysplasia, which is its characteristic usually bone pain due to mechanical insufficiency, patho-
histologic finding. Cells stain positively for cytokeratin, logic fracture through the lesions, angular deformity at the
which is also present in adamantinoma, supporting a rela- knee or fingers, or limb length discrepancy [48]. The condi-
tionship between the two lesions [38–42]. tion typically presents in the first or second decade of life.
Treatment of osteofibrous dysplasia is controversial. The radiographic appearance of multiple enchondromato-
Spontaneous resolution of osteofibrous dysplasia in young sis is quite distinct (Fig. 20.8). Streaky lytic lesions are seen
babies has been described, so treatment in these infants in the metaphysis of the bones. At the knee, the elongated
should be observation in the absence of pathologic fracture areas of enchondroma give a “fanlike” appearance to the
[43]. If fracture does occur, intramedullary fixation such as is metaphysis. There are areas of fine calcification within the
performed in congenital pseudarthrosis of the tibia, com- lesions due to the presence of calcification in the cartilagi-
bined with bone grafting the fracture site, usually is success- nous lesions. The lesions seem to stop at the physis. It is
ful in obtaining and maintaining union [44]. common that multiple enchondromas are seen, and that they
Older children with osteofibrous dysplasia have under- tend to present unilaterally. The cortex of the bone is intact
gone various forms of treatment. Observation of radiograph- in the absence of pathologic fracture. Angular deformity at
ically stable lesions may be chosen when the patient is the knee and limb length discrepancy can be appreciated
asymptomatic. Marginal resection with curettage has been (Fig. 20.9) [49].
Biopsy reveals a pearly white to bluish-white specimen.

Microscopic examination shows bland cartilaginous tissue,
with nests of plump chondrocytes. The nuclei are uniform,
and mitotic figures may be seen in children but should be few
in number. “Chicken-wire” calcification, which are areas of
punctate calcification within the lesion, can be appreciated,
which renders the specimen slightly gritty.
Treatment of children with solitary enchondromas is mer-
ited if there is angular deformity. Small asymptomatic lesions
in the lower extremities may be observed. Multiple enchon-
dromatosis presents for orthopedic treatment more fre-
quently. Curettage and bone grafting of the enchondromas is
in large part ineffective due to the extensive nature of the
disease, but may be of some benefit in the hand. Children
with Ollier’s disease have significant angular deformities,
with a predisposition towards distal femoral varus [49]. The
angular deformity is usually accompanied by varying
amounts of limb shortening which may measure up to 25 cm
at maturity (Fig. 20.10) [48]. In the lower extremity, angular
correction with or without lengthening may be undertaken.
Standard fixation with internal devices such as plates and
screws may be compromised by poor bony integrity due to
the large cartilaginous lesions. Gradual deformity correction
using external fixation devices with fine wire bony fixation
Fig. 20.8 Radiographic appearance of enchondromatosis of the distal
has been successful [50–54]. When pins or wires are inserted
femur and proximal tibia in a 7-year-old male. Note the eccentric loca- intralesionally, fixation can usually be maintained; however
tion of the metaphyseal femoral lesion, and the presence of intralesional it should be expected that the regenerate will contain enchon-
calcification which is typical of cartilage tumors. The right proximal dromatosis tissue (Fig. 20.11a, b) [53]. Lengthening with
fibula is also involved
intramedullary devices has also been reported [55].
Fig. 20.9 (a) Asymmetric genu

valgum and limb length
discrepancy in a young boy with
Ollier’s disease. (b) A close-up of
the left knee shows the streaky
lytic lesions characteristic of
enchondromatosis
340 L. Karol
Malignant transformation has been described in enchon-

dromatosis in up to 20–30 % of patients by the age of 40.
Patients with significant expansion of a lesion or with
increasing pain should be suspected. Chondrosarcoma is the
most frequent malignant tumor seen in up to 25 to 30 % of
adult patients with enchondromatosis [56, 57]. Histologic
overlap between benign enchondromas seen in Ollier’s
disease and low-grade chondrosarcoma makes the diagnosis
of malignant degeneration challenging. Remote non-bony
malignancies are associated with Maffucci’s syndrome,
which carries a nearly 100 % incidence of the development
of a malignant tumor [58]. These children must be carefully
monitored throughout their lifetime for the appearance of
cancerous lesions.
Box 20.4. Enchondroma

Age at presentation: 1st or 2nd decade.
Location: metaphyis, predisposition to asymmetry.
Number: solitary or multiple (Ollier’s disease).
Presenting symptoms: bone pain, pathologic fracture,
angular deformity, limb length discrepancy.
Rx: osteotomy for deformity correction, lengthening,
Fig. 20.10 Severe limb length discrepancy measuring greater than in hand may curettage and bone graft.
15 cm and varus deformity of the left distal femur in 7-year-old male
with Ollier’s disease. Note the presence of enchondromas in bilateral
Malignant degeneration: chondrosarcoma in 25–30 %
femora and tibias as well as the pelvis. The extent of involvement is of Ollier’s disease.
asymmetric
Fig. 20.11 (a) Varus osteotomy

of the distal femur in patient with
Ollier’s disease. External fixation
with half-pins and fine wires was
successful in stabilizing the
osteotomy. (b) Standing
radiograph 1.5 years following
removal of the external fixator
shows satisfactory alignment but
a persistent limb length
discrepancy
Solitary Osteochondromas and Multiple

Hereditary Exostoses
An osteochondroma is a common benign bone growth which

protrudes from the surface of the native bone and is capped
with cartilage. Osteochondromas occur in the metaphyseal
area and have been hypothesized to be the result of seques-
tration of a fragment of physis which causes the outgrowth.
The bone of an osteochondroma is normal bone and is in
continuity with the host bone. Osteochondromas may occur
in any bone, including the pelvis, spine, and fingers, but are
most common in the distal femur, proximal tibia, and proxi-
mal humerus [59].
Multiple hereditary osteochondromatosis or exostoses
(MHE) are an inherited condition typified by the presence of
multiple osteochondromas scattered throughout the skeleton.
It is genetically transmitted as an autosomal dominant trait
with very high (96 %) penetrance but variable phenotypic
expression. The number and location of osteochondromas can
vary among family members. The tumor suppressor genes
EXT1, EXT2, and EXT3 for MHE have been localized on
chromosomes 8, 11, and 19 [60–62]. Patients with MHE have
a loss of function of these genes, thereby allowing growth of
the osteochondromas. Patients with EXT1 mutations gener-
Fig. 20.12 Radiograph of a 10-year-old boy with multiple hereditary
ally have more severe phenotypical expression and shorter exostoses. Note the numerous sessile lesions, such as that seen at the
stature than those with EXT2 mutations [63–67]. It is believed distal tibia, and the pedunculated lesions as seen at the distal femur and
that patients with solitary osteochondromas have somatic, but proximal tibia. Ankle valgus is present at the left ankle, and an intraos-
not generalized, mutations in the EXT gene. The incidence of seous osteochondroma from the right tibia deforms and thins the fibula
multiple osteochondromas is 1 per 50,000. Ninety percent of
patients with MHE have a positive family history and demon- Osteochondromas are not epiphyseal. The cortex of the
strable mutations in the EXT 1 or 2 genes [68]. osteochondroma is in continuity with the native bone, and
Patients with a single osteochondroma often present in the the trabeculae within the osteochondroma are similarly in
first or second decade for evaluation of a painless periarticular continuity with the metaphysis. Osteochondromas may be
mass. Patients with MHE usually present in early childhood pedunculated, with a distinct bony stalk, or sessile, where the
with multiple small masses. The median age of presentation in lesion is broad-based. When pedunculated, osteochondro-
MHE is 3 years [69]. The presence of a positive family history mas typically point away from the joint. Osteochondromas
generally created a heightened awareness of the condition in may vary in size. Large osteochondromas in two-bone seg-
the parents and therefore an earlier presentation. Patients may ments, such as the lower leg, can cause deformity of the
complain of pain if there has been trauma in the area of the smaller bone, in this case the fibula. Osteochondromas in the
osteochondroma, or if the mass is sufficiently large to create distal femur, proximal tibia, and/or proximal fibula are seen
symptoms due to impingement of overlying muscle or ten- in 94 % of children with MHE. Proximal humerus masses
dons. In many cases, the patient is asymptomatic, but the are present in 50 % of cases, the scapula and ribs in 40 %, the
lesion has been identified coincidentally on a radiograph distal radius and ulna in 30 %, the proximal femur in 30 %,
obtained for another reason. Patients with MHE tend to be of the phalanges in 30 %, the distal fibula in 25 %, and the distal
mildly short stature. Stature less than the 25th percentile was tibia in 20 % of MHE patients [69].
seen in 58 % of patients with MHE [66]. Additionally, the The radiographic appearance of an osteochondroma is
extremities will appear shorter than the trunk does, with the sufficiently distinct that biopsy is not needed. When osteo-
amount of shortening being linked to the size of osteochondro- chondromas are excised for relief of symptoms, pathologic
mas present in the limb segment [70]. Angular deformity, such evaluation of the mass should be obtained. The bone of an
as genu valgum and ankle valgus, may be present. osteochondroma is histologically normal bone. The junction
Radiographs show a bony protrusion in the periarticular of the bone and the cartilage cap shows endochondral ossifi-
area originating from the metaphyseal bone (Fig. 20.12). cation. It is important to evaluate the cartilaginous cap of the
342 L. Karol
Fig. 20.13 (a) Valgus deformity

in an 8-year-old male with
multiple hereditary exostoses. (b)
Radiograph 4 years following
growth modulation shows
correction of the valgus
deformity. Note the medial
malleolar screw placed for
treatment of ankle valgus
lesion. In a benign osteochondroma, the cartilage cap is thin of larger lesions prior to surgery may be helpful in identify-
(<2 cm) and the chondrocytes histologically bland, without ing the location of neurovascular structures that may be dis-
mitotic figures or nuclear pleomorphism. In some instances, placed by the osteochondromas. In particular, the peroneal
osteochondromas can undergo malignant transformation into nerve is frequently thinned or displaced by proximal fibular
low-grade chondrosarcomas. The incidence of malignant lesions, and should be identified and protected if the proxi-
transformation is less than 1 % and is more likely in mature mal fibular osteochondroma is to be excised. Even so, post-
patients and those with pelvic lesions. Yet an increase in pain operative peroneal nerve palsy frequently results from
and rapid increase in size should merit excisional biopsy. excision of fibular lesions [75, 76], and preoperative coun-
The cartilage cap in an osteochondroma that has become selling of the patient and family about the possibility of a
malignant is thickened, and the nuclei of the cartilaginous foot drop necessitating use of an ankle-foot orthosis should
region pleomorphic. It is the combination of the histological be performed. Additionally, venous thromboembolism fol-
appearance of the cartilage cap combined with its thickness lowing excision of lesions behind the knee has been seen in
that leads to the diagnosis of chondrosarcoma [71]. rare cases [77].
Treatment of a solitary osteochondroma is excision if Angular deformity of the lower extremities may be pres-
the lesion is causing pain or mechanical symptoms due to ent in patients with MHE. Growth modulation in skeletally
its size. Recurrence is unlikely with complete excision. immature patients may be quite useful, especially at the
Patients with MHE should be offered excision of symptom- knee. When preoperative planning growth modulation, con-
atic lesions, and observation if the masses are asymptom- sideration for inaccuracies in bone age should be made [78].
atic. Due to the number of osteochondromas that many of Most often, the knee assumes a valgus position due to proxi-
these children have, excision of all the lesions is impracti- mal tibial involvement [70]. Patellar instability may be asso-
cal and unnecessary; however recent validated outcome ciated with genu valgum in some children [79]. Medial
questionnaires have highlighted the complaints of muscu- proximal tibial and distal femoral growth modulation plating
loskeletal pain in children with MHE [72–74]. Masses that can be useful (Fig. 20.13a, b). Skeletally mature patients
are creating secondary deformities, such as large distal may rarely require osteotomy. In such cases, the peroneal
tibial lesions that are producing fibular deformity (see nerve is prone to postoperative palsy, so fibular osteotomy
Fig. 20.12), may benefit from excision. Advanced imaging should be approached with great caution.
Ankle valgus is also frequently present in patients with

MHE due to distal tibial and fibular lesions [80]. Box 20.5. Osteochondroma
Radiographs show relative shortening of the distal fibula Age at presentation: 1st or 2nd decade.
and distal tibial valgus tilt. Gradual deformity correction Location: metaphyseal.
via insertion of a staple or fully threaded medial malleolar Number: solitary or multiple.
screw in skeletally immature patients has been shown to be Presenting symptoms: painless mass, mechanical
of some utility [81, 82], but this author finds that correction symptoms.
of the ankle is frequently very slow and often incomplete. Rx: excision of symptomatic lesions.
Rarely is the ankle deformity sufficiently symptomatic, Malignant degeneration: chondrosarcoma in ≤1 %.
however, to merit osteotomy. Other children with MHE Under treatment: correction of angular deformity.
may complain of ankle deformity due to large distal tibial
osteochondromae which exert pressure on the distal fibula,
leading to erosions and bowing of the fibula. Excision of
the osteochondroma can relieve symptoms, and the fibula Dysplasia Epiphysealis Hemimelica
will most likely remodel following excision of the tibial (Trevor’s Disease)
mass. A long-term study of adults with ankle valgus sec-
ondary to osteochondromas linked persistent excessive Dysplasia epiphysealis hemimelica is a condition where
ankle valgus with limitations in recreational activities and intra-articular osteocartilaginous lesions develop, originat-
with the development of arthritis [83]. ing from the epiphysis in skeletally immature children.
Progressive hip dysplasia due to the presence of proximal Lesions are solitary in one-third of patients and multiple in
femoral osteochondromas has been seen in a minority of two-thirds. Lesions in those patients with multiple epiphy-
patients with MHE [84, 85]. Surgical reconstruction via seal involvement tend to be unilateral and to involve only one
excision of the osteochondromas combined with proximal side (i.e. either the medial or lateral) of the epiphyses
femoral osteotomy as needed for containment can be useful. (Fig. 20.14). The disease is not genetically inherited but is
There have been published studies which have raised more common in boys than girls [93, 94].
awareness of the presence of spinal osteochondromas in Patients present either in the first or second decade of
patients with MHE [86, 87]. The incidence of clinically life with limited range of motion of involved joints which
important spinal osteochondromas remains quite rare, but it lead to gait disturbances. The most common sites are the
is imperative that the lower extremity surgeon be aware of distal femur, distal tibia, talus, and navicular. Lower
their occurrence. The patient who presents with numbness or extremity involvement is more common than upper extrem-
tingling in the lower extremity, or with difficulty with ambu- ity involvement. When the condition affects the distal tibia
lation, should have a careful neurologic assessment. It is or talus, the presentation is usually of fixed equinus, while
enticing to explain the symptoms by the presence of osteo- at the knee, a flexion position is adopted. Range of motion
chondromas either in the popliteal fossa or at the proximal may be somewhat painful owing to the presence of the
fibula, but consideration for a spinal etiology of the symp- intra-articular mass. When present in the hip, the lesions
toms should be given. Spinal osteochondromas are readily are typically from the acetabulum and can lead to sublux-
seen via either CT or MRI scanning. Excision of a space- ation if sufficiently large.
occupying lesion in the spinal canal should be offered. Radiographs may initially be normal, as the cartilaginous
As mentioned above, malignant transformation of an masses in the very young child are difficult to detect. As the
osteochondroma may occur in up to 1–2 % of patients [71, child grows older, the lesions resemble those of osteochon-
88–90]. Warning signs include a crescendo of pain, pain at dromas, but the masses originate from the epiphysis and
rest, and unusually rapid growth of a lesion, particularly in a point towards the joint rather than away. Small lesions may
skeletally mature patient. Lesions in the pelvis and shoulder be difficult to fully appreciate on plain radiographs, but MRI
girdle are most prone to malignant degeneration. Preoperative shows the cartilaginous extent of the lesion [95].
imaging of the cartilaginous cap with MRI can support the Histologically, lesions are made up of normal bone and
suspicion of malignancy if the cap is greater than 2 cm in varying amounts of overlying cartilage. The cells in both the
maximal thickness. Referral to an orthopedic oncological bone and cartilage are benign in appearance and mitotic fig-
surgeon is merited in such cases. Treatment is typically wide ures are not seen.
surgical resection. The intra-articular lesions grow in the skeletally immature
Finally, there are several case reports of popliteal artery patient. Treatment consists of excision of symptomatic
pseudoaneurysms in association with large osteochondromas lesions [96]. Unfortunately, due to the intra-articular nature
in the posterior knee area. In such cases, vascular repair is of the masses, complete excision is difficult. While MRI may
needed in conjunction with excision of the mass [91, 92]. show delineation between the lesion and the native epiphysis,
344 L. Karol
Fig. 20.14 Dysplasia

hemimelica epiphysealis
(Trevor’s disease) affecting the
distal fibula and lateral midfoot.
Note the enlargement of the
distal fibular epiphysis and
intra-articular exostoses
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Management of Juxtaphyseal
Malignant Bone Tumors Around 21
the Knee Joint: New Concepts
in Limb-Sparing Surgery
Hidenori Matsubara and Hiroyuki Tsuchiya
(Table 21.1, Fig. 21.1) [9]. Cases indicated for the DO

Introduction method should have tumors of Types I to IV according to
our classification system, of no more than a 15-cm defect
There are various kinds of malignant bone tumors in pediat- after the tumor resection (resulting in a treatment time of
rics. Osteosarcoma is the most common primary malignant less than 1 year), and with a good response to chemother-
bone tumor in children. Prior to the 1970s, amputation or joint apy. Theoretically, the DO method can be applied even in
disarticulations were the standard treatment and the only cases with Types V and VI tumors combined with arthrod-
choice of treatment. However, now over 90 % of osteosar- esis. Currently, however, it has been replaced with the LN
coma resections are limb-sparing surgeries due to advance- method. The LN method can be indicated in all cases. For
ments in chemotherapy, medical imaging, and implant chemotherapy responders, this enables preservation of the
technology [1–3]. Options for limb salvage reconstruction maximum amount of healthy tissue, such as soft tissue,
after wide resection include osteoarticular allografts, allograft growth plates, and joint surfaces, to provide excellent limb
prosthetic composites, recycled autograft, and modular or function [3].
custom-made endoprostheses. However, the limb function
often remains limited and deteriorates over time. Complications
such as infections, nonunion of grafts, and bone resorption Surgical Techniques
could eventually lead to amputation. Therefore, the aim of
biological reconstruction is to achieve better limbs and almost Type I: DO Method (Fig. 21.2)
normal limb function, and to reduce complications.
We first performed transepiphyseal osteotomy and In Type I, the tumor is diaphyseally or metaphyseally located
resected a malignant bone tumor followed by reconstruction over 2 cm away from the epiphyseal plate. The operative
using the distraction osteogenesis technique (DO method) technique of the DO method consists of en bloc tumor exci-
[4–6] and massive frozen tumor-bearing bone treated with sion with preservation of the epiphyseal plate and articular
liquid nitrogen (LN method) [7, 8]. In this chapter, we intro- surface. An external fixator is applied and osteotomy is per-
duce our two original biological reconstruction procedures formed for postoperative bone transport. After docking bone
for malignant tumors. transport, we usually perform bone graft at the docking site.
Shortening-distraction is indicated if acute shortening is pos-
sible during the operation. Optional intramedullary nailing,
Indication if feasible, or plate conversion, is also used to shorten the
external fixation time [4, 6].
To facilitate decision making for reconstruction in cases of
juxta-articular malignant bone tumor around the knee joint,
we developed a new system for classifying tumor excision Type I: LN Method (Fig. 21.3)
One-site osteotomy and pedicle freezing method are applied

in the Type I LN method. After transdiaphyseal osteotomy,
H. Matsubara, MD, PD • H. Tsuchiya, MD, PD (*)
soft tissue is removed, and the tumor is curetted after thor-
Department of orthopedic Surgery, Kanazawa University Hospital,
13-1 Takara-machi, Kanazawa, Ishikawa 920-8641, Japan ough isolation to prevent fracture during freezing. Bony
e-mail: [email protected] lesions connected to the limb are then rotated and frozen in

350 H. Matsubara and H. Tsuchiya
Table 21.1 Decision making for reconstruction based on location and extent of tumor
Type I: Tumor at diaphyseal location or metaphyseal location over 2 cm from the epiphyseal plate
Type II: Tumor at metaphyseal location extending to less than half of the epiphyseal growth plate
Type III: Tumor at metaphyseal location extending to the whole growth plate
Type IV: Metaphyseal tumor extending through the growth plate into part of the epiphysis at least 10 mm from the articular surface
Type V: Tumor extending into less than half of the epiphysis less than 1 cm away from the articular surface
Type VI: Tumor extending into more than half of the epiphysis less than 1 cm away from the articular surface
Fig. 21.1 Classification of tumor excision. (a) Type I. (b) Type II. (c) Type III. (d) Type IV. (e) Type V. (f) Type VI
Fig. 21.2 Type I: DO method. (a) Resection line. (b) Tumor resection. (c) Application of external fixator and osteotomy for lengthening. (d) Bone
grafting at docking site. (e) After bone consolidation
21 Management of Juxtaphyseal Malignant Bone Tumors Around the Knee Joint… 351
Fig. 21.3 Type I: LN method.

(a) Resection line. (b) Pedicle
freezing. (c) Reconstruction with
double plates
Fig. 21.4 Type II: DO method. (a) Resection line. (b) Tumor resection. (c) Application of external fixator and osteotomy for lengthening.
(d) Bone grafting at docking site. (e) After bone consolidation
liquid nitrogen for 20 min, thawed at room temperature for after reconstruction. The operative technique of the DO
15 min, and then thawed in distilled water for 10 min. method consists of hemicortical en bloc tumor excision. An
Reconstructions are performed by osteosynthesis using dou- external fixator is applied, and proximal hemicortical oste-
ble plates [8]. otomy is performed for postoperative bone transport.
Epiphysiodesis of the other side of the growth plate is one
option to avoid future angular deformities for young
Type II: DO Method (Fig. 21.4) patients. After docking bone transport, we usually perform
a bone graft at the docking site. At that time, plate conver-
In Type II, the tumor is located metaphyseally and extends sion is optionally used to shorten the external fixation time.
to less than half of the epiphyseal growth plate. There is If a deformity occurs in the future, correction with or with-
also a possibility of joint deformity due to unequal growth out lengthening is performed.
Fig. 21.5 Type II: LN method.

(a) Resection line. (b) Freezing
tumor-bearing bone. (c)
Reconstruction with single plate
Fig. 21.6 Type III: DO method. (a) Resection line. (b) Tumor resection. (c) Application of external fixator and osteotomy for lengthening.
(d) Bone grafting at docking site. (e) After bone consolidation
Type II: LN Method (Fig. 21.5) Type III: DO Method (Fig. 21.6)
Hemicortical resection and a free-freezing method are In Type III, the tumor is metaphyseally located and extends
applied for Type II resections. After transepiphyseal and to the whole growth plate. Limb length discrepancies are
hemicortical resection, soft tissue is removed, and the tumor anticipated in immature patients, which may be managed
is curetted after thorough isolation. The tumor-bearing bone surgically if necessary. The operative technique of the DO
is frozen in liquid nitrogen and thawed as usual. method consists of transepiphyseal and intercalary en bloc
Reconstruction is performed by osteosynthesis using a tumor excision with the growth plate. Surgical technique is
single-locking plate [7]. basically same as in the Type I DO method.
Fig. 21.7 Type III: LN method.

Reconstruction with double
plates
Fig. 21.8 Type IV: DO method. (a) Resection line. (b) Tumor resection. (c) Application of external fixator and osteotomy for lengthening.
(d) Reconstruction of periarticular structure with diaphyseal bone cylinder. (e) After docking. (f) After bone consolidation
Type III: LN Method (Fig. 21.7) 10 mm away from the joint line. Limb length discrepancies
are anticipated in immature patients, which may be managed
Intercalary resection and the free-freezing method are surgically if necessary. The operative technique of the DO
applied for Type III resection. After transepiphyseal and method consists of transepiphyseal and intercalary en bloc
intercalary resection, free freezing is performed in the same tumor excision with the growth plate, and with preservation of
way as in the Type II LN method. Reconstruction is per- the articular surface. The periarticular structure should be sta-
formed by osteosynthesis using double plates. bilized immediately to prevent joint contracture. If only a thin
epiphysis or articular surface is preserved, a free bone cylinder
should be taken from the diaphysis and fixed to the remaining
Type IV: DO Method (Fig. 21.8) epiphysis with a bone graft and a spike washer and screw, in
order to stabilize and reconstruct the periarticular structure.
In Type IV, the tumor is metaphyseally located and extends to The newly created diaphyseal defect is restored by bone trans-
the whole growth plate into part of the epiphysis at least port. If feasible, optional plate conversion is also used.
Fig. 21.9 Type IV: LN method.

plates and spike wisher
Fig. 21.10 Type V: LN method.

plates and spike wisher
Type IV: LN Method (Fig. 21.9) Type V: LN Method (Fig. 21.10)
Intercalary resection and free-freezing method are applied In Type V, the tumor extends into less than half of the epiph-
for Type IV resection. After transepiphyseal and intercalary ysis. There is a possibility of joint deformity due to unequal
resection, the tumor-bearing bone is frozen and thawed in the growth after reconstruction. The operative technique of the
same way as above. Reconstruction is performed with dou- LN method consists of hemicortical resection with the osteo-
ble plates. When the ligament is detached from tumor- articular surface, and the free-freezing method is applied for
bearing bone, it is reconstructed with a spike washer and Type V. After resection of the hemicondyle, soft tissue is
screw. removed, and the tumor is curetted after thorough isolation.
Fig. 21.11 Type VI: LN method.

Reconstruction with surface
arthroplasty with or without a
locking plate and bone graft
The tumor-bearing bone is frozen in liquid nitrogen and response with significant ossification of the soft-tissue mass
thawed in the same way as above. Reconstructions are per- of the lateral tibia. We planned intercalary resection and
formed by osteosynthesis using single or double plates, a reconstruction with the DO method (bone transport). After
spike washer, and screws. Frozen osteoarticular grafts tend the operation, gradual lengthening was started and bone
to undergo osteoarthritis changes; therefore, an optional transporter docking was completed. As of the 7-year follow-
composite autograft indicated for Type VI is also applied to up, the patient is free of disease, and can run without any
avoid future operations. Otherwise, UKA or TKA is carried pain.
out for degenerative arthrosis.
Case 2 (Type IV: LN Method) (Fig. 21.13)

Type VI: LN Method (Fig. 21.11)
A 10-year-old boy presented with osteosarcoma of the distal
In Type VI, the tumor extends into more than half of the femur. Neoadjuvant caffeine-potentiated chemotherapy was
epiphysis. There is no possibility of preservation of the administered, and the clinical effect was a complete response
whole joint. There is also a possibility of joint deformity and with significant shrinkage of the soft-tissue mass of the
limb length discrepancy after reconstruction. The operative medial femur. We planned transepiphyseal and intercalary
technique of the LN method consists of en bloc resection resection and reconstruction with the LN method.
with the osteoarticular surface, and the free-freezing method Reconstruction was performed by osteosynthesis using
is applied for Type VI. Reconstruction is performed by a plates and cannulated screws. The resection margins were
combination of frozen grafts and surface arthroplasty with or tumor free, and pathological evaluation indicated grade III/
without a locking plate and bone graft. Strictly speaking, IV on the Rosen and Huvos evaluating system. As of the
these are partially biological reconstructions; however, they 22-month follow-up, the patient is free of disease, and can
can preserve bone stock. play football.
In summary, we introduced limb-salvage surgery for
reconstruction after tumor resection using distraction osteo-
Case Examples genesis and a frozen autograft method utilizing liquid nitro-
gen, according to the tumor site, size, and response to
Case 1 (Type I: DO Method) (Fig. 21.12) chemotherapy. The advantages of the DO method include
regeneration of living bone with sufficient strength and
A 17-year-old boy presented with osteosarcoma of the proxi- durability, biological affinity, resistance against infection,
mal tibia. Neoadjuvant caffeine-potentiated chemotherapy and lifelong restored function. The disadvantages include
was administered, and the clinical effect was a complete delayed union at the docking site and pin- or wire-tract
Fig. 21.12 Case (Type I: DO method). A 17-year-old boy with an intercalary osteotomies. (c) X-ray after the operation. (d) X-ray during
osteosarcoma of the proximal tibia. (a) Preoperative X-ray and MRI bone transport. (e) X-ray at the complication of docking of bone trans-
scan. Osteotomy line (broken lines). (b) After tumor resection with porter. (f) X-ray at 26 months after the operation
infection, as well as the fact that the procedure is time con- with bone graft combined with rigid fixation. There are two
suming (Table 21.2). The advantages of the LN method are important points in obtaining better functional results when
simplicity, osteoinduction, osteoconduction, a short treat- either method is selected: firstly, the surgeon should try to
ment time, a perfect fit, sufficient biomechanical strength, preserve the epiphysis if possible; and secondly, it should be
and easy attachment of tendons and ligaments. The disad- attempted to perform biological reconstruction as much as
vantage is basically dead bone at the time of reconstruction. possible.
Moreover, revitalization takes a long time, and degeneration In future, there may be greater use of biological recon-
of the preserved cartilage matrix occurs over time struction methods to obtain better and earlier functional
(Table 21.3). Possible complications with the DO method results, because a natural limb is the best option. To achieve
include pin-site infection managed with antibiotics or this, we have to aim to facilitate bone regeneration and revi-
removal of pins, docking-site nonunion or delayed union, talization much faster, and to adopt biological soft-tissue
and delayed consolidation of regenerated tissue treated with reconstruction, through advances in tissue engineering, such
bone graft. Complications with the LN method are late as with regard to osteogenic and angiogenic growth factors,
infection and nonunion of frozen bone, which are managed or with stem cell implantation.
Fig. 21.13 Case (Type IV: LN method). A 10-year-old boy with an (f) Osteosynthesis with plates and screws. (g) Radiograph 22 months
osteosarcoma of the distal femur. (a) Preoperative MRI scan. Osteotomy after reconstruction. Host-graft junctions achieved complete union.
line (arrows). (b) Intraoperative radiograph. Transepiphyseal and inter- (h) Twenty-two months after reconstruction, no extension lag. (i) The
calary osteotomy was performed. (c) After transepiphyseal and interca- range of motion of the knee was 0–140°
lary osteotomy. (d) Freezing in liquid nitrogen. (e) After freezing.
Table 21.2 Advantages and disadvantages of the DO method Table 21.3 Advantages and disadvantages of the LN method
Advantage Disadvantage Advantage Disadvantage
Living bone regeneration Lengthy external fixation Simple procedure Dead bone reconstruction
Sufficient strength Patient’s burden Reattachment of soft tissue (tendon Long time to be revitalized
Biological affinity Delayed consolidation and ligament)
Resistance against infection Delayed consolidation Osteoinduction and osteoconduction Cartilage degeneration over time
Lifelong restored function Delayed union of docking site Perfect fit Delayed union at host-graft
junction
Sufficient biomechanical strength Bone absorption
No contagion
No harmful denatured protein
References 5. Tsuchiya H, Tomita K, Shinokawa Y, Minematsu K, Katsuo S,

Taki J. The Ilizarov method in the management of giant-cell tumours
of the proximal tibia. J Bone Joint Surg Br. 1996;78(2):264–9.
1. Tomita K, Tsuchiya H. Intermediate results and functional
6. Tsuchiya H, Abdel-Wanis M, Kitano S, Sakurakichi K, Yamashiro
evaluation of limb-salvage surgery for osteosarcoma: an intergroup
T, Tomita K. The natural limb is best: joint preservation and recon-
study in Japan. J Surg Oncol. 1989;41(2):71–6.
struction by distraction osteogenesis for high-grade juxta-articular
2. Tsuchiya H, Tomita K. Prognosis of osteosarcoma treated by limb-
osteosarcomas. Anticancer Res. 2002;22(4):2373–6.
salvage surgery: the ten-year intergroup study in Japan. Jpn J Clin
7. Tsuchiya H, Wan SL, Sakayama K, Yamamoto N, Nishida H,
Oncol. 1992;22(5):347–53.
Tomita K. Reconstruction using an autograft containing tumour
3. Tsuchiya H, Tomita K, Mori Y, Asada N, Yamamoto N. Marginal
treated by liquid nitrogen. J Bone Joint Surg Br. 2005;87:218–25.
excision for osteosarcoma with caffeine assisted chemotherapy.
8. Tsuchiya H, Nishida H, Srisawat P, Shirai T, Hayashi K, Takeuchi
A, et al. Pedicle frozen autograft reconstruction in malignant bone
4. Tsuchiya H, Tomita K, Minematsu K, Mori Y, Asada N, Kitano S.
tumors. J Orthop Sci. 2010;15(3):340–9.
Limb salvage using distraction osteogenesis. A classification
9. Tsuchiya H, Abdel-Wanis ME, Tomita K. Biological reconstruction
of the technique. J Bone Joint Surg Br. 1997;79(3):
after excision of juxta-articular osteosarcoma around the knee: a
403–11.
new classification system. Anticancer Res. 2006;26(1B):447–53.
Part IV
Congenital and Developmental Disorders
Congenital Femoral Deficiency
Reconstruction and Lengthening 22
Surgery
Dror Paley, David Y. Chong, and Daniel E. Prince
Embryogenesis of the extremities occurs between 4 and

Introduction 8 weeks after fertilization, and the majority of congenital
anomalies occur during this period of time. Limb bud devel-
Demographics opment begins with the lateral migration of two layers of
mesoderm and outgrowth into the overlying ectoderm. Cells
The often-cited incidence of congenital femoral deficiency from the underlying somitic mesoderm ultimately form the
(CFD) is 1 in 52,029 based on a review of the Edinburgh muscle tissue of the limb, while cells from the lateral plate
Register of the Newborn by Rogala et al. published in 1974 mesoderm form cartilage and bones. Distinct but coordi-
[1]. In retrospect, this may not be accurate, as the incidence nated molecular pathways primarily control each axis. In
is based on the only case identified during the 4.5-year col- the process of limb patterning, mesenchymal cells in the
lection period from 1964 to 1968, notably, after thalidomide limb bud integrate positional information from the three
use was discontinued. This survey is also representative of a axes, indicating a complex interplay of the responsible
relatively homogenous population, of which no information factors [4, 5].
is given regarding race, age, environmental exposure, medi- Development of the proximal-distal axis is at least par-
cation usage, or socioeconomic status of the population. tially controlled by fibroblast growth factors (FGF) secreted
by the apical ectodermal ridge (AER) [4]. The AER is a thick-
ened layer of the ectoderm that forms over the distal edge of
Embryology the limb bud. FGF secreted by the AER stimulates proximal-
distal growth of the limb via differentiation of the underlying
Fetal growth and development of the lower extremities are mesoderm. As part of a complex positive feedback loop, the
controlled by a complex cascade of a multitude of growth signal to produce FGF is supplied by the underlying meso-
factors that are expressed in a particular sequence and at derm. The AER signaling center is responsible for the differ-
various concentrations during development. In the process of entiation of the underlying mesoderm and the development of
limb patterning, mesenchymal cells in the limb bud integrate the limb in the proximal-to-distal direction. Removal of the
positional information from the three axes [2, 3]. AER results in arrest of limb outgrowth. Furthermore, ecto-
pic implantation of the AER results in formation of an extra
limb. The AER also contributes to interdigital necrosis,
allowing separation of the initially webbed hand [6]. Defects
D. Paley, MD, FRCSC (*)
in the AER lead to anomalies such as limb truncation, trans-
St. Mary’s Medical Center, Paley Advanced Limb
Lengthening Institute, 901 45th Street, Kimmel Building, verse deficiencies, and syndactyly [7]. The genetics of the
West Palm Beach, FL 33407, USA apical ectodermal ridge have been further elucidated as a
e-mail: [email protected] complex interplay of related genes. Five paralogous gene
D.Y. Chong, MD families, named Hox 9 through Hox 13, from Hox A and
Department of Orthopedic Surgery, University of Oklahoma Hox D gene groups, come together co-linearly (HoxA-9 to
Health Sciences Center, Oklahoma City, OK 73104, USA
HoxA-13 and HoxD-9 to HoxD-13) and play important roles
in the AER.
D.E. Prince, MD, MPH
Development in the anteroposterior axis is patterned by
Memorial Sloan Kettering Cancer Center, 1275 York Avenue,
New York, NY 10065, USA secretion of sonic hedgehog (SHH) from the zone of polar-
e-mail: [email protected] izing activity (ZPA), a collection of cells along the posterior

362 D. Paley et al.
aspect of the limb bud. Transplantation of the ZPA from the the fourth or fifth ray, unusual facies, Pierre-Robin sequence,
posterior aspect to the anterior aspect of the limb bud causes and syndactyly of the toes. Considering the other congenital
creation of a mirrored duplication of the ulnar aspect of the conditions that present in select individuals with CFD, the
hand. In addition to its primary role in development of the underlying pathophysiology and, therefore, genetic causes
anterior-posterior axis, the ZPA also contributes to mainte- are likely to be distinct in this group of patients. In other
nance of proximal-to-distal limb development and partici- words, one must be careful to assume that all patients diag-
pates in the feedback loop of the AER [4]. Mutations in nosed by today’s criteria with “CFD” will have the same
Indian hedgehog, SHH, and PITX1 in humans have been underlying causality.
implicated in lower extremity polydactyly, while mice stud- An inheritable genetic defect or susceptibility to other
ies have shown an interaction between the expression of each risk factors or exposures at a particular stage in lower
factor [2, 8]. extremity development of the fetus would commonly pres-
Development in the dorsal-ventral axis is regulated by the ent with bilateral involvement; however, bilateral involve-
Wingless-type (Wnt) signaling pathway within the dorsal ment is rare, and associated with other known genetic
ectoderm [3]. The Wnt pathway induces the underlying meso- defects, such as Pierre-Robin sequence. The Pierre-Robin
derm to develop dorsal characteristics and is blocked in the sequence is primarily an anatomical defect of mandibular
ventral ectoderm, allowing the development of ventral charac- outgrowth that impacts on oropharyngeal volume and
teristics. In mice, inactivation of the Wnt signaling pathway patency of the palate. No single genetic abnormality has
results in the development of biventral limbs. The Wnt path- been identified in Pierre-Robin, though recent evidence
way also contributes to regulation of SHH, reflecting the com- shows that Sox9 may be a crucial step in the pathway [12].
plex interaction and coordination among the 3-dimensional Similarly, it is likely that a variety of inheritable genetic
pathways responsible for limb development [4, 6]. defects are responsible for a common resultant phenotype
It is unlikely that CFD is caused solely by a defect in the with similar but varied congenital abnormalities. Conversely,
overall function of the AER, as the distal portion of an a recent report of a child with Pierre-Robin and bilateral
extremity affected by CFD is often normal. It is more likely CFD found that array comparative genomic hybridization
that the defect is in the underlying mesodermal layer, within analysis showed no abnormalities at 1,543 loci while whole-
the complex interplay of developmental proteins at a point exon analysis identified no mutations suspected to be caus-
more downstream in the differentiation of the limb. ative for the patient’s condition [13].
A subset of bone morphogenetic proteins (BMPs), known
as growth/differentiation factors (GDFs), affect several skel-
Pathophysiology and Genetics etal processes, including endochondral ossification, synovial
joint formation, and tendon and ligament repair. Studies in
A single underlying genetic cause for CFD has not been elu- mice have suggested that GDF-5 deficiency affects the com-
cidated. In the senior author’s personal experience (over position and material properties of cortical bone tissue in the
1,000 patients with unilateral CFD) there was only one uni- femur, but the detailed mechanisms by which this occurs
lateral case that had a parent with unilateral CFD. In con- remain to be determined [4]. Paley et al. reported radio-
trast, in a smaller group of multi-limb congenital deficiencies graphic mineralization of the pseudoarthrotic region of the
including CFD on at least one limb, it was not uncommon to femur in patients with CFD with the application of exoge-
find a history of a first- or second-degree relative with a con- nous BMP-2 directly to the area [14].
genital limb anomaly. Several authors have postulated a Other investigators have supported a teratogenic cause for
genetic cause given that the presence of the condition at CFD as the phenotype is similar to the effects of thalidomide
birth, its association with other abnormalities that have exposure in utero [15]. These effects imply that at a particular
known genetic conditions, occasional bilateral involvement, stage in fetal growth an exposure in utero occurs causing a
and reports of familial cases are supporting evidence for an downstream effect on the remaining growth of the affected
underlying inheritable genetic defect with incomplete pene- lower extremity. The exact stage in development at which the
tration or an autosomal recessive pattern of inheritance [9– abnormality occurs could determine the severity of involve-
11]. It should be noted that phenotype has been used to ment. While a teratogenic exposure is possible as the underly-
diagnose and classify patients with CFD, despite quite varied ing cause, given the embryology of the limb, the explanation
clinical and radiographic presentations. As genetic and would have to involve a teratogen with transient effect only at
molecular understanding of the disease progresses, it is the time of exposure. Thus, fetuses exposed to the teratogenic
likely that grouping all patients into a single diagnosis of agent at one time point might have only mild CFD, exposed
CFD will not be sufficient. Some of the reported abnormali- only at the time of limb bud formation of the proximal femur,
ties associated with CFD are fibular hemimelia, femoral- while those fetuses with more severe involvement have per-
fibular-ulnar complex, patella aplasia/hypoplasia, absence of sistent exposure to the teratogenic agent.
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 363
Endogenous retinoids, including vitamin A and retinoid Ligamentous Structures

acid (the active form of vitamin A), have long have been Anterior-posterior instability of the knee is common in CFD,
implicated in limb development and abnormalities [5–7]. but its severity is variable. Multiple modern era studies uti-
Embryological studies of quail, rats, and mice demonstrate a lizing MRI have evaluated the ligamentous structures at the
role for retinoic acid in the organization of the dorsoventral knee in patients with various degrees of CFD. The results
axis [7, 16]. These results suggest that a combination of support the conclusion that like the hip, the distal femur and
maternal vitamin A supplementation and excess vitamin A in proximal tibia require interaction of bony and soft tissues to
the neonatal period could potentially be unfavorable for car- develop normally.
tilage development. It can be speculated that neonatal vita- Manner et al. [27] performed a radiographic study of 34
min A supplementation may be beneficial for bone only if knees associated with CFD with radiographs and MRI. The
the mother’s vitamin A status is low. anterior cruciate ligament (ACL) was affected in all knees
In summary, the embryologic and genetic data point studied, with 15 % hypoplastic and 85 % absent. The PCL
towards a somatic mutation disorder of cells within the was hypoplastic in 21 % and absent in 24 %. The most com-
developing limb bud as opposed to a germ cell mutation mon type (14 knees, 41 %) was aplasia of the ACL and a
(hereditary, familial) disorder for unilateral CFD. In contrast, normal PCL. The cruciate ligament dysplasias were differen-
multi-limb cases may be related to a germ cell mutation. tiated into three groups (Fig. 22.2). Type I had a hypoplastic
or absent ACL and a normal PCL. The intercondylar notch
width and height were decreased compared to the normal
Deformity and Pathoanatomy side, and the lateral tibial spine was hypoplastic. Type II
knees had aplasia of the ACL and hypoplasia of the PCL.
Osseous Deformities They had even narrower and shorter intercondylar notches,
CFD was initially grouped with cases of coxa vara, as this and both tibial spines were hypoplastic. Two cases had lat-
is the most obvious deformity in the majority of cases [17]. eral femoral osteochondral lesions. Type III knees had apla-
Cases were often separated into congenital short femur sia of both cruciate ligaments. The intercondylar notch was
and proximal femoral focal deficiency (PFFD), for mild essentially absent and covered with hyaline cartilage, and
versus severe cases, respectively. Recognition that these both tibial spines were aplastic. The distal femoral joint sur-
were a spectrum of pathology of the same congenital defi- face is concave, matching the convex tibial plateau like a ball
ciency leads to a consensus name change to CFD [18]. and socket. Three of these cases had discoid meniscus.
Although the femoral deformity and deficiency are the Overall, the authors found that on the tunnel-view radio-
most obvious, non-femoral structures are also involved. graphs, narrowing or absence of the femoral notch and flat-
These include the acetabulum, musculature, vessels, liga- tening of tibial eminences corresponded to a hypoplasia or
ments of the knee, tibia, fibula, and foot. In utero ultra- deficiency of the cruciate ligaments. Thus, the shape of the
sounds have shown both a shortened femur and a shallow distal femur and proximal tibia can be used to predict, with
acetabulum [19]. As with other pediatric proximal femur relative accuracy, the presence or absence of the ACL and
pathologies, dysplasia of the acetabulum whether initially PCL ligaments.
present or not is progressive [20]. These radiographic studies have been validated with the
The deformities of the femur vary from coxa valga in very use of arthroscopy to directly confirm the presence or
mild cases to severe coxa vara in more severe cases absence of the ACL and PCL. Johansson and Aparisi [28]
(Fig. 22.1). There is a flexion and retroversion deformity of published a case series of six patients with cruciate ligament
the upper femur. The distal femur demonstrates a valgus dysplasia. Three patients had both an anterior and posterior
deformity often referred to as a hypoplastic lateral femoral drawer sign, and arthroscopy confirmed aplasia of both the
condyle since the distance from the physis to the knee joint ACL and PCL. Three patients had an isolated anterior
line is smaller laterally than medially. In other words, there is drawer, and had ACL aplasia on arthroscopic examination.
a greater valgus convergence between the joint line and the All knees were found to have hypoplastic tibial spines on
distal femoral physis. The deformity of the proximal femur radiographs.
will be discussed in greater detail in a later section. Chugiak [29] looked at a larger series of 21 patients with
The ratio of growth in length of the short limb compared clinical and arthroscopic examinations. All patients were
to the long limb remains relatively unchanged throughout found to have an anterior drawer sign, and nine patients
growth [21–23]. This enables the final discrepancy in leg (43 %) had a posterior drawer sign. Four patients (19 %) had
length to be predicted from the initial radiographs [24]. On medial instability, including one patient who also had lateral
this basis the Paley multiplier method is able to accurately instability. The instrumented and clinic drawer tests were not
calculate the predicted limb length discrepancy (LLD) at found to be reliable enough, leading the authors to recom-
skeletal maturity [25, 26]. mend imaging of the cruciate ligaments prior to lengthening
364 D. Paley et al.
Fig. 22.1 Anteroposterior (a), lateral (b), and inferior-superior (c) be retroverted due to the 90° hip flexion of a 130° neck-shaft angle
views of pelvis and hip joints. These illustrations serve to simulate and femur. Just the flexion makes it appear to have 50° of retroversion.
recreate the proximal femoral deformity seen with severe CFD on the Since the distal femur is fixed to the proximal femur in external rotation
right hip. The model is based on a normal proximal femur with a 130° this retroversion is increased even more. Also note that the varus defor-
neck-shaft angle. First, flex the proximal femur 90° relative to the pel- mity is caused by the abduction of the proximal femur with the hip
vis. Next, abduct the flexed proximal femur 45° relative to the pelvis. flexed. The hip flexion places the greater trochanter facing posteriorly.
Now reconnect the distal femur to the proximal femur. The distal femur The proximity of the greater trochanter to the ilium and to the sacrum in
should be placed in 45° of external rotation relative to the pelvis. The this position explains why the gluteus medius and minimus, and pirifor-
resulting deformity is the CFD femur deformity typically seen in most mis muscles, are short
type 1b and some type 1a cases. Note that the femoral neck appears to
to avoid knee dislocation. The majority of patients (38 %) Muscle Pathoanatomy

were found to be completely deficient in both the ACL and Despite the wide spectrum of CFD, the underlying muscular
PCL. The ACL was completely deficient in 16 (71 %) and anatomical differences appear relatively consistent. Pirani
hypoplastic in 3 (14 %) patients. The PCL was completely et al. showed that in patients with Aitken types A through D,
deficient in 10 (48 %) and hypoplastic in 3 (14 %) patients. the muscles were all present but altered in their size, struc-
Only one patient had an intact ACL and PCL. ture, and location [30]. The study used MRI to qualitatively
In the majority of patients, both menisci were intact, with assess the musculature. The majority of muscles were found
only three (14 %) hypoplastic and unrelated to the cruciate to be smaller: gluteus maximus, gluteus medius, gluteus
ligaments. Changes in the intercondylar notch and tibial emi- minimus, quadriceps, adductor magnus, adductor longus,
nences were noted in some patients but it was not specifically adductor brevis, pectineus, semimembranosus, semitendino-
studied, as the authors felt that they were not as appreciable sus, and biceps femoris. The exception is the sartorius mus-
in patients under 6 years of age. They did note that the femo- cle that was found to be hypertrophied, to which Pirani et al.
ral intercondylar notch developed in some patients that had and Panting et al. attributed as the underlying cause of
aplasia of both cruciate ligaments. the deformities of the proximal femur, given the sartorius’
Fig. 22.2 Manner et al. [27] classification of intercondylar notch using This corresponds to aplasia of the ACL and hypoplasia of the PCL. Type
the tunnel view correlates to deficiency of the cruciate ligaments. Type III: The intercondylar notch is absent and covered with hyaline carti-
I: The intercondylar notch width and height are decreased compared to lage, and both tibial spines are aplastic. The distal femoral joint surface
the normal side. The lateral tibial spine is hypoplastic. This corresponds is concave, matching the convex tibial plateau. This corresponds to
to a hypoplastic or absent ACL and a normal PCL. Type II: Narrower aplasia of both cruciate ligaments
and shorter intercondylar notch with hypoplasia of both tibial spines.
orientation [31]. The obturator externus was found to be Vascular Pathoanatomy

elongated and the muscle belly extended almost entirely to The normal embryological development of the arteries of the
its insertion. The short external rotators had a larger cross- lower extremity parallels the formation of limb buds, occur-
sectional diameter and were found to insert on the postero- ring between the 4th and 8th weeks of gestation. The limb
medial greater trochanter. Overall, the course of the muscles begins with a single axial dorsal artery that continues as the
was more perpendicular and proximal than normal, inserting ischiadic artery and on to the popliteal artery. The external
onto the proximally migrated femur. iliac artery arises at 5 weeks of gestation and bifurcates into
We have also found this to be true in our surgical experi- the inferior epigastric artery and the femoral artery, which
ence: the musculature is present, the muscle bellies extend then bifurcates at 6 weeks gestation into the lateral and
nearly to their insertion, and the course of the muscles is medial branches. The medial branch becomes the deep fem-
proximal. Panting and William had previously noted similar oral artery and gives rise to the medial and lateral circumflex
findings, including the hypertrophied sartorius muscle, the arteries and connects to the ischiadic artery, while the lateral
relatively normal gluteal muscles, and a hypoplastic quadri- branch develops into the femoral artery. By 7.5 weeks the
ceps muscle [31]. Biko et al. also used MRI in seven patients femoral artery caliber is larger than the ischiadic artery and
to primarily evaluate the osseous structures, and reported a becomes the major blood supply to the popliteal artery and
qualitative decrease in size of the musculature in general. At leg. After full development, the dorsal axial artery remains as
the level of the triradiate cartilage, the cross-sectional size of the inferior gluteal artery while the ischiadic artery remains
the gluteus muscles was significantly smaller in comparison as the arteria comitans nervi ischiadici, running as the vasa
to the uninvolved contralateral limb [32]. vasorum of the sciatic nerve [33].
Pirani et al. postulated that the muscles became the pri- The vascular anatomy is also usually abnormal in an
mary stabilizers of the deformed hip joint, since the osseous extremity affected by CFD. Chomiak et al. reported the
structures did not impart inherent stability. However, in our results of 21 patients with various degrees of CFD studied
experience the majority of untreated patients with CFD do with computed tomography (CT) angiograms to identify vas-
not report ipsilateral hip pain, even after knee fusion and cular abnormalities [34]. The authors found that more severe
with prosthetic use and weight bearing. cases of CFD had a smaller diameter and a shorter length of
366 D. Paley et al.
the femoral artery; however, the severity of osseous abnor- with prenatal ultrasound early in the pregnancy by measur-
malities did not directly correlate with the topographical vas- ing the lengths of the two femurs. In such cases the predicted
cular anatomy abnormalities. All patients, at the least, had leg length discrepancy at birth and maturity can be calcu-
differences compared to the contralateral unaffected extrem- lated using the multiplier method [36].
ity: mainly with smaller vessel caliber, decreased number of
vessels to the thigh, and more proximal bifurcation of the
external iliac into femoral artery and deep femoral artery. Physical Exam
Despite this, 19 of the 21 patients had the blood supply to the
femur and pseudoarthrosis from branches of the deep femo- There is an obvious leg length discrepancy. Associated fibu-
ral artery, which originated from the external iliac artery. lar hemimelia (FH) and ray deficiency may be present. The
Notably, Chomiak found 2 of the 21 patients with a persis- hips, knees, and ankles should be examined for range of
tent ischiadic artery as the dominant vascular supply to the leg motion and flexion contractures. Neonates and young infants
and a diminutive femoral artery that supplied solely the medial normally have such contractures for the first 3–6 months. At
thigh [34]. This is an extremely unusual finding in otherwise the hip, patients may have external rotation and fixed flexion
normal humans, but is seen in lower mammals [35]. deformity of the hip, as well as limited abduction when coxa
It is critical to identify these vascular anomalies preopera- vara is present. At the knee, patients may have flexion con-
tively when reconstruction using rotationplasty is consid- tractures, hypoplastic or subluxation-dislocation of the
ered. The senior author has identified several cases with a patella, AP, or rotary instability of the knee.
dominant ischiadic artery and absent superficial femoral Finally, muscle length tests are recorded to identify pre-
artery while performing rotationplasty. In our opinion, an existing limits to muscle lengthening. These tests include the
MR angiogram (MRA) is indicated in all such cases prior to Ely test for rectus femoris tightness, popliteal angle mea-
a rotationplasty. surement for hamstring tightness, and Ober sign for fascia
lata or iliotibial band tightness (Fig. 22.3).
Box 22.1. CFD Pathology

• The incidence of CFD is approximately 1 in 50,000.
• Limb bud formation is affected by a complex inter- Box 22.2. Physical Examination Findings Often Seen in
play of signaling, including AER, ZPA, and Wnt. Patients with CFD
• CFD is likely caused by a somatic mutation during • Hip: External rotation (ER) deformity or increased
the development of the limb bud, but some cases ER versus internal rotation (IR); fixed flexion defor-
may be caused by an inherited germ cell mutation. mity (FFD) of hip; limitation of abduction (when
• CFD usually presents with proximal coxa vara and coxa vara is present).
distal femoral valgus. • Knee: Fixed flexion deformity of knee; no limita-
• Cruciate ligament deficiency is common and can tion of knee flexion; hypoplastic patella; lateral
often be identified on radiographs. tracking or subluxated or dislocated patella; antero-
• Generally, most muscles around the pelvis are pres- posterior instability of knee; rotary instability of
ent and hypoplastic, though the sartorius may be knee; anterior dislocation of tibia on femur with
hypertrophied. knee extension followed by reduction of knee with
An ischiadic artery may be present and more attempted flexion; hypermobile meniscal clunks;
dominant than the femoral artery in some patients temporary locking of the knee during flexion.
with CFD. • Ankle: Limitations of ankle dorsiflexion; obligatory
eversion with dorsiflexion; hypermobility of ankle
with increased eversion; lateral malleolus more
proximal than medial malleolus.
Evaluating the Child with Unilateral CFD
History
Imaging
Most children born with unilateral CFD have no family his-
tory of this or other congenital anomalies. Nevertheless, Radiographic Examination
inquiry should be made into family history, exposure to Radiographs should include a full-length anteroposterior
drugs, medications, radiation, or infectious diseases during (AP) standing legs with the patellas pointing forward. In
the first trimester. Many cases of CFD are now identified children who are unable to stand, a pull-down X-ray may be
Fig. 22.3 Muscle lengthening tests: (a) The Ely test checks for rectus (c) the Ober test checks for fascia lata tightness; a positive sign is the
femoris tightness, demonstrating pelvic flexion with prone knee bend; thigh going into an abduction position when the hip is hyperextended
(b) the popliteal angle is the angle between the vertical and the line of and the knee flexed to 90°
the tibia with the hip at 90° flexion and the knee maximally extended;
performed. It allows measurement of the length of the femurs pseudoarthrosis, the MRI can help determine if the cartilage
and tibia, though it does not include foot height. Long lateral of the femoral head is fused to the acetabular cartilage. For
leg radiographs in maximum extension allow for evaluation optimal imaging, the cuts of the proximal femur should be
of knee flexion contractures and more accurate length mea- reformatted in an oblique plane to see the entire proximal
surement. A supine AP pelvis allows more accurate measure- femur as a single image. MRI can also help outline the intra-
ment of the center-edge angle (CEA) of both hips to assess articular pathology of the knee, identifying deficiency of the
for hip dysplasia. It is also a better quality X-ray to assess for cruciate ligaments and outlining the shape of the joint sur-
ossification of the femoral neck. It is important that the pel- faces in the frontal and sagittal plane.
vis be level for more accurate measurement.
Computerized Tomography (CT)
Magnetic Resonance Imaging CT is only useful at an older age when the acetabulum and
Magnetic resonance imaging (MRI) is useful to assess the proximal femur are nearly fully ossified. Three-dimensional
integrity of the proximal femur. It can help determine CT reconstruction is useful to compare the normal acetabu-
whether the femoral head is joined to the shaft of the femur lum with the dysplastic side. In older children 3D CT can
via a cartilaginous femoral neck. In case of a femoral neck show the pathologic anatomy.
368 D. Paley et al.
pretation of these images with better reliability. As a result,

Box 22.3. Evaluating the Child with CFD MRI dramatically improves the ability to correctly catego-
• CFD can be bilateral; check the contralateral side as rize patients over conventional radiographs alone [37].
well. Table 22.1 shows a comparison of several of the classifica-
• The hip, knee, and ankle should all undergo thor- tion schemes further elucidated below.
ough physical examinations.
• Fibular hemimelia is commonly associated with
CFD. Aitken, 1959
• Radiographic imaging should include a full-length
AP legs (standing or pull-down), long leg laterals in In 1959 Aitken proposed a four-class schema for PFFD (see
maximum extension, and a supine AP pelvis. Fig. 22.4) [17]. This became the most commonly utilized
• MRI is useful to evaluate soft tissue, ligaments, and classification system for PFFD. Aitken focused the classifi-
a cartilaginous femoral neck. cation system on the development of the femoral head, neck,
and acetabulum, with progressive dysplasia from one class to
the next. The Aitken classification does not include the con-
genital short femur group discussed earlier.
Aitken initially proposed that Class A patients were not
Classification Systems technically PFFD, as all the structures were present, albeit
deformed. These patients had a femoral head, a cartilaginous
Multiple classification systems have been described over the femoral neck, an adequate acetabulum, and a very short fem-
years in attempts to categorize the pathology and direct sur- oral segment. Severe subtrochanteric femoral varus and fem-
gical treatment (Figs. 22.4, 22.5, and 22.6). Most older clas- oral shortening were appreciated. A pseudoarthrosis of the
sification schemes were based on plain radiographs. Newer proximal femur was appreciated between the femoral shaft
classification systems incorporate modern imaging modali- and head, either between shaft and trochanteric component
ties, specifically MRI, which allows detailed evaluation of (subtrochanteric pseudoarthrosis) or between trochanteric
osseous, cartilaginous, and soft-tissue structures, including component and head (femoral neck pseudoarthrosis). Aitken
the non-ossified femoral head, non-ossified acetabulum, noted that the pseudoarthrosis did not spontaneously resolve
labrum, pseudoarthrosis, and musculature [32]. The ability in all patients by skeletal maturity, but did eventually heal in
to perform MRI under anesthesia for infants allows for inter- some patients.
Fig. 22.4 Aitken classification of congenital femoral deficiency. The to shaft, which instead has a proximal ossified tuft; (c) dysplastic ace-
Aitken classification is based on the relationship of the acetabulum and tabulum, minimal or absent femoral head, disconnected femoral shaft
the femoral head: (a) Adequate acetabulum, femoral head is present and with tuft; and (d) no acetabulum, no femoral head, shortened proximal
attached to shaft; (b) adequate acetabulum, femoral head not connected femoral shaft with no tuft
Fig. 22.5 Pappas classification of congenital femoral deficiency. The nection between femoral shaft and head, (V) midfemoral deficiency
Pappas classification is divided into nine classes: (I) congenital absence, with hypoplastic proximal and distal development, (VI) distal femoral
(II) proximal femoral and pelvic deficiency, (III) proximal femoral defi- deficiency, (VII) hypoplastic femur with coxa vara and sclerosed diaph-
ciency with no osseous connection between femoral shaft and head, ysis, (VIII) hypoplastic femur with coxa valga, and (IX) hypoplastic
(IV) proximal femoral deficiency with disorganized fibrosseous discon- femur with normal proportions
The hallmark of the Aitken Class B PFFD was a lack of femoral head and a deformed, shortened femoral shaft with-
connection, either bony or cartilaginous, between the femo- out tufting on the proximal shaft of the femur.
ral shaft and head that persists into skeletal maturity. These
patients have a femoral head within an adequate acetabulum
and a shortened, deformed femoral shaft. The proximal por- Pappas, 1983
tion of the shaft often has an ossified tuft. The femoral head
and shaft do not move as a unit. The Pappas classification (see Fig. 22.5), published in 1983
Aitken Class C is defined by a severely dysplastic acetab- [38], was based on the anatomy of 139 cases evaluated in
ulum. The femoral head does not ossify and shaft of the Massachusetts. Pappas describes nine classes based on the
femur is short with an ossified tuft at the proximal end of the femoral deficiency and associated deformity, with diminishing
shaft. There is no cartilaginous or osseous femoral neck or severity as the class number increased. This was the first clas-
trochanteric component. sification scheme to separate middle and distal femoral defi-
Aitken Class D is the most dysplastic form of PFFD. This ciencies into their own class. Recommendations were given
class is characterized by the absence of the acetabulum and for treatment for each class; however, treatment was depen-
370 D. Paley et al.
Fig. 22.6 (a) Paley classification of congenital femoral deficiency. and retroversion; 1a3: coxa vara, retroversion, and mild genu valgum;
There are four types of congenital femoral deficiency: (1) intact femur 1b1: subtrochanteric type; 1b2: neck type; 1b3: combined neck and sub-
normal ossification (a) or delayed ossification (b); (2) mobile pseudoar- trochanteric type; 2a: mobile femoral head; 2b: partially fused femoral
throsis; (3) diaphyseal deficiency; (4) distal deficiency. (b) Each Paley head; 2c: completely fused or absent femoral head; 3a: mobile knee
type has several subtypes: 1a1: Normal-shaped short femur with mild joint with greater than 45° of motion; 3b: stiff knee with less than 45°
genu valgum; 1a2: normal-shaped short femur with mild genu valgum of motion; 3c: absent femur or fused knee joint
Table 22.1 Comparison of different classification schemes

Gillespie and
Paley [42] Pappas [38] Torode [39] Hamanishi [15] Aitken [17]
Normal anatomy/minimal deformity/ 1A 7,8,9 Group 1 Type 1, type 2 Class A
hypoplasia
Subtrochanteric delayed ossification 1B subtrochanteric 4, 5 Group 1 Type 3a Class A
Femoral neck delayed ossification 1B femoral neck 3 Group 1 Type 3b Class A
Combined delayed ossification 1B combined 3 Type 4a
True pseudoarthrosis with no cartilaginous 2a, 2b 3 Group 2 Type 4b Class B
or osseous femoral neck
Dysplastic acetabulum, no femoral head 2b, 3 2 Group 2 Type 4c Class C
No proximal femur, short distal femur only 3a,b 2 Group 2 Type 4c Class D
Femoral absence 3c 1 Type 5
Distal femoral deficiency only 4 6
dent on the patient’s joint stability and function, which are not Group 1 was defined as those patients who could have
part of the classification system. Therefore treatment could not good hip and knee function with or without surgical correc-
be based directly on the classification schema of Pappas. tion. Radiographically, group 1 patients had coxa vara, lat-
eral femoral bowing, and hypoplasia of the knee with valgus
but the predicted leg length discrepancy was no greater than
Gillespie and Torode, 1983 30 % of the normal femur and femoral deficiency was
40–60 % of the contralateral femur. Clinically this group had
In 1983, Gillespie and Torode proposed a straightforward laxity in the sagittal plane and mobile joints without flexion
division of CFD into two categories: congenital short femur deformities at hip or knee.
and true PFFD [39, 40]. Each of the two groups had both Group 2 patients had hip and knee joints that were not
radiographic and clinical criteria, but were ultimately defined adequate to support the patient without pain. The appropriate
by the function of the hip and knee joints, rather than the treatment recommendation for this group of patients was
type of surgery each would necessitate. amputation and/or rotationplasty of the lower limb. These
patients had markedly short femurs. Radiographically, these Group 1 was defined by hypoplasia of the femur, either
patients had less than 40 % of the femoral length of the nor- without deformity type 1a or with minimal angulation and
mal side and deficiency or absence of the femoral head and/ cortical thickening, type 1b. The type 1a was associated with
or neck. The authors defined deficiency of the femoral head ipsilateral below the knee deformities, while the type 1b was
as a tenuous cartilaginous connection between femoral shaft associated with severe, symmetric bilateral below the knee
and head. Clinically, these patients had fixed flexion defor- deformities. The second group was defined by hypoplasia
mity at hip and/or knee. and femoral varus angulation. Type 2a had a normal neck-
The overall goal of the Gillespie and Torode classification shaft angle but type 2b had a decreased neck-shaft angle.
was to simplify the decision making. The authors rationalized A transverse subtrochanteric ossification defect was noted in
for families and surgeons a “life plan” regarding treatment this type as well, but all ossified later in life.
of the affected limb based on each of the two groups. This The type 3 group had marked coxa vara from delayed and
classification aims to aid families and physicians communi- inconsistent ossification of the femoral neck and subtrochan-
cate more effectively and correlate early clinical signs with teric region. This group also has marked retroversion of the
long-term treatment plans. The value of this classification femoral neck and distal femur external rotation. Type 3a with
schema is its simplicity and its guidance in surgical treat- a straight femoral shaft was considered “stable” as the proxi-
ment. The drawbacks to this classification scheme are its mal femoral varus would not progress, while the “unstable”
inability to adapt to improved surgical techniques based on a type 3b with an increased epiphyseal head angle >60° was
better understanding of pathoanatomy, as well as advances in reported to progress with time.
biology and technology. The type 4 femurs had absent or stunted and tapered prox-
imal femoral shafts with the femoral head attached directly
to the shaft, when present. The femoral head was connected
Hamanishi, 1980 to the shaft by a pseudoarthrosis and the entire shaft was
proximally migrated. The type 4a had a pseudoarthrosis of
Hamanishi [15] published a five-tiered classification system the femoral neck and subtrochanteric region. The type 4b
for congenitally short femur. This schema involved a scoring had an absent proximal femur with the femoral head con-
system that included the entire lower limb and upper limb nected directly to a tapered proximal shaft. The type 4c had
involvement, as opposed to other classification systems, a complete absence of the proximal femur.
which focus only on the femur. However, the schema does Type 5 femurs indicated a rudimentary femur, which ossi-
not include specifically the acetabulum, as the author fied later, or complete absence.
believed that this component is directly related to the femoral One major flaw in this classification system is that it
head. It was intended to differentiate those patients with the divides the groups based on measurements with arbitrarily
teratologic sequelae of thalidomide exposure from those chosen cutoffs, such as the neck-shaft angle and femoral
patients with idiopathic CFD. The diagnosis was based on a varus which change with time. Thus, young patients could
series of 60 total patients, 14 of which were known to have move from one group to another without intervention and the
had in utero thalidomide exposure. It is an observational treatment plan would also therefore be variable. Additionally,
classification system and does not aim to guide treatment or this classification scheme does not include the stability of the
prognosis. The study was quite comprehensive as the authors hip or knee, and the presence of knee ligaments, and does not
included socioeconomic class, an extensive family history, guide surgical or nonsurgical treatment.
parental age, birth history, and in utero history in their review. Some important remarks from this study are that the leg
The age of the study subjects at the time of evaluation varied length discrepancy was consistent, when stated as a percent-
from 6 months to 50 years. age of the unaffected normal side, which was consistent with
Hamanishi identified several differences between the older retrospective reports [21, 22] and newer mathematical
two groups: overall, the thalidomide exposure group had methods to predict limb leg discrepancy at maturity [26].
femora that were more severely affected than the idiopathic
group; the thalidomide exposure group more often had
concomitant abnormalities of the tibia and radius as Kalamachi, 1985
opposed to the fibula and ulna in the idiopathic CFD group;
the thalidomide group had lower extremity preaxial (tibial) The Kalamachi classification (1985) [41] attempted to relate the
polydactyly, whereas the idiopathic group had lower femoral deficiency directly to the limb function. Type I patients
extremity postaxial (fibular) deficiency. The author defined had congenital shortening of the femur with a normal hip and
five groups with subgroups in some utilizing radiographic knee morphology and function. Type II patients had a congeni-
measurements of the affected femur to quantify the degree tally short femur with coxa vara, with some irregularity of the
of deformity. growth plate, a located hip, and a well-developed acetabulum.
372 D. Paley et al.
Type III patients had a developed acetabulum but a deficient degree of development and integrity of the femur (see
proximal femur. With time, they were separated into two Fig. 22.6). Unlike the previous classifications, Paley wanted
groups. In the IIIA group, the radiographically apparent to create a classification where the type would not change
proximal femoral defect ossifies into a bony bridge, often in with the degree of ossification of the femur (age dependent).
varus. In the IIIB group, the defect does not ossify, and A longitudinal follow-up of different classification systems
develops into a pseudoarthrosis. Type IV patients had no by Sanpera and Sparks [43] showed that the existing classifi-
acetabulum and no connection between the pelvis and the cation systems were inaccurate in predicting the final femo-
distal femoral fragment, which often was a spike proximally. ral morphology based on the initial radiographs. Paley also
Type V patients had no hip joint and no femur. For all five wanted a classification that was oriented towards reconstruc-
classification types, the most common treatment was a Boyd tion and not amputation. As such, the classification types and
or Syme amputation. Knee fusions were used to correct subtypes each have a separate surgical reconstructive
deformity as well as provide a large lever arm for a severely prescription.
shortened femoral segment. Femoral osteotomies were
used mostly in type II and IIIA patients to correct coxa vara.
Limb equalization procedures were done in nine patients Treatment Options for Congenital
(four epiphysiodesis, five lengthening) of type I and II. Femoral Deficiency
The rest were treated non-operatively or with orthotic and
prosthetic fitting. Rotationplasty
Rotationplasty has been a commonly accepted treatment for

Paley, 1998 patients with CFD with significant proximal deficiency,
which is deemed “unreconstructable” [44–48]. The most
Paley proposed a classification system for CFD (first pre- commonly employed surgical technique is the Van Nes rota-
sented at a symposium in Dallas in 1996 documented in a tionplasty and variations of this technique. In the Van Nes
book published from that symposium [42]) based on the rotationplasty, the lower limb is rotated 180° to use the ankle
and foot as a functional knee joint with a prosthesis. The
rotationplasty can be combined with a knee fusion when the
Box 22.4. Femur Deficiency Classification knee is unstable, or as in the Brown modification the knee is
(Paley, 1998) [42] maintained after 180° rotation and the distal femur is fused
to the pelvis to substitute for a deficient hip allowing flexion
Type 1: Intact femur with mobile hip and knee:
and extension [44]. This will be discussed in more detail in a
(a) Normal ossification proximal femur
later section.
(b) Delayed ossification proximal femur (neck, sub-
Gillespie and Torode reported their surgical recommenda-
trochanteric, or combined neck subtrochanteric
tions along with their classification schema in 1983 [39, 40].
types)
Prior to the advent of the Wagner device in 1971, patients clas-
Type 2: Mobile pseudoarthrosis (greater trochan- sified as Group 1 with congenital short femur underwent rota-
teric apophysis present), knee usually mobile: tionplasty. After the Wagner technique demonstrated the
(a) Femoral head mobile in acetabulum feasibility of lengthening up to 20 cm, femoral lengthening
(b) Femoral head partially fused to acetabulum became the standard recommendation for Group 1, but to a
(c) Femoral head and acetabulum completely fused or maximum of 20 % of the femur length because the risk of hip
absent and knee subluxation or dislocation was considered too high.
Of the five Group 1 patients that underwent rotationplasty,
Type 3: Diaphyseal deficiency of femur
three had good function but two required a Syme amputation
(greater trochanteric apophysis absent):
and fitting with an above-the-knee prosthesis. Patients classi-
(a) Distal physis present; knee motion ≥45°
fied by Gillespie and Torode as Group 2 had true PFFD and
(b) Distal physis present; knee motion <45°
received rotationplasty, Syme amputation for prosthesis fitting,
(c) Complete deficiency of distal femur or fusion of
and/or knee fusion. Of 43 Group 2 patients with instability of
distal femoral remnant to tibia (distal physis absent)
the hip and knee, 21 underwent rotationplasty, and of those, 8
Type 4: Distal deficiency of femur required de-rotational surgery and 2 required a Syme amputa-
(proximal end normal) tion. They strongly advocated knee fusion for Group 2 patients
to correct the valgus and flexion deformities at the knee and
tibial rotationplasty to restore “knee flexion” with the ankle full tibial rotation (seven subjects) demonstrated prosthetic
dorsiflexion. knee function during stance as they were able to support a
Brown reported on three patients with severe CFD who flexed-knee posture at both speeds and produced greater
underwent a modification of the Van Nes rotationplasty with knee-extensor moments at preferred speeds as compared
mean of 6-year follow-up who had satisfactory results and with the Syme group. Non-prosthetic limb compensatory
range of motion of both the hip and knee to 90° of flexion [44]. mechanics were significantly exacerbated in subjects with a
The Brown modification includes resecting the deficient Syme amputation compared with the Van Nes group: (1)
proximal femur, externally rotating the limb 180°, fusing the stance-phase vaulting, resulting in greater inappropriate
femoral remnant to the pelvis, and leaving the muscles distal to ankle-power generation at both walking speeds; (2) exces-
the knee undisturbed. Using this technique there was no dero- sive hip-extensor moments at fast speeds; (3) excessive hip-
tation as previously recurred with the Van Nes rotationplasty power absorption and generation at both speeds; and (4)
which incorporated a midtibial osteotomy for rotation. excessive knee-joint power generation at both speeds. The
Ackman et al. reviewed 12 patients who underwent Van improved gait after Van Nes rotational osteotomy is one fac-
Nes rotationplasty for CFD to determine the long-term out- tor that should be considered when making clinical decisions
come [48]. At a mean of 21.5 years (11–45) after their rota- for children with PFFD.
tionplasty, a total of 12 prosthetic patients were compared Other studies have found some potential issues with Syme
with 12 normal age- and gender-matched controls. The amputation in the long term: heel pad migration, skin
authors found no differences between the groups in overall sloughs, and problems with prosthetic fitting. Anderson et al.
health and well-being on the SF-36, but significant differ- reviewed 69 Syme amputations performed in 62 children
ences were seen in gait parameters in the CFD group. Patients with the major indication of leg length discrepancy, due to
who had undergone Van Nes rotationplasty had a high level either paraxial fibula hemimelia (33 cases) or proximal focal
of function and quality of life at long-term follow-up, but femoral deficiency (19 cases) [51]. The average age at ampu-
presented with significant differences in gait and posture tation was 5.6 years, with an average follow-up of 10.5 years
compared with the control group. (range 1–25 years). Although the results were assessed by a
combination of chart review, patient recall examinations, and
questionnaires, satisfaction in adulthood was found to be
Syme Amputation high. Early complications included three skin sloughs and
one infection. Late complications included 2 retained os cal-
Alman et al. reviewed the results of treatment of 16 patients cis apophyses, 1 exostosis, and 16 cases of heel pad migra-
who had had an isolated unilateral PFFD; 9 were managed tion. Only one of the heel pad groups required revision;
with a rotationplasty and 7 with a Syme amputation com- prosthetic adjustment resolved symptoms in the remaining
bined with an arthrodesis of the knee [49]. The perceived patients. However, prosthetic knees were often too low
physical appearance, gross motor function, and metabolic because of failure to limit the length of the stump appropri-
energy expended in walking were assessed. The mean dura- ately, though this finding was not as problematic in the CFD
tion of follow-up was 9.9 years (range, 4–14 years). The group as the residual limb was significantly shorter.
mean age of the patients at the time of the study was 13.9 years
(range, 8–18.4 years) in the rotationplasty group and
14.8 years (range, 9.5–19.9 years) in the Syme-amputation Limb Lengthening
group. There were three female patients in each group.
Roentgenograms showed that the femoral head was in the Aston et al. published a series of 27 patients with Paley type
acetabulum (Aitken class A or B) in four of the seven patients 1 CFD (Pappas grades VII, VIII, and IX) who underwent a
in the Syme-amputation group and in five of the nine patients total of 30 lengthening procedures, with 3 patients undergo-
in the rotationplasty group; the remaining patients did not ing a second femoral lengthening [52]. All patients under-
have this finding (Aitken class C or D). There was no differ- went femoral lengthening with a multiplanar Ilizarov-type
ence in gross motor function or perceived physical appear- external fixator. The mean increase in length was 5.8 cm
ance between the groups. Rotationplasty was associated with (3.3–10.4 cm) and 18.65 % of the total length of the femur
a more energy-efficient gait than was Syme amputation. (9.7–48.8 %). The mean time in the frame was 223 days
Fowler et al. measured lower limb kinematics and kinet- (75–363) with a mean distraction index of 1.28 months per
ics during preferred and fast speeds of walking in persons cm. The authors initially performed the osteotomy distal, but
with PFFD to compare outcomes after Syme amputation after 17 distal osteotomies changed to a proximal osteotomy
(nine subjects) with those after Van Nes rotationplasty (ten for lengthening, noticing a significant increase in mean range
subjects) [50]. Subjects with a Van Nes rotationplasty and of knee motion from 98.1° to 124.2° (p = 0.041) and a trend
374 D. Paley et al.
towards a reduced requirement for quadricepsplasty, excellent and good in over 93 % in all groups, with a mean
although this was not statistically significant (p = 0.07). The overall lengthening of 6.0 cm (range, 2.2–12.5).
overall incidence of regenerate deformation or fracture Complications included femur fracture, pin site problems
requiring open reduction and internal fixation was similar in requiring surgery, premature consolidation, nerve irritation/
the distal and proximal osteotomy groups (56.7 % and palsy, delayed union, knee subluxation, and hip subluxation.
53.8 %, respectively). However, in the proximal osteotomy The fracture rate in the older age group was significantly
group, preplacement of a Rush nail reduced this rate from lower than the other groups, but it included several patients
100 % without a nail to 0 % with a nail (p < 0.001). When that were undergoing lengthening over nails. There was no
comparing a distal osteotomy with a proximal one over a significant difference in unplanned surgery rate between
Rush nail for lengthening, there was a significant decrease in groups 1 and 2 (see Table 22.2). The difference with group 3
fracture rate from 58.8 % to 0 % (p = 0.043). This supports was related to refractures. Prophylactic rodding was not per-
the premise of Paley and Herzenberg that CFD patients formed at the time of fixator removal in this series of patients,
should be lengthened over a Rush rod or have a Rush rod but is now part of the standard treatment algorithm, given the
inserted after frame removal to reduce the risk of regenerate high rate of fractures [56].
bending or fracture [53]. The authors have previously presented their results on
Aston et al. concluded that lengthening of the femur with femoral lengthening after a hip stabilization procedure
an Ilizarov construct should be carried out through a proxi- [57]. A retrospective review was performed of 35 patients
mal osteotomy over a Rush nail and lengthening to a maxi- with CFD, Paley types 1a and 1b, who underwent femoral
mum of 6 cm during one treatment, or 20 % of the original lengthening after a pelvic osteotomy, proximal femoral
length of the femur, to reduce the risk of complications [52]. osteotomy, or a combination. Patients underwent a hip sta-
Paley and Standard have published on a distal osteotomy for bilization procedure at a mean age of 2.4 years (2–5.5). The
CFD to avoid lengthening through the abnormal, sclerotic mean age at first femoral lengthening was 3.7 years
bone often seen in the proximal femoral shaft as much as (3–10.7). The mean overall limb length difference prior to
possible with good success [54, 55]. femoral lengthening was 64.8 mm (47–100), with a mean
Saghieh and Paley studied a group of 95 CFD patients postoperative difference of 8.1 mm (−14 to 32). The aver-
who had undergone lengthening between the years 1988 and age amount of time in the external fixator was 186.1 days
2000 (Table 22.2). These patients did not undergo the (107–311), or 1.1 months/cm. Preoperatively, average knee
Superhip or Superknee procedures that are described later in ROM was 2°–131° and hip ROM was −2° extension to
this chapter. All femoral lengthenings were with the Ilizarov 115° flexion. Follow-up at a mean of 14.4 months (5.6–
device with extension of the fixator across the knee to the 45.2) showed return to baseline ROM in knee of 2°–119°
tibia with hinges. The postoperative result based on knee and and hip from −1° extension to 91° flexion. The overall rate
hip range of motion score, gait score, lengthening goal score, of obstacles and complications was 37.1 % and 32.4 %,
and alignment score, pain score, and activity level score was respectively [58].
Table 22.2 Limb lengthening results and complications in 95 patients

Group 1 (<6 years) Group 2 (6–13 years) Group 3 (>13 years)
Number of patients (n) 30 40 25
Amount of length achieved
Length (cm) 5.4 (2.8–8.5) 6.2 (2.5–11) 6.3 (2.2–12.5)
Relative (%) 39 (12–71) 24 (7–54) 20 (5–58)
Lengthening index (month/cm) 1.0 (0.5–1.8) 1.1 (0.5–2.2) 1.1 (0.5–2.2)
Good/excellent outcome score 28/30 (93 %) 37/40 (93 %) 24/25 (96 %)
Problems, obstacles, and complications
Femur fracture 13 14 4
Pin-site problems requiring surgery 4 3 1
Nerve irritation/palsy 3 3 1
Premature consolidation 0 3 3
Delayed union 2 4 3
Knee subluxation 5 5 3
Hip subluxation 3 3 1
Total unplanned surgery 21 (70 %) 22 (55 %) 9 (36 %)
Treatment of CFD has always been divided between knee. In cases with combined CFD and FH, the strategy for
amputation and lengthening. In the past, the indications for FH must be combined with that of the CFD [18].
amputation were much more liberal. Currently, the dividing
line can be based more on pathoanatomy. Treatment varies, Step 1: Preparatory Surgery for the Hip and Knee
depending on the severity of the underlying deficiency. The Prior to lengthening one must determine whether the hip and
less involved patients, such as Paley type 1a, usually only knee joints are stable and/or deformed and whether surgical
require leg length equalization surgery, consisting of limb procedures for these joints are required before initiating limb
lengthening and/or contralateral epiphysiodesis at the appro- lengthening. At the hip, if the acetabulum has an acetabular
priate time. The current treatment of more severely affected index with comparable slope to the normal contralateral side,
patients, such as Paley type 1b and type 2, consists of com- a center edge angle (CEA) ≥ 20°, and a neck-shaft angle
plex surgical interventions consisting of reconstruction of (NSA) ≥ 110°, no separate hip surgery is required before the
the hip and knee joints, correction of femoral deformity and first lengthening [59]. If the acetabulum shows signs of dys-
length, ossification of the pseudoarthrosis, and reconstruc- plasia then a pelvic osteotomy should be performed prior to
tion of lax or absent ligaments. This is discussed in the sub- lengthening. Increased slope of the sourcil (acetabular roof)
sequent sections. The most severely affected patients, Paley or acetabular index compared to the other side is a subtle but
type 3, usually undergo surgery to improve prosthetic use, as sensitive sign of acetabular dysplasia. Coxa vara should be
regaining satisfactory function with limb lengthening and corrected prior to lengthening if the NSA is less than 110°.
reconstruction is often not feasible. Similarly, external rotation deformity of the hip is a factor to
consider for correction at the same time as the acetabular
dysplasia. If a Dega-type osteotomy is chosen, then there is
usually a gain of about 1 cm in leg length. Associated hip
Box 22.5. Classification and Treatment deformities of retroversion, hip flexion contracture, and hip
• Multiple classification schemes exist, mostly based abduction contracture should be simultaneously addressed.
on their anatomic deformity, though sometimes The flexion contracture of the hip is treated by recession of
they are age dependent or have arbitrary cutoffs. the psoas tendon and release of the rectus femoris tendon. A
• The Paley classification separates into types and bony flexion deformity may be treated by a proximal femoral
subtypes based on their surgical treatment for extension osteotomy. The abduction contracture is treated by
reconstruction. lengthening or resection of the fascia lata and, if necessary,
• Van Nes rotationplasty has been used historically an abductor muscle slide at the iliac crest. When all of these
on severely shortened femurs with good results. deformities are present together and especially with higher
• Patients undergoing Syme amputation with knee degrees of angulation, the reconstructive procedure is called
arthrodesis often have more altered gait mechanics the Superhip procedure [60].
with greater energy expenditure compared to those Other factors should be examined prior to lengthening.
undergoing a rotationplasty. The proximal femur should be normally ossified for the
• In the properly selected patient, limb lengthening patient’s age. Often, severe cases of CFD have delayed
often has good outcomes, but can have significant ossification of the femoral neck or subtrochanteric region,
complications. However, these untoward events can and bone morphogenic protein (BMP) may be added to the
be lessened with prophylactic joint stabilization and femoral neck to promote ossification [14]. The fascia lata
intramedullary rodding techniques. is a thin but very tough limiting membrane that resists
lengthening and applies pressure across the knee joint and
the distal femoral growth plate. Thus, it should always be
removed or released before lengthening. It can also be
Recommended Surgical Reconstructive used to reconstruct absent knee cruciate ligaments if pro-
Strategy for Paley Type 1 CFD cedures are done concurrently. A hemiephysiodesis may
also be performed to correct frontal plane deformities. The
Outlining a “Life Plan” for the Family ideal age for the preparatory procedure is between ages 2
At the initial consultation, a surgical reconstructive strategy and 3 years.
or “life plan” projected to skeletal maturity should be out-
lined for the child and the family. It is helpful to write this Step 2: Serial Lengthenings of the Femur
out on the back of a printed radiograph for the family to put and/or Tibia
in the child’s scrapbook and to refer to in the ensuing years. A prediction of total leg length discrepancy at skeletal matu-
The strategy is based on the type of CFD, the projected LLD rity helps determine the approximate number of lengthening
at maturity, and the reconstructive potential of the hip and surgeries required. This can also be done using the Paley
376 D. Paley et al.
multiplier method [26]. The majority of CFD cases will preferable to complete all the lengthenings by age 14 when
require at least two lengthenings. The goal of each lengthen- the child starts high school education. If a fourth lengthening
ing depends on the total discrepancy at maturity. The safe is required it can be done around age 16 years. Patients may
range of lengthening is from 5 to 8 cm, as long as a good also have lengthenings performed later in life as an adult.
physical therapy program is available. This amount seems to One severe case treated by the author equalized a 25-cm dis-
be independent of the initial length of the femur and age of crepancy with two lengthenings over a nail. If the tibia is also
the child, and can be performed safely even in a toddler. In short, a combined femoral and tibial lengthening can allow
most cases, the maximum of 8 cm is attempted because of a for greater total lengthening amount in a shorter time frame.
large total discrepancy, as long as the patient maintains ade- However, a combined lengthening may not be able to achieve
quate knee range of motion. as much total length as two separate lengthenings, and
The first lengthening of the femur can be performed 12 growth inhibition in the tibia has been reported [62]. In addi-
months after the preparatory surgeries, assuming that the tion, with the advent of smaller and more reliable implant-
femoral neck has ossified. If the preparatory surgery is per- able nails, in the author’s experience, internal lengthening
formed between the ages of 2 and 3 years, then the first can be started as early as 7 years of age.
lengthening can follow between the ages of 3 and 4 years.
However, if the femur is excessively short (total femoral Step 3: Hemiepiphysiodesis and Epiphysiodesis
length <75 mm) for an external fixator or if the femoral neck Contralateral epiphysiodesis around the knee is used as an
fails to ossify, then it would be beneficial to wait for a year or adjuvant method to equalize LLD. It should be calculated
two for more growth. By beginning lengthening at a young into the total strategy of equalization surgeries, and can be
age, the level of prosthetic or orthotic need is reduced earlier used for up to 5 cm of LLD equalization. Judicious use in
in a child’s life. In the senior author’s experience, the compli- some cases may avoid the need for one lengthening. For
cation rate for limb lengthening is no different for the example, a predicted discrepancy of 12 cm may instead be
younger age group [61]. Lengthening of the femur in chil- treated with only one 7-cm lengthening and a 5-cm epiphys-
dren younger than 6 years may be associated with sustained iodesis around puberty. Calculation of the timing of epiphys-
growth stimulation [62]. iodesis can be achieved quickly and accurately using the
Between 4.5 and 7 years, children may have more diffi- multiplier method [26].
culty psychologically dealing with limb lengthening. This is Ipsilateral hemiepiphysiodesis is very useful to correct
related to the normal cognitive stages of children at this age. the valgus deformity of the knee from distal femoral or prox-
Children at this age group seem to understand too little and imal tibial origins. The 8-plate, developed by Peter Stevens,
too much at the same time. They are beginning to be more or a similar device, is a simple way to temporarily arrest the
independent and may appear to be mature enough to handle one side of physeal growth [63, 64]. Correction of the valgus
the process. They understand that they have one short leg. deformity of the femur permits future implantable lengthen-
They do not connect the recognition of a short leg with the ing of the femur since there is no angular deformity.
solution of limb lengthening. Their cognitive level is insuf-
ficient to understand why their parents allowed someone to
do this to them. The younger children do much better because
their cognitive level accepts everything their parents decide Box 22.6. Planning Surgery for the CFD Patient
without questioning. Beyond the age of 6 or 7 years, the • Determine the classification and total discre-
child enters the age of reason and begins to understand that pancy of the deficiency to plan out a timeline of
there is a problem with a solution. Their cooperation is vol- surgeries.
untary rather than coerced. One way to explain this to chil- • Have a low threshold to perform preparatory sur-
dren is to focus on the size of their shoe lift. It is easier for geries of the hip and knee to prevent subluxation or
them to understand that they can wear a normal shoe after the dislocation, which can be disastrous.
operation instead of understanding that their leg lengths will • Do not lengthen until the femoral neck has ossified.
be equal. BMP can be used to promote ossification.
The frequency of lengthening should be spread out to no • The fascia lata must be released or resected before
less than every 3 years and preferably 4 years or greater. The lengthening.
rule of 4 is a good guiding strategy: one lengthening every 4 • The safe range of each lengthening is 5–8 cm.
years starting by 4 years. Assuming that a preparatory sur- • The Rule of 4: First lengthening at 4 years of age
gery is done between ages 2 and 3 years, the first lengthening and then every 4 years thereafter as needed.
can be done between ages 3 and 4 years. The second length- Contralateral epiphysiodesis can be used to
ening would occur around age 8 years and a third lengthen- avoid an extra lengthening.
ing around age 12 years. For psychosocial reasons, it is
The Superhip Procedure

Box 22.7. (continued)
Is the proximal femur bony deformity (see Fig. 22.1) caused procedures as Paley hip, knee, and ankle procedures.
by the hip joint contractures, or vice versa? The net effect in As I learned from my mentor Dr. Robert Salter from
the frontal plane is that the insertion of the abductor muscles his “no name no fame” comment regarding the innom-
(gluteus medius and minimus) on the greater trochanter is inate osteotomy now called the Salter osteotomy, it is
abnormally close to the pelvis. This leads to several prob- not for a surgeon to name this procedure after them-
lems, including impingement of the trochanter on the iliac selves. I have given this procedure a generic name. If
bone and contracture of the gluteal muscles since the dis- others choose to name it after me, then so be it.
tance between their origin and insertion is short. The gluteus My understanding of CFD began with my experience
maximus and fascia lata, with its iliotibial band extension to at the Hospital for Sick Children, Toronto, in 1984.
the tibia, are the most lateral of the soft-tissue structures and I worked with Drs. Robert Gillespie and Ivan Krajbich,
therefore contribute the greatest to the abduction contracture who treated the severe CFD cases by rotationplasty [49].
of the hip. They also contribute significantly to flexion con- In 1986 and 1987, I witnessed reconstructive options for
tracture of the hip. The abduction contracture is not obvious, these deformities in Kurgan, USSR. Drs. Popkov and
since hip adduction is preserved due to the femoral bony Maltzev showed me how to do pelvic support osteotomy
varus deformity, and hip abduction is limited by ilio- to bypass the tethered and deformed hip. The message
trochanteric impingement. If the bony coxa vara is corrected of these experts was that the CFD deformed hip did not
by osteotomy, without soft tissue releases, the abduction lend itself to anatomic reconstruction.
contracture will be uncovered and will prevent the hip from After beginning my practice in 1987 at the
coming back to a neutral position relative to the pelvis, pro- University of Maryland, I tried to find alternative
ducing a fixed pelvic tilt. An abduction pelvic tilt on the reconstructive solutions to the CFD hip. I performed
short leg makes the LLD appear less than before surgery. In many valgus, extension, and internal rotation osteoto-
the face of an open growth plate or a non-ossified neck or mies on young children with this deformity. Initially,
subtrochanteric segment, as in type 1b cases, the abduction they had excellent bony correction with reduction of
contracture leads to recurrence of the coxa vara after osteot- the leg length difference due to a fixed pelvic obliquity
omy. The mechanism for this recurrence may be differential that developed after surgery. Gradually, I watched the
growth of the physis, bending at the non-ossified tissues, or deformity recur, presumably through their proximal
slipped capital femoral epiphysis. femoral physis or unossified femoral neck. I realized
To correct these deformities, a three-dimensional osteot- that the reason for this recurrence was the hip flexion
omy combined with a series of selective soft tissue releases and abduction contractures that existed prior to the sur-
was developed by Paley in 1997. It is now known as the gery. In 1996, I recognized that in order to succeed,
SUPER hip procedure because of its complexity. I needed to untether the proximal femur from the hip
abductors and flexors and then realign the proximal
femur to a neutral position at the hip joint. This was a
radical concept, and I was left with the challenge of
Box 22.7. The Evolution of the Superhip Procedure how to safely release the hip abductors. Borrowing
Dror Paley, MD from the concept of the Hardinge approach to the hip,
The Superhip name originally arose as a billing which took down the anterior third of the hip abductors
code to avoid writing down the multiple CPT codes as a sleeve with the quadriceps muscle, I decided to
that comprised this multistep conglomerate procedure. take down the entire hip abductors with the vastus late-
While there was no intention to call this operation the ralis as one sleeve. In 1997, I performed the first such
Superhip procedure, the name stuck. To avoid the procedure.
name being misperceived the SUPER prefix was then While the psoas, rectus, and TFL were all length-
made into an acronym: Systematic Utilitarian ened as they are today, treating the hip abductors by
Procedure for Extremity Reconstruction. Other releasing the gluteus and vastus tendons off of the
SUPER joint reconstructive procedures for congenital greater trochanter together turned out to be a bad idea.
knee and ankle deformities developed by Paley were While this freed up the proximal femur to rotate into
subsequently renamed the SUPER knee and SUPER neutral by extension, adduction, and internal rotation of
ankle procedures. John Birch of Dallas has expressed the hip joint, it also changed the muscle-tendon length
an aversion to the SUPER prefix and refers to these ratio, thus permanently weakening the hip abductors.
(continued) (continued)
378 D. Paley et al.
Box 22.7. (continued) Box 22.7. (continued)

This is discussed in more detail below. I initially fixed femoral epiphysis (SCFE). In retrospect, we realize
the femur using a Rush rod and a tension band wire. that both were related to the release of the superior
There was no fixation up the femoral neck. When this capsular ligament from the pelvis to the greater tro-
procedure was performed for a subtrochanteric type chanter. Our selective release of the superior capsular
Paley 1b or for a Paley 1a, no recurrence of the defor- ligament demonstrated the importance of this band.
mity occurred since the delayed ossification part was This superior capsular pelvic-trochanteric band is
resected. When it was performed for a neck type Paley essential to prevent the femoral head from moving lat-
1b, the varus usually recurred and the neck did not ossify. erally relative to the acetabulum. After recognizing
In 2001, I switched to using a fixed-angle plate. this, we stopped releasing this band and we no longer
I used the 130° sliding hip screw (Smith and Nephew, experienced dislocations or slips following a Superhip.
Memphis, TN). Some of the cases ossified, but most After 10 years of performing the Superhip, we had
did not. Some of the plates broke and the deformity solved the recurrent varus deformity problem, the
recurred, while in others the plate began to cut through delayed ossification problem, and the dislocation/
the head as the varus recurred. I decided that a blade SCFE problem. We noticed, however, that the children
plate would be the best implant to avoid cutout as well walked with a marked lurch or Trendelenburg gait. We
as to control flexion and extension forces. With the attributed this to the abductor tendon release. By
engineers at Smith and Nephew, I designed an infant detaching the hip abductors from the greater trochan-
and pediatric 130°-angle cannulated blade plate, to ter, we had changed the muscle-tendon length ratio of
correspond to the normal neck-shaft angle. Since 2004, the gluteus medius and minimus muscles, which was
I have been using this new plate. This minimized difficult to recover. Essentially, we had added tendon
recurrent deformities, but incomplete ossification of length to the glutei due to the quadriceps tendon mov-
the femoral neck was the usual result. ing proximally. To solve this problem in 2008, I began
It was apparent that while we could correct this com- to perform the abductor muscle slide off of the ilium
plex deformity, we could not get the delayed ossifica- instead of the distal tendon lengthening. This pre-
tion of the cartilage to ossify. To get some of these failed served the muscle-to-tendon length ratio, keeping the
cases to ossify, I resorted to insertion of BMP up the muscle tendon length and tension constant after sur-
femoral neck into the non-ossified cartilage. The result gery. The slide is achieved by shortening the height of
was dramatic: the recalcitrant-delayed ossification cases the iliac crest. As an added benefit, the glutei are a
ossified. Since BMP-2 (Infuse, Medtronic, Memphis, peculiar group of muscles since they have a growth
TN) was not FDA approved to use in children, we were plate connected to them that grows in a direction that
initially hesitant and reserved in its application. We only lengthens the muscles. After the repair of the abductor
used it to salvage previously failed Superhips. With the slide, the iliac apophysis can increase the tension and
unexpected success that we saw from such application, re-elevate the origin of this muscle. Since adding the
I decided to apply it to new cases in 2006. The results abductor slide, patients do not have the previously
were equally remarkable. All of the necks ossified and noted lurch or Trendelenburg gait.
there were almost no recurrent deformities. Clearly,
we needed to combine a mechanical with a biologic
solution to solve the CFD deformity puzzle.
The soft tissue part of the operation also went Superhip Surgical Technique (Figs. 22.7, 22.8,
through an evolution to its present state. In the first 22.9, 22.10, and 22.11)
5 years, we not only employed release of the conjoint
tendon off of the greater trochanter to treat hip abduc- 1. Positioning, prepping, and draping. An epidural is
tion contracture, but we also released the hip capsule placed by the anesthesia service with a catheter running
off of the greater trochanter. This was done in an extra- up the back on the non-operative side. A Foley catheter
articular fashion so that the joint was never exposed. is placed and also routed to the non-operative side. The
No initial consequence of this was observed. However, patient should be moved to the edge and foot of the
when we started to lengthen femurs that had previously radiolucent table in a supine position. The ipsilateral
had a Superhip procedure, we encountered two new arm should be appropriately padded and placed across
complications: hip dislocation and slipped capital the patient’s chest. A radiolucent bump (usually a
folded towel or sheet) is placed beneath the ipsilateral
(continued) ischium to roll the pelvis 45° towards the opposite side.
Fig. 22.7 Superhip surgical technique illustrations. (a) Straight mid- arthrogram to visualize femoral head. (j) Lateral view shows concentric
lateral incision from top of iliac crest to below tuberosity of knee. (b) rings of ossific nucleus, femoral neck, and femoral head, which should
Reflect fascia lata-iliotibial band distally and leave attached at Gerdy’s be positioned in a bull’s-eye pattern. (k) Insert first guide wire from tip
tubercle. (c) Release rectus femoris tendon, decompress femoral nerve, of greater trochanter to center of femoral head. (l) Insert second guide
and recess iliopsoas tendon. The TFL muscle remains attached to the wire at 45° to first. (m) Geometric rationale behind 45° angle.
ASIS. (d) Decompress sciatic nerve and release piriformis tendon at Neck-shaft angle is 130° and medial proximal femoral angle is 85°.
greater trochanter. (e) Adduction is limited by hip abductor muscles. (f) Therefore the angle between these must be 45°. (n) The second guide
Split apophysis and do abductor muscle slide. (g) All extra-articular wire is centered in the femoral head and neck on both views. (o) Insert
tethers released. Hip can be placed in neutral position. (h) The quadri- a cannulated chisel up the femoral neck. (p) The chisel should be
ceps muscle is elevated to expose the lateral and anterior femur. (i) Hip perpendicular to the posterior aspect of the greater trochanter.
Fig. 22.7 (continued) (q) Replace the chisel with a 130° blade plate. (r) it with the proximal femur. (v) Overlap the bone ends and mark the level
The blade plate is in place. Note the flexion deformity of the femur shaft of overlap. The femur cannot be reduced due to the tethering medial and
relative to the plate. (s) Cut perpendicular and parallel to the plate. (t) posterior soft tissues. (w) Perform a shortening osteotomy of the femur
Perform a second osteotomy oblique to the femur. More recently this and reduce the femur to the plate. (x) Fix it with three diaphyseal screws
osteotomy starts inside the notch that was removed eliminating the and one interfragmentary neck screw. (y) Drill a channel parallel to the
medial lip. (u) Rotate the distal femur internally and abduct it to realign blade. (z) Insert BMP-2 collagen sponges up the femoral neck
Fig. 22.8 Paley modification of Dega osteotomy. (a) The cut should resected from the femur to fit into the opening wedge space. (e) Resect
start 2–3 cm proximal to the lateral acetabulum converging on the trira- the top of the iliac wing to reduce the tension on the hip abductors so
diate cartilage medially. (b) The cut should be parallel to the acetabu- that the apophysis can be sutured closed. This is part of the abductor
lum circumferentially stopping at the triradiate between the ilium and slide. (f) After the resection. (g) Transfer the rectus femoris to the TFL.
the ischium. (c) The roof of the acetabulum is levered down to cover the (h) Close the apophysis
femoral head such that the sourcil is horizontal. (d) Fashion the bone
Fig. 22.9 CFD Paley type 1b with delayed ossification of femoral neck 4 years with Smith & Nephew Modular Rail System (Memphis, TN)
(a, b). MRI showing the cartilaginous femoral neck that remains unos- external fixator with articulation across the knee joint (g). Eight centi-
sified at 2 years of age (c). Superhip with Superknee procedure at 2 meters of lengthening achieved (h). Removal of external fixator with
years of age, including insertion of BMP in femoral neck (d). The neck Rush rodding of bone to prevent fracture (i). At the age of 8 years she
is fully ossified by 3 years of age. Note the significant growth that has underwent a second lengthening, this time using the PRECICE (Ellipse
occurred since the year before (e, f). First lengthening performed at age Technology, Irvine CA) 8.5-mm-diameter lengthening nail (j)
382 D. Paley et al.
Fig. 22.10 Two-year-old girl with CFD Paley type 1b with delayed zation. This X-ray is 1 year after the Superhip and Superknee showing
ossification and severe angulation of the subtrochanteric level of the left significant growth and remodeling which is why the screws are protrud-
femur (a). MRI showing the delayed ossification of subtrochanteric ing medially (c). Lengthening of the femur was performed at age 4
region. The neck is normally ossified (b). The deformity is fully cor- years (d). X-rays after lengthening of the femur 6 cm and insertion of
rected and the femur is healed after the Superhip surgery. The knee has Rush rod at the time of removal surgery (e)
full extension following posterior capsulotomy and Superknee stabili-
Fig. 22.11 (a) Erect leg radiographs of a 22-year-old woman with pre- recovery. (h) Ganz PAO modified for Superhip procedure. Start by oste-
viously untreated CFD and 25-cm discrepancy. She has marked coxa otomizing the iliac crest and peeling off the abductor muscles to do an
vara and acetabular dysplasia as well as rotatory subluxation of the knee abductor muscle slide. (i) The five bone cuts of the Ganz seen from the
joint and dislocation of the patella. (b) Clinical picture before any sur- lateral side. (j) The acetabulum is internally rotated to gain posterior
gery with and without her prosthesis. (c) AP pelvis X-ray showing the coverage. (k) The acetabulum is then abducted to gain lateral coverage.
hip deformities. (d) AP knee X-ray showing the rotatory subluxation of (l) The iliac wing is shortened, and then the crest is reattached using the
the knee. (e) AP and lateral radiographs after Superhip procedure with same screws that are used to fix the PAO, 3 antegrade and one retro-
Ganz periacetabular osteotomy (PAO) and Superknee procedure for grade. (m) Radiograph after first lengthening of femur and tibia (6-cm
reduction and stabilization of the knee and patella. (f) Active hip abduc- femur and 5-cm tibia). The femur and tibia were both rodded using
tion after recovery. (g) Active hip flexion and knee extension after Rush rods at the time of fixator removal to prevent fracture
384 D. Paley et al.
The bump should not be beneath the iliac crest or lower Distally, reflect the flap to the patella if no ligament
back. The entire side should be prepped and draped free reconstruction is to be done and all the way to the medial
from the nipple to the toes. The drapes should extend side if ligament reconstruction is to be done. The fascia
from the crack of the buttocks to the fold between the lata is now fully exposed from the patella to a couple of
scrotum or labia and the thigh. The lower limb should be centimeters posterior to the intermuscular septum dis-
completely free of the drapes. This is called a forequar- tally and from the anterior edge of the TFL to the mid-
ter prep. gluteus maximus proximally.
2. Incision. With the leg fully extended, a long mid-lateral 4. Fascia lata release. The fascia is incised at the TFL-
incision is made from the top of the iliac crest to the sartorius interval making sure to stay on the TFL side in
tibial tuberosity. The incision is kept as straight as pos- order to avoid injury to the lateral femoral cutaneous
sible, passing over the proximal femoral “bump” and nerve. The fascial incision is extended distally to the lat-
continuing longitudinally towards Gerdy’s tubercle eral border of the patella ending at the tibia. The poste-
before curving slightly anteriorly towards the tibial rior incision of the fascia lata starts distally and posterior
tubercle. The incision is carried down to the depth of the at the intermuscular septum and extends proximally to
underlying fascia lata and iliotibial band. overlie the gluteus maximus in line with the incision.
3. Flap elevation. The subcutaneous tissues and skin are The gluteus maximus (GMax) should be separated from
elevated as one large flap anteriorly and posteriorly off the overlying fascia anterior to the posterior fascial inci-
the fascia of the thigh and pelvic region. The fat is adher- sion. The fascia should be retracted anteriorly and away
ent to the fascia and should be dissected preferably with from the underlying muscle, while the GMax should be
an electrocautery. The electrocautery should be held flat, dissected off of the fascia and the intermuscular septum
parallel to the plane of dissection, and can be quite tech- that separates it from the TFL. The GMax should not be
nically difficult. It is important not to incise or damage split in line with the fascial incision to avoid denervating
the fascia if it is being used for knee ligament recon- the muscle anterior to the split. It can now be reflected
struction. Dissection may also be carried out with scis- posteriorly to allow exposure of the greater trochanter,
sors. Anteriorly, the flap is extended medial to the piriformis muscle, and sciatic nerve.
Smith-Peterson interval (tensor fascia lata (TFL) and If knee ligamentous reconstruction is planned, the
sartorius) proximally. Posteriorly, the subcutaneous flap fascia lata is cut proximally and anteriorly at the muscu-
is elevated to just posterior to the intermuscular septum. lotendinous junction. The fascial cut should be a step cut
or sloped posteriorly and proximally to include a longer The greater trochanter should be identified by palpation.
fascia segment posteriorly from the fascia that was dis- The gluteus medius muscle posterior border is very dis-
sected off of the GMax. The fascia lata is reflected distinct and proceeds down to the greater trochanter where
tally to Gerdy’s tubercle. The TFL can be left in place it inserts. Deep to the medius is the gluteus minimus,
without further dissection. It does not have to be sepa- and just distal to the minimus is the piriformis muscle.
rated from the underlying gluteus medius (GMed). The Its tendon can be palpated through its muscle. It may be
two muscles are often adherent to each other, and it may difficult to identify the piriformis from the minimus.
be difficult to differentiate the muscle fibers. The distin- Care should be taken to avoid dissection at the distal
guishing feature is that the GMed fibers insert on the border of the piriformis tendon. This is where the medial
greater trochanter while the TFL does not. The distal femoral circumflex branch anastomoses with the infe-
fascia lata becomes the iliotibial band and blends with rior gluteal artery branch. The entire piriformis is tran-
the underlying lateral knee capsule, which may be par- sected about 1 cm from its insertion onto the trochanter.
tially reflected with the iliotibial band. The fascia should The sciatic nerve can be identified and if necessary
be mobilized all the way until Gerdy’s tubercle. The fas- decompressed. It is more posterior to the trochanter and
cia can then be divided into two halves using a straight runs deep to the piriformis.
pair of scissors. It should be kept moist while the rest of 7. Abductor muscle slide. The abductors may not appear to
the surgery proceeds. The two limbs of the fascia are be tight on first inspection because of the coxa vara.
ready for later use in the Superknee procedure. Adduction into a true AP of the hip, with the neck ori-
5. Hip flexion contracture releases. The dissection is car- ented normally in the acetabulum, is now restricted by
ried beneath the sartorius to find the rectus femoris ten- the gluteus medius and minimus since the fascia lata has
don. The rectus femoris tendon insertion is identified at already been cut. Furthermore, the Dega osteotomy,
the anterior inferior iliac spine. The constant ascending which lengthens the height of the ilium, makes the
branch of the lateral femoral circumflex artery and vein abductors even tighter. As noted previously, the abduc-
is cauterized prior to cutting the tendon. The conjoint tors should be detached at their origin and not their
rectus femoris tendon (distal to the split into reflected insertion. This avoids changing the muscle-tendon
and direct heads) is cut and allowed to retract distally. length ratio and avoids weakening the hip abductors,
Care should be taken not to go too distal on the rectus avoiding a lurch or Trendelenburg gait.
femoris to avoid injury to its innervating branch of the The subcutaneous tissue flaps should be elevated to
femoral nerve. Just medial to the rectus is the iliopsoas provide adequate exposure to the iliac crest apophysis.
muscle. The iliocapsularis muscle (capsular origin head There is a tendency to have inadequate posterior expo-
of iliopsoas muscles) can also be seen here. The femoral sure, and the flaps should be elevated just beyond the
nerve lies on the anteromedial surface of the iliopsoas highest lateral point of the apophysis. The anterior extent
muscle. Before looking for the psoas tendon, the femo- is the anterior superior and inferior iliac spines, which has
ral nerve should be identified and decompressed below been exposed for the release of the rectus femoris tendon.
the inguinal ligament. The posterior aspect of the ilio- The abdominal external oblique muscles are partially
psoas muscle belly is now elevated from lateral to released off of the entire length of the apophysis. Once the
medial. The psoas tendon is located on the posterome- apophysis is bare, it can be split from the ASIS to
dial surface in the substance of the muscle. The tendon the AIIS, and then split from anterior to posterior along
is exposed and cut. Any remaining flexion contracture of the iliac crest. This should be done with a #15 blade. To
the hip is due to the gluteus medius and minimus (the know where to split, pinch the apophysis between thumb
part of these muscles originating anterior to the center of and index finger of the hand not holding the knife. Stay in
rotation of the femoral head in the sagittal plane), and the middle of the apophysis along its entire length, push-
the anterior fascia of thigh. The release of the gluteus ing down hard with the knife blade until one feels bone.
medius and minimus muscles is accomplished by the Using a periosteal elevator, pop off the apophysis from
abductor muscle slide technique (see step 7). If the ante- the ilium. This should be done at multiple sites to get the
rior thigh or sartorius fascia is still tight, it can also be entire apophysis to peel back as a unit from the ilium. The
released, taking care not to injure the lateral femoral apophysis and lateral periosteum are reflected distally,
cutaneous nerve, which should be identified and decom- thus relaxing the abductor muscles. The medial half of the
pressed. It runs inside the fascia covering the sartorius apophysis is reflected medially with the iliacus muscle.
muscle just medial to the anterior superior iliac spine. Since some of the abductors act as flexors of the hip, the
6. External rotation contracture release. The piriformis abductor slide helps eliminate any remaining flexion
tendon is contracted and prevents internal rotation of the deformity of the hip. Furthermore, the iliacus muscle
hip. It should be released off of the greater trochanter. slide also relaxes any residual tension in this muscle.
386 D. Paley et al.
8. Elevation of quadriceps. The quadriceps is now elevated between the two wires. This will be the length of the
off of the femur in a subperiosteal fashion. Since the blade of the blade plate to be used. The position of the
femur is so short, the exposure may extend as far as the neck wire can also be confirmed by flexing the hip to 90°
distal femoral physis. The perforator vessels need to be and looking at a frog leg lateral of the neck.
cauterized as the quadriceps is detached from the linea 11. Blade plate insertion. The cannulated chisel for the
aspera. Proximally, the vastus lateralis should be ele- blade plate should now be hammered up the femoral
vated off of part of the cartilage of the greater trochan- neck guided by the second guide wire. The chisel should
teric apophysis by sharp dissection. be rotated until it is perpendicular to the posterior aspect
9. Arthrogram. A hip arthrogram is now performed using a of the greater trochanter. This will guide it to the correct
20-gauge spinal needle. With the trocar inside, the nee- angle in the sagittal plane. Tap the chisel out of the
dle is placed into the hip joint from the anterolateral femur and reinsert the guide wire in its previous posi-
side. Traction of the femur may facilitate placement. tion. Insert the appropriate length 130° blade plate over
Once the needle appears to be in the joint on the image this wire to the depth of the bend of the plate. Make sure
intensifier, the trocar is removed and normal saline is on the image intensifier that the tip of the blade is not too
injected. If the needle is in the joint the saline should deep into the femoral head. Check its position on AP
go in with little pressure, and when the syringe is and lateral planes, as well as using the approach-
removed from the needle, the saline should drip back withdrawal technique with live fluoroscopy to ensure
out. These signs confirm that the needle is in the joint that the blade is not advanced too close to the articular
space. The arthrographic dye can now be injected into surface of the femoral head. If the blade is suspected of
the hip joint to outline the femoral head, acetabulum, being too long, then replace it with one with a shorter
and femoral neck. length. Furthermore, if plate placement is off center,
10. Guide wire insertion. Since the abduction, flexion, and there is greater risk of protrusion into the joint.
rotation contractures have all been released, the femoral 12. Subtrochanteric osteotomy. The femur should be cut
head and neck can now be placed in a neutral orientation perpendicular to the shaft of the plate, starting just below
to the pelvis by extending and maximally adducting the the bend in the plate. This cut should be below the level
lower limb across the opposite leg. A guide wire should of the greater trochanter cartilage. To guide this cut, drill
now be drilled up the center of the femoral neck to guide a wire perpendicular to the plate. Keep the plane of the
the insertion of a fixed-angle fixation device. Since the saw blade perpendicular to the plate in all planes. The
femoral neck is unossified and short, it is very difficult width of the perpendicular cut surface should be as wide
to drill a guide wire at the correct angle up the femoral as the femoral diaphysis. If the deformity angle is very
neck. The goal is to create a 130° neck-shaft angle and a large, it may be parallel to the lateral cortex. A second
medial proximal femoral angle (MPFA) of 85°. In the subtrochanteric osteotomy should be made oriented less
normal femur, the angle between the neck-shaft line and than 90° to the first osteotomy towards the base of the
the tip of the greater trochanter to center of femoral head femoral neck to remove the bone protruding medially.
line is 45°. The femoral head and neck can be manually tested for
The first guide wire is inserted from the tip of the any residual impingement, removing any blocks to
greater trochanter to the center of femoral head. Since achieving 90° of hip flexion. The anteromedial corner
the tip of the trochanter is cartilaginous in young chil- often needs to be excised to prevent impingement.
dren, it cannot be seen radiographically and the tip of the 13. Periosteum release. After the second osteotomy, the dis-
trochanter is located by palpation using the wire tip. tal femur is easily exposed and the surrounding perios-
From this point, the wire is then drilled towards the cen- teum is peeled off circumferentially. Medially, it is very
ter of the femoral head as shown in the arthrogram. The thick and restricts correction of the varus and rotation
image intensifier is placed into the lateral view and the deformity. Cut the periosteum transversely around the
leg rotated until a bull’s-eye of three concentric circles is femur, carefully separating it from the surrounding mus-
seen. This is formed by the overlapping dye shadows: cle. Be careful to avoid injury to the profunda femoris
the outermost circle is the femoral head, the middle cir- and its perforators, which pass immediately under the
cle is the femoral neck, and the inner circle is the ossific periosteum. Cutting the periosteum allows the thigh to
nucleus of the femoral head. A second wire should be stretch longitudinally, reducing the amount of shorten-
drilled into the center of the bull’s-eye at a 45° angle to ing required of the femur.
the first wire. Using a depth gauge or another wire of the 14. Femoral shortening. The distal femur is now mobile and
same length as the second wire, measure the amount of can be corrected into valgus and rotated internally. The
wire inside the femoral neck by placing it alongside the distal femur is too long to fit end to end with the proxi-
second wire and measuring the difference in length mal femoral cut. The two ends should be overlapped,
and a mark should be made at the point of overlap. The radiographically after the cannula is removed. It should
distal femur should be shortened at this level. A wire is be emphasized that such use of BMP (Infuse implant) is
drilled perpendicular to the femur at the level of the oste- an off-label use of this product.
otomy and a saw is used to cut, using frequent irrigation 17. Pelvic osteotomy. Surgical technique is covered later in
to prevent heat necrosis. The segment of bone is kept this chapter. The type of pelvic osteotomy depends on
moist on the back table for use as bone graft for the Dega the age of the patient and the degree of dysplasia. In
osteotomy. This is usually a trapezoid-shaped piece most patients under age 6 years, the Dega osteotomy is
about 1–1.5 cm long. preferred. In hips with severe dysplasia, a periacetabular
15. Distal femur fixation. The femur is now brought to the triple osteotomy or a Ganz periacetabular osteotomy is
plate. The bone ends should oppose without tension. used depending on the closure of the triradiate cartilage.
The femur is internally rotated to correct the external With both periacetabular osteotomies, the iliac wing
torsion deformity. To adjust the femur to the correct shortening osteotomy should be performed prior to fixa-
anteversion, the guide wire should be reinserted into the tion of the acetabular fragment. Consider doing the pel-
cannulation of the plate to show the orientation of vic osteotomy prior to shortening the distal femur
the femoral neck. The knee should be flexed to 90° and segment, as the amount of shortening needed may
the angle between the wire and the frontal plane of the change after the pelvic osteotomy. Furthermore, the pel-
femur as judged by the perpendicular plane to knee flex- vic osteotomy changes the rotation of the hip, since it
ion is observed. This wire should appear at least 10° rotates the capsule. Thus, the amount of rotation changes
anteverted, relative to the knee. The most distal hole in if the femur fixation is done after the pelvic osteotomy.
the plate can now be drilled with the femur held in this 18. Iliac wing osteotomy. After the pelvic osteotomy, the
rotation. The drill hole should be made at the distal edge apophysis can be sutured back together. Due to the
of the hole to compress the osteotomy with screw inser- abductor muscle slide, the lateral apophysis cannot
tion. Two more screw holes are drilled and screws are reach the top of the iliac crest. Part of the crest has to be
inserted. The most proximal hole in the plate is designed resected to allow repair of the apophysis. Putting
to drill parallel to the blade of the plate and secures the traction on the lateral apophysis, one can see and mark
plate to the proximal femur. The wire in the cannulated the level to which it reaches. A saw is used to cut and
hole can be used to guide the drill bit. In type 1b cases, remove the proximal part of the iliac wing, effectively a
the blade of the plate and the oblique screw goes across shortening osteotomy. The removed bone is used as
the proximal physis and into the femoral head. In type graft for the acetabular osteotomy or the subtrochan-
1a cases with a horizontally oriented growth plate, nei- teric femoral osteotomy. The apophysis is then repaired
ther the blade nor the screw should cross the growth with a running #1 Vicryl suture. The medial and lateral
plate of the upper femur. In type 1a cases with a verti- halves of the apophysis should be well opposed to avoid
cally oriented growth plate, the blade but not the screw a bifid iliac wing. The external abdominal muscles
should cross the physis. should be advanced and repaired over the apophysis
16. BMP insertion. In type 1b neck cases, BMP should be after the closure.
inserted into the upper femur to stimulate ossification of 19. Muscle repairs and transfers. The TFL muscle is
the cartilaginous neck of the femur. A wire is drilled repaired to the rectus femoris tendon. This essentially
proximal and parallel to the guide wire in the cannula- elongates the rectus femoris while allowing it to main-
tion of the plate. A 3.8-mm hole is then drilled over this tain its origin on the pelvis. The interval between the
guide wire. The drill hole should extend all the way into TFL and the sartorius is closed, carefully avoiding the
the ossific nucleus. BMP-2 (Infuse, Wright Medical) is lateral femoral cutaneous nerve. The quadriceps is
then prepared on collagen sponges that are manually sutured to the region of the linea aspera. Finally, the glu-
pushed into the hole with a 3.2-mm drill and forceps. A teus maximus is advanced back to the posterior border
more recent method of insertion of the BMP is to use a of the TFL.
cannula, such as the Craig needle biopsy tool, which is 20. Closure. If no knee releases or reconstruction is required,
4 mm in its outer diameter. The BMP sponge is labeled the fascia lata should be resected from Gerdy’s tubercle.
by applying radiographic contrast dye onto its surface. The wound can now be closed. Since there is no fascia
The sponge is then inserted into the end of the cannula, lata, the deepest layer is the subcutaneous fat layer,
and the cannula is inserted into a 4-mm predrilled hole called the “underlayer.” One or two medium-sized drains
in the femoral neck. Once the cannula is located at the are placed, exiting anterosuperiorly. The second drain is
desired depth, a blunt trocar is used to push the sponges laid medially if a Superknee procedure is performed.
out of the cannula into the femoral neck. Since the The drains are usually secured with a clear adhesive
sponges are labeled by contrast dye, they can be seen sterile dressing (e.g., Tegaderm, 3 M, Minnesota).
388 D. Paley et al.
It is important to close the wound in a fashion that the Postoperative Course

opposite layers get sutured at the same level. The deep
edges of the subcutaneous underlayer are brought Parents are initially educated on transfers, cast care, and
together with a running #1 braided absorbable suture. hygiene. The bivalved spica cast can be converted into a
The Scarpa’s fascia is closed with a running 2-0 braided removable cast in about 1 week. The patient can then start
absorbable suture. The deep dermal layer is closed with gentle passive flexion and extension of the hip from 0 to 90°,
a running 3-0 braided absorbable suture, and the skin is as well as passive abduction. The spica cast is discontinued
closed using a subcuticular 4-0 monofilament suture. after 6 weeks, and if radiographs show adequate bony heal-
Dermabond™ may be used, and sterile dressings are ing, then the patient is progressed to full weight bearing, gait
applied. training, strengthening, and active range of motion. The end
Final radiographs are taken: an AP pelvis and lateral goal is to restore the child to normal function before they
knee, both including the femur. The patient is then proceed with limb lengthening.
placed into spica cast. The operative limb should be
placed in full hip and knee extension with the foot and
ankle left free. The opposite limb can be in a flexed, Pelvic Osteotomies
abducted, and externally rotated position, with the cast
stopping short of the knee. Alternatively, a one-leg spica Paley Modification of Dega Osteotomy
with a molded pelvic band can be used. The cast should (Paley-Dega Osteotomy) (See Fig. 22.8)
be bivalved before leaving the operating room.
In the Superhip procedure, the iliac apophysis has already
been split and the periosteum from the lateral and medial
aspects of the ilium has already been reflected back with its
overlying muscles. The lateral periosteum is reflected back
Box 22.8. Superhip Tips and Tricks to the edge of the acetabulum and to the sciatic notch. The
• Pay careful attention to soft tissue handling, making periosteum should be dissected off the anterior wall of the
sure that the subcutaneous flaps are cleanly dissected sciatic notch, feeling for the soft cartilage of the triradiate
to preserve their integrity and prevent fat necrosis. cartilage where it separates the ilium from the ischium.
• The lateral femoral cutaneous nerve is usually Using the image intensifier, a guide wire is drilled approxi-
found just under the sartorius fascia and originates mately 2 cm proximal to the lateral edge of the acetabulum
from deep to the inguinal ligament. towards the triradiate cartilage medially. On the lateral wall
• The femoral nerve lies on the anteromedial side of of the ilium, the osteotomy is a curved line running
iliopsoas muscle. The tendon lies posteromedial. perpendicular to the triradiate cartilage, separating the ilium
• Do not dissect distal or deep to the piriformis ten- from the ischium. It runs parallel to the anterior wall of the
don to avoid interrupting the inferior gluteal anasto- sciatic notch and extends proximally to the level of the guide
mosis with the medial femoral circumflex artery. wire. The cut curves to make a 90-degree bend heading
• The apophysis should be split with a single continu- towards the superior border of the anterior inferior iliac spine.
ous cut, pushing hard down to bone. Multiple passes In the frontal plane, the osteotomy is inclined from proximal
will piecemeal the apophyseal cartilage. to distal, parallel to the guide wire. The osteotomy ends at the
• Palpate the back of the greater trochanter while triradiate cartilage medially. It is easier to start distally and
placing the chisel and blade plate to make sure that work proximally up to the guide wire. At the most posterior
they stay perpendicular to its posterior border in the distal part of the osteotomy, it is important to stay very near
sagittal plane. the sciatic notch to avoid entering the hip joint inadvertently.
• The extent of the flexion and adduction deformity The osteotomy is levered distally to bring the roof of the
should be fairly evident by the orientation of the acetabulum down. A laminar spreader is used to distract the
blade plate to the uncut femur. Greater than 45° of osteotomy. The vertical limb of the osteotomy extending
flexion deformity and up to 90° of varus deformity down to the triradiate cartilage at the ischium allows for
are not uncommon. greater bending and greater lateral coverage. It is a myth that
• Place the guide wire in the cannulated plate to help the Dega osteotomy gives posterior coverage. It cannot do so
guide the rotational correction and BMP hole place- because it cannot extend distal or posterior than the posterior
ment, and determining femoral anteversion. limb of the triradiate. The original Dega, as well as subse-
quent publications of this osteotomy, goes through the inner
Fig. 22.12 (a) Periacetabular triple osteotomy lateral view (Paley). rior coverage and treat the retroversion of the acetabulum. Lateral view.
The bone cuts are outlined. The sacrospinous ligament is also released (e) AP view of internal rotation of osteotomy. (f) Abduct the osteotomy
to untether the acetabulum. (b) Periacetabular triple osteotomy, AP to gain lateral coverage. (g) Abduct the osteotomy to gain lateral cover-
view (Paley). (c) The ischium is cut under direct vision after identifying age. (h) Resect the upper end of the iliac wing to allow closure of the
and decompressing the sciatic nerve. The nerve is not disturbed since hip abductors. (i) Fixation is with several antegrade and one retrograde
the bone is deeper. (d) Internally rotate osteotomy to gain better poste- screw. (j) Front view of coverage and fixation
table of the pelvis, especially anteriorly. The Paley-Dega At this point, the femoral shortening should be carried out
modification avoids doing so and also extends farther poste- and the excised bone segment cut to fit the opening wedge
rior and distal than other publications of the Dega. In this gap of the pelvic osteotomy. Additional bone from the iliac
manner, there is true hinging in a three-dimensional way on crest, which is resected during the abductor slide, may be
the triradiate. Even the anterior part of the cut does not exit inserted to fill the remaining space.
the cortex and extends to the triradiate junction between the
ilium and pubis.
The bump under the buttocks should be removed at this Paley Modification of Periacetabular Triple
stage. This allows one to assess the coverage of the femoral Osteotomy (Paley PATO) (Fig. 22.12)
head in the true frontal plane. The laminar spreader can be
distracted as needed to gain additional coverage and reorien- 1. Pubis: The pubis is exposed subperiosteally by reflecting
tation of the acetabular sourcil. The laminar spreader should the medial iliac apophysis and periosteum more medially.
be kept posterior to avoid increasing the anterior coverage. The triradiate cartilage arm at the pubis is exposed and the
In this manner, predominantly lateral coverage is augmented. dissection carried out just distal to it. The superior pubic
390 D. Paley et al.
ramus can be cut under direct vision under the protection the base of the iliac wing from anterior to posterior. A same
of two Hohmann retractors with an osteotome or a saw. sized drill bit is used to compare lengths with the drill bits
2. Ischium: The ischium is exposed by returning to the back inside the bone to determine the screw lengths. One by one,
of the femur and finding the sciatic nerve again. The nerve the drill bits are removed, and replaced with solid,
is followed to the ischium. To avoid stretching the nerve, non-cannulated screws, making sure that they do not enter
do not place a retractor between the nerve and the ischium. the triradiate cartilage or the hip joint.
Subperiosteal elevation of the ischial periosteum is car-
ried out. The ischium should be cut under direct vision
near the junction where it forms the inferior wall of the Ganz Periacetabular Osteotomy (PAO)
acetabulum, distal to the triradiate cartilage. (See Fig. 22.11)
3. Sacrospinous ligament release: For large corrections, it is
important to release the tether of the sacrospinous liga- The technique for this has been previously published, though
ment. This ligament can be palpated after dissecting the there are some variations when performed with a Superhip
medial iliac periosteum off of the quadrilateral plate procedure. This osteotomy is indicated for patients who are
inside the true pelvis. A finger can be placed on the tip of skeletally or near-skeletally mature, usually with quite severe
the ischial spine, and the ligament is then felt. Using pro- acetabular dysplasia.
prioception, a finger from the other hand can be used to To do the abductor slide, an osteotomy of the iliac crest
palpate the finger that is on the ligament. Make sure that is done from lateral to medial. There is no iliac apophysis to
the sciatic nerve, which can be visualized laterally, is not be split, so the proximal 1 cm of crest is cut with a saw after
in the way. With the ligament isolated in this manner, it first elevating the medial periosteum with the iliacus mus-
can be cut with scissors from the lateral side. cle. The periosteum of the medial side of the ilium is present
4. Ilium: The ilium is cut with a saw from the anterior supe- at every age and dissects off the bone easily, even in older
rior iliac spine towards the junction of the true and false patients. The periosteum on the lateral side of the ilium will
pelvis. On the lateral side of the ilium, it should be oste- not strip off easily after childhood. The glutei fix to bone
otomized towards and into the sciatic notch. On the with Sharpey’s fibers, making subperiosteal dissection
medial side it should be cut with an osteotome into the impossible in adulthood. Therefore, the glutei are not
sciatic notch. The osteotomy on the medial side has an detached from the top of the crest and are stripped laterally
angle of about 120°. This bend in the cut rounds off the after an osteotomy of the crest of the ilium. This includes
corner of the distal segment and creates a spike of bone on the TFL. The sartorius and the iliacus muscles are stripped
the proximal segment. This increases the surface area and medially. A small cube of bone is cut with a saw and
creates a posterior buttress while making the distal seg- reflected with the sartorius.
ment rotate more easily with less distraction. The Ganz PAO consists of five osteotomies of the ilium,
To gain posterior and lateral coverage, the acetabular seg- ischium, and pubis:
ment should first be rotated internally along the long axis of 1. The first osteotomy is of the pubis. The pubis is exposed
the body. This gives posterior coverage. It should then be subperiosteally and cut with a saw and an osteotome. A
rotated laterally. This gives lateral coverage. To rotate inter- sharp Hohmann retractor can be driven into the distal
nally, a lion-jaw bone clamp is placed anteroposteriorly, pubis and retracted to provide better exposure.
superior to the acetabulum and the bone fragment rotated 2. The second osteotomy involves part of the ischium. The
internally. It can also be placed around the pubis to lever the ischium is cut from anterior to posterior through half of
acetabulum laterally. If the acaetabular fragment does not its thickness. A special angled osteotome is used for this.
move sufficiently, then each osteotomy site should be A space is created just distal to the pubis between the
checked for completeness. The ischial osteotomy is often the femur and the ischium. The hip is kept partially flexed at
culprit, and the periosteum around the ischium may need to all times to help protect the femoral nerve. A blunt instru-
be divided. Fixation of the triple osteotomy is achieved using ment can be inserted to feel for the ischium, which is
a long 3.5-mm or 4.5-mm screw from the ilium to the acetab- quite deep. The curved osteotome is inserted down to the
ular fragment. However, because of the abductor slide, the ischium. It is then cut part way through the bone.
proximal part of the iliac wing must be resected prior to 3. The third osteotomy is partway through the ilium. The
screw fixation. The description of how to do this is described periosteum should be dissected off of the quadrilateral
in a later step. Once complete, multiple drill bits can be plate inside the true pelvis. With a finger, the surgeon can
placed from the remaining iliac wing towards the acetabular feel and find the ischial spine and feel the sacrospinous
fragment, from proximal lateral to distal medial. One drill bit ligament. An iliac osteotomy is made from just distal to
is placed from the anterior inferior spine, retrograde towards the anterior superior iliac spine to the brim of the true
pelvis. A mark is placed at the junction of the true pelvis patella dislocates with flexion. Finally, many cases of CFD
with the false pelvis in line with the sciatic notch. An have a fixed flexion deformity of the knee.
imaginary line can be drawn from this point parallel and
just anterior to the anterior wall of the notch, ending at the
level of the ischial spine. This osteotomy can be made Indications for Preparatory Surgery
with a saw. of the Knee Prior to Limb Lengthening
4. The fourth bone cut is made with an osteotome from
the pelvic brim into the true pelvis to end anterior to the Isolated anteroposterior instability is not necessarily an indi-
ischial spine. To avoid stretch to the femoral vessels the cation for reconstructive knee ligament reconstructive sur-
hip should be flexed at all times during this osteotomy. An gery in children with CFD. However, grade 3 ligamentous
osteotome is inserted with its lateral part posterior and its instability with no endpoint on anterior and posterior drawer
medial part anterior. It should be inclined with its handle tests will usually become symptomatic as the child gets
towards the belly. Either by feel or using the image inten- older. If the child is going to undergo a Superhip procedure
sifier, we need to orient this parallel to the anterior wall of or Dega osteotomy prior to lengthening, instead of excising
the sciatic notch and stop opposite the ischial spine. the fascia lata, it can be used to reconstruct the knee liga-
5. The fifth and final osteotomy is the completion of the ments. In some children, there is a catching or locking sensa-
ischial osteotomy. The same curved osteotome used for tion in the knee when going from extension to flexion. It may
the anterior ischium is used here to complete the ischial be painful and require a trick motion to release it. This is due
cut. The osteotome is oriented towards the quadrilateral to contracture of the iliotibial band, combined with aplasia of
plate. It should be inserted to start at the end of the fourth the ACL. In more severe cases, the tibia actually subluxates
cut. Once the ischial cut is complete the acetabular seg- or dislocates anteriorly on the femur and reduces at about
ment is mobilized. 30° of flexion. In older patients, the posterior aspect of the
A lion-jaw bone clamp is placed on the pubis and the tibia may be rounded, contributing to anterior dislocation of
acetabulum is rotated laterally outwards. The segment may the tibia on the femur. It is not clear whether this is a second-
need to be internally rotated due to retroversion. If it will ary change due to chronic dislocation or a primary deformity,
not move sufficiently, there is often a tether at the pubis due since the tibia is not ossified posteriorly in infancy.
to the attached muscles. Releasing these frees the acetabu- Patellar hypoplasia and instability are also very common.
lar segment to move. Once the segment is placed in the The patella frequently maltracks laterally with progressive
desired position, screw fixation is used to secure it. Again, knee flexion. In some cases, the patella may dislocate with
the wing of the ilium should be shortened first to allow the knee flexion. This is due to a combination of factors: valgus
abductors to slide distally. Three antegrade screws and one distal femur, hypoplastic or absent patellar groove, contrac-
retrograde screw (as in the above periacetabular triple oste- ture of the lateral retinaculum with the tight iliotibial band,
otomy) are inserted to secure the fragment. The abductors and external rotatory instability of the tibia on the femur due
are secured back with screws through the crest segment to cruciate ligament deficiency, which lateralizes the patellar
into the iliac wing. tendon insertion. Patellar maltracking or subluxation/dislo-
cation should be corrected prior to limb lengthening.
Flexion contracture of the knee is another congenital
Knee Considerations in CFD deformity that may be present and should be corrected before
proceeding with limb lengthening. When the femur is very
The knee in CFD may range from a normal, stable, unde- short, the acute angle created by the posterior thigh muscles
formed knee to a contracted, unstable, deformed joint. The gives the appearance of a flexion contracture. The definition
most common deformity of the knee is valgus, which is usu- of a flexion contracture is a flexed angle between the anterior
ally nonprogressive. The distal femoral physis is usually cortical line of the femur and tibia in maximum extension.
closer to the knee joint on the lateral side, often due to hypo- When the contracture is greater than 15°, it should be cor-
plasia of the lateral femoral condyle. CFD cases often have a rected surgically. Knee flexion deformity can be due to bony
variable degree of anteroposterior and rotatory instability of or soft tissue causes. In CFD, it is commonly an intra-
the knee related to absent or hypoplastic cruciate ligaments. articular capsular contracture. There may be some extra-
In some cases, the tibia dislocates anterior or posterior on the articular contribution due to contracture of the hamstring
femur during extension or flexion, respectively. Furthermore, muscles and gastrocnemius muscles. Release of these mus-
there may be rotatory instability present. One study has cles alone rarely corrects the contracture while capsular
related the radiographic appearance of the tibial spines to the release without complete hamstring release corrects the con-
degree of hypoplasia of the ACL [27]. The patella is usually tracture. In some cases there is a true bony flexion of the
hypoplastic and may maltrack laterally. In some cases, the distal femur that may need to be corrected by osteotomy.
392 D. Paley et al.
Box 22.9. History of Superknee Procedure Box 22.9. (continued)

Dror Paley, MD useful technique for congenitally deficient knees. In
The knee reconstruction that I developed in 1994 [42] CFD, the instability pattern is different than in an iso-
is called the Superknee procedure. The Superknee is a lated tear of the ACL. There is more of a rotary insta-
conglomerate procedure combining two or more of the bility in CFD. Therefore, a purely intra-articular
following five procedures, three of which were previ- ligament reconstruction is insufficient and can lead to
ously described by other authors, and two of which recurrent instability. Thus, the combination of extra-
were developed by me: (1) the Langenskiöld proce- and intra-articular ACL ligament reconstruction is
dure [65] for congenital dislocation of the patella; (2) ideal. Having studied under MacIntosh and having
the MacIntosh procedure [66] for ACL deficiency learned this procedure directly from him, it was natural
including extra and intra-articular ACL reconstruction for me to think about its application in the CFD cruci-
using the fascia lata; (3) the Grammont procedure [67] ate-deficient knee. I have modified this procedure
for recurrent dislocation of the patella; (4) the Paley slightly to adapt it to the skeletally immature knee. In
procedure also referred to as the reverse MacIntosh my experience, it can now be safely done as young as
[66] to prevent external rotatory instability and to act 2 years of age.
as an extra-articular posterior cruciate ligament; and To prevent the tibia from rotating externally, which
(5) the Paley anterior approach to posterior capsulot- leads to subluxation of the patella and of the tibia on
omy of knee. Combinations of two or more of these the femur posteriorly, a medial extra-articular ligament
five procedures are referred to as the Superknee proce- can be created using the medial half of the fascia lata.
dure and can be performed at the same time as a pelvic This creates a strap going around the medial tibia teth-
osteotomy and/or Superhip procedure. ering it to the medial distal femur. This is the opposite
The Superknee procedure is a combination of some direction of the extra-articular ligament created with
or all of these components including extra- and intra- the posterior half of the fascia lata, which is described
articular knee ligament reconstruction, patellar realign- in the next step. This lateral extra-articular ligament
ment, posterior capsulotomy, and knee flexor tendon was described by Dr. David MacIntosh for ACL recon-
releases. Typically, the Superknee consists of the struction. In his honor and memory (he was one of my
MacIntosh extra- and intra-articular ACL reconstruc- professors and mentors in Toronto in 1985) and in rec-
tion, the reverse MacIntosh (Paley) PCL extra-articular ognition of his idea of a lateral extra-articular liga-
reconstruction, the Grammont patellar tendon realignment, I have referred to the medial extra-articular
ment, lateral release of the patella, and in some cases the ligament procedure as the reverse MacIntosh, or extra-
modified Langenskiöld (Paley) procedure for patellar articular PCL procedure.
reduction. If performed with a Superhip procedure, the
incision is a distal extension of the Superhip incision. If
performed as an isolated procedure, it can be done
through one midline anterior incision or one medial and Superknee Surgical Technique (Ligamentous
one lateral incision. If the Superknee is performed with- Reconstruction Only) (Figs. 22.9 and 22.13)
out the Superhip procedure, the entire surgery can be
performed under tourniquet control. My personal prefer- 1. Fascia lata harvest. The knee is exposed through a long
ence is to use the Hemaclear tourniquet (HEMACLEAR, lateral incision ending just distal to the tibial tuberosity
New Jersey) because of its narrow width. There is typi- distally and midlateral proximally. The anterior margin
cally not enough room in a young patient with a con- of the fascia lata (iliotibial band) and the posterior mar-
genital short femur for a pneumatic tourniquet to be gin where it blends with the intermuscular septum are
placed proximal to the surgical incision. The release of incised longitudinally. The fascia lata is transected as at
the posterior capsule is performed only when there is a its musculotendinous junction with the TFL and
significant knee flexion contracture of greater than 15°. reflected distally until its insertion onto the tibia
David MacIntosh first described an extra-articular (Gerdy’s tubercle).
ligament reconstruction for the ACL-deficient knee and 2. Fascia lata preparation. The fascia lata should be split
later a combined extra- and intra-articular reconstruc- into two longitudinal strips to make two ligaments. If the
tion with the “over-the-top” technique. This method, fascia is not very wide, bias the posterior limb to be larger.
although no longer used in sports medicine, is a very The posterior half is tubularized using nonabsorbable
suture (#2 Tiger loop™) and a Krackow whipstitch from
(continued) distal to proximal. The medial half is left flat.
Fig. 22.13 Superknee surgical technique including MacIntosh and tibia and pass the ITB through this hole. Secure with interference screw
Paley reverse MacIntosh illustrations. (a) Harvest iliotibial band. (b) with the knee in extension (MacIntosh). (f) Pass the anterior half of the
Divide the ITB into two halves. Do lateral release if maltracking patella. ITB under the patellar tendon and around the adductor magnus and (g)
(c) Pass the posterior half under the LCL. (d) Pass it over the top of the suture it back to itself with the knee in 90° flexion (Paley reverse
intermuscular septum. (e) Drill a hole in the epiphysis of the proximal MacIntosh). (h) Suture the two new ligaments together
3. Lateral release and Grammont patellar tendon anterior skin flap is kept as thick as possible and is
realignment: If the patella maltracks laterally, but it is not reflected medially until the posterior border of the vastus
dislocated or dislocating, a lateral release and patellar ten- medialis can be visualized. The medial intermuscular
don transfer should be performed to move the tendon septum (very rudimentary) and the adductor magnus ten-
medially. The lateral capsule and edge of the vastus late- don are located posterior to the vastus medialis muscle.
ralis should be cut, but not through the synovium. The Care should be taken dissecting in this area to isolate and
vastus lateralis is elevated from the lateral intermuscular not damage the saphenous nerve, as it exits from the
septum and distal femur. If it is still acting as a major quadriceps as the terminal branch of the femoral nerve.
lateral tether of the patella, its tendon should be detached A subperiosteal tunnel is created around the adductor
from the patella and then transferred medially at minimal magnus tendon. The anterior limb of the fascia lata is now
tension. passed under the patella tendon, through a tunnel of
The lateral release is extended distally to the lateral superficial medial retinaculum, looped around the adduc-
aspect of the patellar tendon. This longitudinal deep inci- tor magnus insertion from posterior to anterior, and then
sion should be extended past the tibial tuberosity along the sutured to itself with nonabsorbable suture. This extra-
crest of the tibia incising the proximal tibial periosteum. A articular ligament should always be tensioned with the
parallel periosteal, para-tendinous deep incision is made knee in 90° of flexion. If it is tensioned in extension it
medial to the patellar tendon. Using sharp dissection with may restrict flexion. The excess “ligament” is saved and
a knife blade, the patellar tendon is elevated off the tuber- sutured to the remaining end of the reconstructed ACL as
osity trying not to remove any cartilage if possible. Once it exits the tibia in the next step.
the tendon is detached the periosteal extension of the ten- 5. MacIntosh extra + intra-articular ACL reconstruction.
don is elevated with the tendon so that the detached tendon The lateral collateral ligament (LCL) is identified using
remains tethered distally. This procedure (described by Grant’s test. This is performed by putting the leg in a
Grammont in German [67]) is the pediatric equivalent of figure-four position, which allows the tensioned LCL to
the Elmslie-Trillat [67] procedure in adults. The patellar be easily palpated. Dissect the LCL under tension, identi-
tendon can now be displaced medially and sutured medial fying its anterior and posterior borders. An extra-articular
to the tuberosity with an absorbable suture. tunnel is created under the LCL. The posterior limb of the
4. Extra-articular PCL reconstruction (reverse MacIntosh). fascia lata is passed through the LCL tunnel from anterior
To anchor the ligament to the medial femur, it is neces- to posterior. A subperiosteal dissection is done in the
sary to elevate the skin flap in a medial direction. The over-the-top region, preserving the intermuscular septum
394 D. Paley et al.
attachment. A curved tonsil clamp introduced through the ceps are also separated from the synovium distally and
“over-the-top” tunnel is used to perforate the posterior proximally, respectively.
knee capsule centrally. The synovium is now incised circumferentially around
Next, a drill hole needs to be made in the anterior tibial the patella, separating the patella from the synovium com-
epiphysis to anchor the reconstructed ligament to bone. pletely. The quadriceps tendon is left attached to the patella
Drill a guide wire through the anterior tibial epiphysis proximally, and the patellar tendon remains attached to the
using image intensifier guidance. The wire should start patella distally. The synovium is separated from these struc-
proximal to the proximal tibial physeal line, lateral to the tures. The synovium now has a patella-sized hole in it, which
patellar tendon. The wire should be directed proximally is sutured closed in a longitudinal direction. This leaves the
and posteriorly, exiting into the center of the knee about patella temporarily as an extra-articular bone. The patellar
halfway back on the tibia. As CFD patients lack a true tendon is elevated from the apophysis by sharp dissection
notch and a notch-plasty is not an option, it is better to after circumscribing a medial and lateral incision extending
keep the ligament more posterior on the tibia than the nor- distally into periosteum (Grammont procedure).
mal knee. This avoids damage to the ligament with knee After the tendon is elevated, it is shifted medially at least
extension. a centimeter. In the original Langenskiöld, the patellar ten-
Once the wire is confirmed to be in the correct position don is detached from the tuberosity. A longitudinal incision
in both the AP and lateral views, a hole is drilled with a is made in the synovium, the center of the patella is inserted
cannulated ACL reamer. The diameter of the reamer cho- into this new hole, and the synovium is sutured to the patella
sen is matched to the diameter of the tubularized fascia circumferentially. The medial capsule with the vastus media-
lata ligament, which can be measured with a sizer. A lis is now advanced over the top of the patella and stitched to
suture passer is then passed through the tibial epiphyseal its lateral border. The lateral capsule is left open. If the Paley
tunnel and out the capsular perforation through the “over- reverse MacIntosh procedure is used, the fascia lata should
the-top” tunnel laterally. The suture connected to the fas- not be fixed in place until after the Langenskiöld repair is
cia lata is looped through the suture passer and pulled completed.
through the knee to exit anteriorly through the epiphyseal
tunnel. The tubularized fascia lata is now pulled through
the knee capsule and out the epiphyseal tunnel. Superknee Procedure with Knee Flexion
A transosseous suture is placed into the supracondylar Deformity (Figs. 22.10 and 22.15)
region to help anchor the extra-articular ligament and to
support the over-the-top point. The extra-articular liga- Knee Flexion Contracture Release
ment should be tensioned in full knee extension. Once it If there is a knee flexion deformity >15°, it can be treated by
is secured to the anchor, the rest of the graft is anchored posterior capsular release. This is often done in combination
to the bony tibial epiphyseal tunnel using an absorbable with a Superhip procedure or one of the knee reconstruction
headless interference screw. Again, tension the ligament procedures described above. To avoid direct surgical and
in full knee extension to prevent a flexion contracture of indirect stretch injury to the peroneal nerve, this nerve should
the knee. As the patient’s epiphysis grows, the ligament be identified and decompressed at the neck of the fibula (see
will become more taut. Therefore, mild laxity in the liga- surgical technique below, peroneal nerve decompression).
ment is acceptable. The ligament can now be sutured to Next, the biceps tendon should be Z-lengthened. Extend
the anterior limb graft for additional fixation. the decompression of the common peroneal nerve under the
biceps muscle. With the nerve protected and visualized,
Z-lengthen the biceps muscle and tendon. In congenital
Superknee Procedure with Patellar cases, there is little tendon and mostly muscle. The biceps
Realignment for Dislocated/Dislocating muscle consists of two parts: the short and long heads. To
Patella (Figs. 22.11 and 22.14) avoid damage to this muscle, the short head should be dis-
sected off of the periosteum of the femur. Try and keep as
Langenskiöld Patellar Realignment much of the muscle together and reflect this part proximally.
If the patella is dislocated or dislocating, a modified version Isolate as much of the tendon as possible with sharp dissec-
of the Langenskiöld [65] procedure is performed before the tion off of the muscle and reflect this part distally.
ligament reconstruction. First, the capsule is incised and The lateral head of the gastrocnemius should be released
separated from the patella and synovium both medially and from the femur. It has a very broad insertion of muscle and
laterally. On the medial side, the two layers are separated all tendon. The lateral capsule can now be identified. Dissect the
the way to the medial gutter. The medial capsule is cut trans- contents of the popliteal fossa away from the posterior cap-
versely at its distal end. The patellar tendon and the quadri- sule. To confirm that it is the posterior capsule, incise it
Fig. 22.14 Superknee surgical technique including Grammont, modi- (h) Incise the synovium between the marks. (i) Suture the lateral border
fied Langenskiöld, and Paley reverse MacIntosh procedure illustra- of the synovium to the patella and continue circumferentially
tions. (a) Release capsule on the lateral and medial sides of the patella. (Langenskiöld). (j) Once the entire synovium is sutured to the edge of
Medially cut through the vastus medialis and separate it from the rest of the patella the patella will track correctly. The medial capsule with the
the quadriceps. (b) Do not cut through the synovium. (c) First separate vastus medialis can now be advanced. (k) The medial capsule is
the capsule from the synovium extensively and circumferentially advanced and sutured to the lateral side of the patella. The medial patel-
around the patella and then cut the synovium to release the patella. (d) lar tendon is also sutured to secure the medial advancement of the patel-
The synovium is also released from the underlying muscle and tendon. lar tendon (Grammont). (l) Pass the anterior half of the ITB under the
This leaves a patella-sized hole in the synovium. (e) Suture the hole in patellar tendon and under the medial retinaculum and loop it around the
the synovium longitudinally. (f) Sharply dissect the patellar tendon off adductor magnus tendon (Paley reverse MacIntosh). (m) Secure it back
of the cartilaginous apophysis extending the dissection distally with a to itself with the knee in flexion. If ACL reconstruction is needed the
strip of periosteum (Grammont procedure). (g) Centralize the patella MacIntosh procedure can be done using the posterior half of the ITB
and mark this new position on the synovium with the knee in extension. and secured in extension as in Fig. 22.13e
posterolaterally and enter the knee joint. With the knee visualize the medial capsule. The medial head of the gastroc-
flexed, the only vascular structures that one should see at the nemius is identified as the only structure with a muscle
level of the knee joint are the central geniculate artery and inserting proximally. The medial head should be released
vein. These can be dissected free and cauterized. Care should from the femur, being careful not to injure the femoral ves-
be taken to make sure that the dissection does not inadver- sels that lie immediately lateral to the muscle. The postero-
tently go distal to the level of the knee joint. If the dissection medial capsule is dissected free from the popliteal fossa to
is behind the tibia instead of the knee joint line, the anterior communicate with the lateral dissection. The posterior cap-
tibial vessels may be encountered or injured. sule can then be cut under direct vision from both sides. The
The rest of the popliteal soft tissues can be carefully dis- knee should now be able to fully extend.
sected off of the capsule all the way to the medial side. The The collateral ligaments are left intact. If the medial ham-
capsule can be cut under direct vision. A head lamp may be strings are felt to be tight after the capsulotomy, they can be
useful for this part of the procedure. Once the posterior cap- lengthened through the medial part of the dissection. I prefer
sule is open, try and extend the knee joint. If there is still too to recess the aponeurosis of the semitendinosus and semi-
much resistance, then the medial side will need to be exposed. membranosus tendons rather than doing a Z-lengthening.
If performing a Superknee with ligament repair, a medial After doing the knee capsular release, the knee is examined
skin flap will need to be elevated anyway. If the posterior for instability. If it is unstable, then the ligamentous recon-
capsulotomy is being performed without the rest of the struction of the Superknee procedure is carried out as
Superknee, then the dissecting scissors can be inserted from described above. Occasionally, there is posterior instability
lateral to medial until they can be seen under the medial skin. that requires an intra-articular PCL reconstruction. This can
A separate small skin incision can be made medially to be easily done using the iliotibial band by passing one limb
396 D. Paley et al.
Fig. 22.15 Superknee surgical procedure including posterior capsu- (d) The medial capsule is incised posterior to the medial collateral liga-
lotomy, fascia lata, biceps and gastrocnemius tendon lengthening, and ment. (e) The popliteal contents are dissected free of the posterior cap-
peroneal nerve decompression illustrations. (a) After lengthening the sule from lateral and medial sides to connect together with the knee in
fascia lata (TFL) the biceps tendon is lengthened in a Z fashion. The flexion. The central geniculate vessels are cauterized. (f) The capsule
peroneal nerve is decompressed as detailed in Fig. 22.16. (b) The lateral can now be safely cut from the lateral side. (g) The capsular incision is
head of gastrocnemius is released off of the lateral femur. The lateral completed from the medial side. (h) The biceps tendon is repaired in a
capsule posterior to the LCL is incised. (c) Through a medial incision lengthened fashion. (i) The fascia lata can be used for ligamentous
the medial head of the gastrocnemius is released off of the femur. reconstruction as previously described if needed
of it through an epiphyseal drill hole to the back of the knee. fascial coverings of this compartment. The lateral compart-
The band is then passed from posterior to anterior through a ment fascia folds over itself to form an arcade over the com-
drill hole in the medial femoral epiphysis. It only needs to be mon peroneal nerve. The common peroneal nerve then splits
secured at the femoral end. into superficial and deep branches. The superficial peroneal
nerve continues unimpeded through the lateral compartment,
innervating the peroneal muscles.
Peroneal Nerve Decompression (Fig. 22.16) The second tunnel is the intermuscular septum. The deep
branch passes under the intermuscular septum to enter the
Paley identified two sites of entrapment and described a sur- anterior compartment of the leg, proximal to the intraosseous
gical technique to decompress both tunnels [68]. In a cadaver membrane. Anteriorly, the septum is confluent with the lat-
study, Nogueira demonstrated that nerve tension increases eral and anterior compartment fascia. Posteriorly, it abuts but
after varus osteotomy and decreases to normal levels after does not become part of the intraosseous membrane.
peroneal nerve decompression [76]. Surgery can be performed with or without a tourniquet, but
The first peroneal nerve tunnel is located at the neck of without long-acting neuromuscular blockade. The patient is
the fibula. The common peroneal nerve leaves the undersur- positioned supine, with a bump under the ipsilateral buttock.
face of the biceps muscle to enter the lateral compartment of The common peroneal nerve can be palpated in most peo-
the leg. In order to enter the lateral compartment, the com- ple over the neck of the fibula. To palpate the nerve, roll it with
mon peroneal nerve must perforate the otherwise intact the thumb or finger over the neck of the fibula. Make a 3–5 cm
Fig. 22.16 Peroneal nerve decompression. (a) The peroneal nerve fascia covering the nerve. This fascia can now be cut under direct vision
goes through two very tight fascial tunnels. The first is at the entrance in a retrograde fashion. (d) Extend the transverse fasciotomy to the
to the lateral compartment and involves the entire nerve. The second is anterior compartment and expose the intermuscular septum between
between the anterior and lateral compartments and involves the deep the anterior and lateral compartments. Cut this fascia to complete the
nerve only. (b) Start by incising the superficial fascia and identifying decompression of the nerve. (e) The intermuscular septum comes down
the borders of the common peroneal nerve proximal to the first tunnel. over the deep peroneal nerve like a guillotine. This narrow space under
Incise the lateral compartment fascia in line with the nerve. (c) After this septum is the second tunnel
incising the fascia retract the peroneal muscles to expose the first tunnel
oblique incision parallel to the course of the nerve over the overlying both compartments. Dissect the muscle off both
neck of the fibula. Cut through skin and subcutaneous tissues sides of the septum. Be careful not to cut any superficial
down to the fascia, posterior to the lateral compartment. Feel sensory branches of the nerve that may ascend the septum
for and identify the nerve again. Dissect and expose the nerve and innervate the skin overlying the compartments. With the
proximally and distally. It is not necessary to remove the fat septum exposed on both medial and lateral aspects, cut it
over the nerve. Follow the common peroneal nerve distally from anteromedial to posterolateral. Be careful to stop as
until it disappears into the lateral compartment. Make a trans- soon as the septum ends. Immediately below the septum, a
verse incision over the lateral compartment fascia and expose band of fat can often be seen, which contains the deep pero-
the muscle of the lateral compartment. Retract the muscle of neal nerve. This completes the decompression of the second
the lateral compartment medially to expose the underlying tunnel of the deep peroneal nerve.
arcade of fascia covering the common peroneal nerve. This is Let down the tourniquet at this point. The nerve can be
the first tunnel, which should be released. The nerve can then stimulated with a disposable nerve stimulator. If the tourni-
be seen dividing into two branches, the superficial running quet time exceeds 20 min, the nerve may not initially respond
down in the lateral compartment, and the deep branch going in to electrical stimulation due to the tourniquet ischemia. If the
the direction of the anterior compartment of the leg. nerve was paralyzed before decompression, it can be stimu-
Extend the transverse compartment fasciotomy across to lated before the decompression at the common peroneal
the anterior compartment. Notice the intermuscular septum nerve and then again after the decompression to evaluate if
separating the two compartments, confluent with the fascia there is any immediate change.
398 D. Paley et al.
angle and further increasing the tendency to lateral sublux-

Box 22.10. Superknee Tips and Tricks ation/dislocation of the patella in these patients. Varus defor-
• The stability of the knee cannot be fully assessed mity of the hip or proximal femoral diaphysis is corrected
until the fascia lata has been released. using a proximal valgus osteotomy, whereas valgus defor-
• Take a small portion of biceps fascia and intermus- mity of the knee is corrected using a distal varus osteotomy.
cular septum for a wider graft. If the graft is still If the patient has previously undergone preparatory hip
narrow, bias the more important posterior limb of surgery, such as a Superhip reconstruction or proximal femo-
the graft to be larger. ral osteotomy and Dega pelvic osteotomy, the proximal fem-
• Send the ACL reamer all the way into the knee to oral deformities will already have been corrected and no
make sure that the tunnel is adequately drilled for proximal osteotomy is required at the time of lengthening. If
passing the ACL graft. hip preparatory surgery was not required, but there is exter-
• Tighten the reverse MacIntosh with the knee in nal femoral torsion and proximal varus, then a proximal
flexion, and the ACL graft with the knee in internal rotation and valgus osteotomy of the femur are car-
extension. ried out together with a distal lengthening osteotomy.
• Suture the ends of both limbs of the graft together at A distal femoral lengthening osteotomy can be used to
the end for additional fixation. gradually (ring fixator) or acutely (monolateral fixator) cor-
When cutting the posterior capsule, do not stray rect the valgus and any mild flexion deformity of the knee
distal to the knee joint. (see Figs. 22.10 and 22.11). For valgus correction alone, no
peroneal nerve decompression is required. However, if a
flexion deformity is to be corrected acutely, the peroneal
nerve should be decompressed just prior to the correction at
Femoral Lengthening for Paley Type I CFD the same surgery. As noted above, this distal region of the
femur has a wider cross-sectional area than the proximal
Choice of Osteotomy Level femur and is not in the zone of poor healing sclerotic bone.
Distal femoral osteotomy for lengthening has the advantage Therefore, the regenerate bone in the distal femur is wider,
of a broader cross-sectional diameter for better bone forma- stronger, and subject to more axial deviation muscular forces
tion and less deforming forces from the adductors and ham- than in the proximal femur.
strings. If distal femoral valgus is present, it can be corrected In older children with a wider medullary canal, implant-
using a distal lengthening osteotomy. However, the distal able limb lengthening (Figs. 22.9 and 22.17) or lengthening
osteotomy applies greater forces to the knee joint and over nail (LON) can be performed (see Fig. 22.17) [69]. A
increases the risk of knee stiffness and subluxation. These proximal osteotomy can be used for lengthening over nails
risks can be reduced by articulating across the knee joint and because there is little risk of refracture. The internal rod sup-
extending fixation to the tibia. Proximal femoral osteotomies ports the bone until consolidation is complete. Intramedullary
have less effect on the knee, but have a greater effect on the nailing in children risks disturbing the growth of the trochan-
hip joint. They produce a much higher risk of adduction con- teric apophysis and avascular necrosis of the femoral head. To
tracture, hip subluxation, and dislocation. Extension of the avoid the latter, we use a greater trochanteric starting point
external fixator across the hip is more complex and less and a nail with a proximal bend (e.g., trochanteric entry fem-
desirable than articulated distraction of the knee. Proximal oral, humeral, or tibial nail). To avoid a coxa valga deformity,
lengthening osteotomies are also more prone to poor bone we prefer to use this technique in patients with coxa vara. A
formation and prolonged consolidation phase in patients theoretical epiphysiodesis, created by the nail, may lead to
with CFD. There is also a higher rate of fracture after removal gradual correction of residual coxa vara, although this has not
of external fixation in CFD. For these reasons the author pre- been observed. Fixator-only lengthening is typically used for
fers to lengthen at the distal femur in patients with CFD. the first lengthening. LON or implantable lengthening is used
However, the external rotation deformity of the femur for subsequent lengthenings if the anatomic dimensions of
with CFD should be corrected only by using a proximal oste- the femur permit. In the author’s experience, there is a higher
otomy. The quadriceps muscle is in a normal relationship to risk of deep infection in patients who have had previous
the knee joint, and because most of the quadriceps muscle external fixators. It is unclear if this is related to the number
originates distal to the level of a proximal femoral osteot- or degree of previous pin infections. It is suspected that latent
omy, a proximal femoral internal rotation osteotomy does bacterial forms remain in the bone in a dormant state and are
not change the orientation of the quadriceps relative to the activated at the time of reaming and nail insertion. During
knee joint. A distal osteotomy leaves the bulk of the quadri- external fixator removal, we recommend aggressive curettage
ceps muscle attached proximally in a lateral position and of all granulation tissue in the soft tissues and bone of the pin
rotates the knee medially, thus increasing the effective Q tract in an effort to reduce such latent infection.
Soft Tissue Releases for Limb Lengthening The rectus femoris is evaluated with the Ely test (see
In patients with CFD, soft tissue releases in conjunction with Fig. 22.3a). A positive Ely test demonstrates pelvic flexion at
femoral lengthening are essential to prevent subluxation and the hip during prone knee bend. The rectus femoris should be
stiffness of the knee and hip. If there is no contracture or released through a small anterior inguinal incision. Even
tightness at the time of the index procedure, the soft tissue with a negative Ely test, the rectus may be released from
releases can either be performed at the index procedure or anterior inferior spine, as it still tends to tighten with
delayed until these soft tissues become contracted, approxi- lengthening.
mately 6 weeks later. Usually, these are done at the index Hamstrings are evaluated by the straight leg raise, mea-
procedure to avoid an additional anesthetic. If a preparatory suring the popliteal angle (see Fig. 22.3b). If the knee can
hip or knee surgery has already been performed, then the fully extend with the hip flexed to 90°, then the popliteal
fascia lata, rectus femoris, and biceps femoris may have angle is 0° and the hamstrings are not tight and thus such a
already been lengthened, and there is no need to repeat them. patient would require no treatment before femoral lengthen-
However, if soft tissue releases have not been performed, ing. If there is a popliteal angle >0°, the hamstrings are
then they should be carried out at the time of the lengthening already tight and will lead to contractures during lengthen-
surgery. ing. The author prefers lengthening the biceps femoris with
Before surgery, the range of motion of the hip and knee the iliotibial band through a lateral approach. If needed, the
should be evaluated using muscle lengthening tests and the medial hamstrings can be lengthened from the medial side.
presence of contractures or limitation identified. The muscle If hip abduction is limited, percutaneous adductor tenoto-
lengthening tests are the prone knee flexion test (Ely test) for mies of the adductor longus and gracilis tendons should be
the rectus femoris (see Fig. 22.3a), the straight leg raising performed. If hip subluxation or more severe adduction con-
test (popliteal angle) for the hamstrings (see Fig. 22.3b), hip tractures develop during lengthening, then an open, more
abduction range of motion for adductor contractures, and hip extensive adductor release, including the adductor brevis, is
adduction range of motion with the knee and hip in extension indicated. The adductor release is very important in proximal
for the fascia lata. The Ober test is helpful for identifying lengthening, but for distal lengthening, it is usually not nec-
fascia lata tightness (see Fig. 22.3c). essary. Distal adductor magnus release has been described
Fig. 22.17 Two-year-old girl born with Paley type 1b subtrochanteric monolateral external fixator (Orthofix LRS and Sheffield components,
type. AP and lateral radiographs (a, b) showing characteristic deformity McKinney, TX). The knee is able to flex and extend with a lateral knee
as previous illustrated in Figs. 22.1 and 22.7. After the Superhip proce- hinge connected to an arch fixed to the tibia (j). The femur was length-
dure performed at age 2 years, the femur appears to have a normal ened 7 cm (k) over a Rush rod similar to Bost’s lengthening over nail
anatomy as shown on AP and lateral views (c, d). Standing X-ray prior method [69]. Third and final lengthening of 5 cm at age 15 years (l) was
to first lengthening. A 1-cm gain was achieved due to the Dega osteot- performed using an implantable lengthening nail (PRECICE, Ellipse
omy (e). First lengthening performed at age 4 years, using Ilizarov Technologies, Irvine, CA). Note that prior to this a contralateral distal
apparatus (Smith & Nephew, Memphis, TN) with knee hinges and fixa- femur epiphysiodesis (5-cm equalization) was performed at age 10 and
tion to the tibia (f). After 8-cm lengthening (g). Full recovery of hip and pelvic osteotomy and distal femoral varus osteotomy were performed at
knee range of motion and strength following first lengthening (h, i). age 14. Total limb length equalization was 26 cm. The hardware was
Second lengthening performed at age 8 years, using a multiplanar removed 1 year after the lengthening (m)
400 D. Paley et al.
for congenital femoral lengthening [70], but this has not been lengthening, if it has not already been excised, the fascia
found to be helpful in the senior author’s opinion. lata should be lengthened. A 3-cm longitudinal incision is
To confirm that the fascia lata is tight, perform an Ober made over the posterior edge of the fascia lata, where it con-
test (see Fig. 22.3c). The patient lies on his or her side, and nects with the intermuscular septum. The entire fascia lata is
the knee is flexed and hip extended by pulling back on the transected at the level of the proximal pole of the patella by
foot and pushing forward on the buttock. The knee will stay dissecting anterior to the incision. If the incision is made
up in the air and not fall down due to contracture of the fas- more posteriorly, the biceps femoris can be easily exposed
cia lata. This is called a positive Ober sign and indicates that on the posterior side and the lateral biceps fascia can be
the fascia should be excised or cut. In every case of CFD safely recessed.
Fig. 22.18 Intra-articular osteotomy. (a) CFD Paley type 2 with rota- plateau. A hemi-plateau osteotomy of the posterior plateau was per-
tory subluxation of the knee joint. The posterolateral tibial plateau is formed, with allograft and internal fixation. (c) After complete bone
round (arrow) and contributes to the subluxation. (b) The Superknee healing the lateral plateau is now flat and not round. The subluxation
procedure was performed with decompression of the peroneal nerve was eliminated by the Superknee procedure
and taking down the proximal tibiofibular joint to expose the lateral
Patients may develop hyperlordosis or a hip flexion or being transmitted to the knee joint cartilage. Hinges are an
abduction contracture secondary to the femoral lengthening. integral part of circular external fixators such as the Ilizarov
If so, the proximal fascia lata is cut transversely through a device, as well as some monolateral external fixators such as
small lateral incision just distal to the greater trochanter. the Orthofix Limb Reconstruction System (LRS), Smith and
Nephew Modular Rail System.
Botulinum Toxin Injection Less common is anterior subluxation or dislocation of the
The effect of botulinum toxin has been shown to be helpful tibia on the femur. This occurs as the knee goes into exten-
in prevention of muscle contractures [71, 72]. In the author’s sion. It is important to document the angle of flexion the
experience, it decreases muscle stretch pain due to muscle knee relocates or, conversely, at which angle short of full
spasm, which is primarily in the quadriceps muscle during extension the knee dislocates. These dislocations are more
physical therapy. Botulinum toxin can be injected into the commonly due to a posterior deficiency with rounding of the
quadriceps up to a limit of 10 U/kg body weight. The added tibial plateau, but they can also be due to an anterior defi-
volume of the saline is limited to avoid systemic toxicity, so ciency of the distal femur, in which the lateral radiograph of
injection is usually done with a total of 3–4 mL. the knee shows a lack of the anterior protuberance of the
femoral condyles. If the posterior condyle is rounded but the
Knee Instability anterior part of the knee is straight, an intra-articular osteot-
Almost all cases of CFD can be assumed to have hypoplastic omy is required to elevate the posterior condyle: intra-
or absent cruciate ligaments, with mild to moderate antero- epiphyseal in children and metaphyseal in adults (Fig. 22.18).
posterior instability. Some patients also have medial-lateral This can also be combined with a PCL reconstruction as
and torsional instability. Despite this, the knee tracks nor- noted above.
mally preoperatively, and there is no indication to perform
ligamentous reconstruction in most cases. The significance
of knee instability is the tendency of the knee to subluxate Surgical Technique
with lengthening. The subluxation is usually posterior, or
posterolateral (posterior plus external rotation of the tibia on There are currently a multitude of different devices used for
the femur), but it can also be anterior. Knee extension usually external fixation and distraction osteogenesis. The original
reduces posterior subluxation before lengthening. Therefore, Ilizarov fixator technique requires the use of rings or arches.
to prevent posterior subluxation, some surgeons recommend It allows multiplanar fixation in small segments of bone and
splinting the knee in extension throughout the distraction can incorporate hinges to correct angular deformity gradu-
phase [42]. This promotes knee stiffness while protecting the ally or acutely. It allows for hinged articulation across the hip
knee from subluxation. The author prefers to protect the and knee joints, thus protecting these joints from subluxation
knee by extending the external fixation to the tibia with a during distraction without restricting joint motion. However,
hinge. The hinge permits knee motion while preventing pos- it is bulkier than a monolateral external fixator and forces the
terior as well as anterior subluxation. It also prevents pressure legs to be abducted due to the ring protruding medially.
402 D. Paley et al.
Monolateral external fixators traditionally lacked the abil- perpendicular to the floor with the leg crossed over and
ity to span across joints. They have evolved to permit articu- rotated as described above for correction of deformity.
lated fixation to the tibia and pelvis. They also allow locking Two Ilizarov rings, properly sized for the distal femur, are
of the hinge to prevent contracture. Deformities can also be applied to a distal femoral reference wire, which is parallel to
corrected acutely. Monolateral fixators are less bulky and the knee joint. For young children, we obtain an arthrogram
easier to wear for the patients. Nevertheless, they are more to better outline the cartilaginous femoral condylar line and
constrained in their pin location options and are therefore visualize the posterior femoral condyles for hinge place-
less space efficient than the circular fixators. The Orthofix ment. Conical washers or hinges are used between the two
LRS (Orthofix, Lewisville, TX) and the Smith & Nephew distal rings because of the valgus of the distal femur. The
Modular Rail System (MRS, Smith & Nephew, Memphis, rings are at a valgus deformity angle to each other. A lateral
TN) are two examples of currently available monolateral half-pin is inserted into the mid-segment of the femur. This
external fixators that allow articulated spanning of joints. pin is at 7° to the shaft of the bone.
Their application for CFD lengthening together with the At this point, the proximal subtrochanteric osteotomy can
application of the Ilizarov device will be described. be performed. This is done percutaneously by making mul-
In the femur, half-pin fixation is the rule, even with the tiple drill holes and then using an osteotome. The osteotomy
circular fixator. Half-pin size is determined by the diameter is internally rotated, laterally translated, and then angulated
of the bone, with 6.0-mm pins used for most older children into valgus and extended to correct all components of the
and 4.5-mm pins for younger children with smaller bones. proximal femoral deformity. The order of correction is
For very young children, small tapered 3.5- to 4.5-mm pins important to achieve the necessary displacement without
can be used. The author prefers to use hydroxyapatite-coated loss of bone-to-bone contact and stability.
pins to decrease the chance of loosening and to lower the risk Two additional half-pins are inserted and fixed onto the
of pin-site infection [73]. distal ring, one from posteromedial and the other from pos-
terolateral, between the quadriceps and the hamstring mus-
cles. One more middle pin is inserted. In small children, all
Distal Femoral Lengthening: Ilizarov™ Fixator half-pins are inserted by using the cannulated drill technique.
Technique (See Fig. 22.17) This involves insertion of a wire first, then a cannulated drill,
and then a half-pin. This technique permits very accurate
All the acute soft tissue releases are performed first. If soft placement of large-diameter pins in narrow bones to avoid
tissue releases are to be performed on a delayed basis, pro- eccentric placement. Eccentric placement of drill holes and
ceed directly with the frame application. If a second-level, half-pins in the femoral diaphysis, especially anteriorly, can
proximal femoral derotation, valgus, and/or extension oste- lead to fracture. The distal femoral osteotomy is performed
otomy is needed, the proximal pin is inserted into the proxi- percutaneously, with multiple drill holes and an osteotome.
mal femur with the hip in the position in which it will lie The only wire used is removed to avoid tethering of the
after the correction. For example, for an internal rotation quadriceps and fascia lata.
osteotomy, the proximal pin should be inserted with the knee
in external rotation. For a valgus osteotomy, the proximal pin Knee Hinges
should be inserted with the hip adducted. For an extension The last step is to extend the fixation to the tibia using hinges.
osteotomy, the proximal pins should be inserted with the hip The center of rotation of the knee is located at the intersection
flexed. For correction of varus, flexion, and external rotation, of the posterior femoral cortical line and the distal femoral
the femur should be externally rotated and crossed over the physeal line in the plane where the two posterior femoral con-
other thigh to adduct and flex the hip. This places the hip in dyles are seen to overlap on the lateral view (Fig. 22.19) [42].
the true neutral position. For younger children, it is helpful to inject arthrographic dye
The proximal arch is attached parallel to a line from the into the knee to visualize the posterior femoral condyles. It is
tip of the greater trochanter to the center of the femoral head, important that the distal femoral ring, which is parallel to
the middle ring is perpendicular to the mechanical axis of the the distal femoral condyles, appears to be perpendicular to the
shaft of the femur (7° to the shaft), and the distal ring is par- X-ray beam. The medial and lateral skin is marked at the
allel to the knee joint line. After the osteotomies, when the location of the planned hinge placement. A single half-ring is
rings and arch are parallel, the mechanical axis of each seg- attached to two threaded rods from the hinges. This half-ring
ment will be aligned and the joint orientation of the hip and is oriented perpendicular to the tibia with the knee in full
knee will be parallel. The first half-pin is from lateral to extension. The first half-pin is inserted from anterior to poste-
medial in the frontal plane, parallel to a line from the tip of rior into the tibia. After fixing this pin to the proximal tibial
the greater trochanter to the center of the femoral head. A half-ring, the knee is flexed and extended through a range of
second proximal half-pin is inserted on the proximal arch motion. A drop test is performed to see if the tibia flexes
from 30° anterolateral to the first pin. The proximal arch is through the hinge without catching. If this range feels
Distal Femoral Lengthening: Orthofix™ Fixator

Technique (See Fig. 22.17)
1. Start by identifying the center of rotation axis of the knee

joint (see description of this above). A 1.8-mm wire is
drilled into the lateral edge of the physis at the intersec-
tion of the posterior cortex of the femur with the physis in
line with the plane of overlap of the posterior femoral
condyles. The Orthofix LRS comes in pediatric and adult
sizes. The hinge axis is lined up through its most distal
hole.
2. A commercially available “sandwich” clamp is used, or
an extra lid is used in the pin clamp to create a second
layer of pin holes more anteriorly. The fixator bar is lined
up with the shaft of the femur and the most proximal half-
pin inserted.
3. The most distal pin is then drilled one hole proximal to
the center of rotation pin. The LRS without sandwich
clamps is then used to place the rest of the pins, three
proximal and three distal. If the distal femoral valgus is to
be corrected acutely, a swivel clamp is used for distal pin
placement. When using a pediatric LRS, the three-hole
Fig. 22.19 Lateral fluoroscopic view of knee arthrogram. The center pin clamp contains two half-pins only, since one hole is
of rotation knee axis pin has been inserted at the level of distal physis used up for the knee center of rotation. A third pin is
and the intersection with the posterior cortex. The medial and lateral
femoral posterior femoral condyle outlines can be seen because of the added using Ilizarov cubes connected via these two pins.
radiographic dye. They both overlap each other 4. Before reapplying the sandwich clamps, the osteotomy is
performed and the distal valgus corrected acutely. After
frictionless, a second and third tibial half-pin are added. the correction, the fixator can be exchanged for one with
Finally, a removable knee extension bar is inserted anteriorly straight clamps and with the sandwich attachments. All
between the distal femoral and the tibial half-ring. the pins should be in the upper deck of the double-decker
sandwich clamps. The only pin in the lower deck is the
knee axis pin. This pin does not enter the patient’s leg. It
Box 22.11. The Drop Test is a 6-mm segment of pin that protrudes laterally. A
• A half-pin is placed in the proximal tibia from ante- Sheffield clamp from Orthofix™ is applied to this pin to
rior to posterior. act as a surrogate knee hinge. It is locked in place by put-
• The half-pin is attached to the proximal tibia ring ting a Rancho™ cube laterally with a set screw to prevent
and secured. it from moving outward. Conical washers are used
• The tibia is raised up in full extension with the hip between the Sheffield clamp and the LRS to prevent fric-
flexed. tion. The Sheffield clamp is left partially loose to permit
• While supporting the femur, the tibia is released to motion.
flex with gravity. 5. A 1/3 Sheffield arch is attached to the clamp arching
• A positive drop test and a well-placed knee hinge towards the tibia. An anteroposterior pin is inserted and
allow the tibia to flex to 90° without catching. the drop test (see above) is performed. If there is friction,
• If there is significant friction, make adjustments to the Sheffield clamp should be loosened. If friction per-
the half-pin. sists adjust the connection of the pin to the Sheffield arch.
• Securing the half-pin with the knee in full flexion or If friction persists the axis pin may need to be bent slightly
full extension or 45° may improve the drop test. to alter the axis of rotation. Once the drop test is negative
• Do not add any more pins until the drop test demon- for friction, two more oblique pins are inserted into the
strates no friction. tibia and connected to the Sheffield arch using cubes.
• The success of the drop test depends on the initial 6. A removable knee extension bar is fashioned from
accurate placement of the hinge. Ilizarov parts to be used especially at nighttime. If there
Perform the drop test after each pin is added. is an unstable hip, an axis pin for the hip can also be
Use a total of three pins in the tibia. fashioned and attached from the proximal clamp. The
same Sheffield clamp and arch arrangement are used.
404 D. Paley et al.
Fig. 22.20 Modular Rail System (MRS, Smith & Nephew, Memphis, lated spanning across to the tibia. The arthrographic dye is in place. The
TN) technique. (a) The external fixation pin is inserted parallel to the fixator hinge line corresponds to the knee hinge center of rotation axis.
knee axis pin. The external fixator is used as a template. (b) AP and (c) Illustration of the Modular Rail System external fixator articulated
lateral radiographs showing the external fixator in place and the articu- across the knee joint. Note location of knee hinge
Two pins are placed in the pelvis from the anterior infe- to the Steinmann pin. This wire should be as proximal as
rior and superior spines extending posteriorly. These are possible in the femur but distal to the trochanteric
fixed to the Sheffield clamp to prevent proximal sublux- apophysis. Confirm the location of this wire making
ation of the hip during lengthening. Thus, the same prin- sure that it is located in the mid-diaphysis or slightly
ciples are applied when using monolateral as with posterior to the midline. Drill a hole using a cannulated
circular fixation, i.e., hinge fixation across joints when drill appropriate to the pin size preferred (e.g., 4.8 mm
there is a joint at risk. for a 6-mm pin, 3.8 mm for a 4.5-mm pin, and 3.2 mm
for a 3.5- to 4.5-mm pin).
5. Insert a pin of the appropriate thread length correspond-
Distal Femoral Lengthening: Modular Rail ing to the diameter of the bone at that level. The bending
System (Smith & Nephew, Memphis) Technique strength of the pin is related to the smallest diameter
(Figs. 22.9, 22.10, and 22.20) protruding from the near cortex. It is better not to leave
threads outside of the near cortex to obtain the maxi-
This external fixator was designed specifically for the CFD mum bending strength out of each pin.
patient. It is able to articulate and span across the hip and 6. Mount the pre-constructed MRS onto the Steinmann pin
knee joint either separately or at the same time. It also has and the proximal half-pin. The Steinmann pin goes
clamps that allow fixation in the oblique plane in addition to through the cannulated hinge of the fixator.
the frontal plane, producing delta fixation. 7. Drill a 1.8-mm wire through the distal most pin hole of
1. Pre-construct the external fixator using pediatric or adult the clamp. An overhang clamp can be used to minimize
rail segments with two pin clamps, knee hinge, and tib- the distance from the most distal pin to the hinge. This
ial rail segment. pin should be just proximal to the physis. Overdrill the
2. Inject radio-contrast solution into the knee joint and wire with a cannulated drill and insert the pin.
obtain a true lateral projection, rotating the femur until 8. Insert the other two pins into the clamp, making sure
the posterior aspect of the medial and lateral femoral that the holes line up with the bone. All the pins should
condyles overlap. be parallel to each other. Make sure that the distal pin
3. Insert a 2-mm Steinmann pin into the center of rotation clamp is locked down to the fixator with 7-mm red bolts.
of the knee joint. This is defined as the point of intersec- Slide off the fixator from the pins.
tion of the posterior femoral cortex with the distal femo- 9. Make a 7-mm incision just proximal to the most proxi-
ral physis. In adults, it is the intersection of the posterior mal distal pin. Drill the femur at this level with multiple
cortex of the femur with Blumensaat’s line. Confirm on drill holes. Complete the osteotomy using a sharp
the AP view that the pin is parallel to the joint line. osteotome.
4. If there is no valgus or flexion deformity, proceed to 10. Reapply the fixator to the pins. Make sure that the
drilling a 1.8-mm wire into the proximal femur parallel osteotomy does not displace or translate, making
adjustments as needed. If AP clamps are being used, Modification for Valgus ± Flexion Deformity
drill at least one hole for an oblique posterolateral proxi- of the Distal Femur
mally and distally. There should be three or four total
number of pins per segment, depending on the size of If there is a valgus or flexion deformity of the distal femur,
the femur and the thigh being lengthened. changing the pin placement will provide correction. The first
11. Drill and place an anterior to posterior pin in the proxi- wire is placed into the distal femur, using the MRS as a guide
mal tibia, making sure that it is distal to the tip of the to keep the wire parallel to the knee hinge pin. A cannulated
proximal tibial apophysis. Connect the tibial pin to an drill is then used and a half-pin is inserted. The most proxi-
arch mounted off of the tibial rail extension using a mal pin is then placed into the proximal femur at about 7° of
cube. Test the motion of the knee to confirm that the valgus in relation to the shaft. In reference to the distal pin, it
knee moves freely. By placing the first tibial pin in the should be in valgus angulation and in the same rotational
sagittal plane, anteroposterior adjustment can be done if orientation as the distal pin. A distal femoral osteotomy is
there is subluxation. Apply a set screw to the pin once performed at the distal femur at the planned level, leaving
the knee motion is free. Confirm with a drop test: if the enough room to add two more pins distal to the osteotomy.
leg drops with no friction from 0° to 90°, then the hinge Once the pins are connected to the external frame and paral-
is perfect. Apply two more pins with a different orienta- lel to each other, the valgus deformity is corrected. If there is
tion in the proximal tibia. also a flexion deformity of the knee, extend the knee with the
12. Apply a distractor bar between the two clamps, making tibia to level the distal segment into extension before adding
sure that it turns freely and that the osteotomy is distract- the remaining frontal plane pins.
ing appropriately. The bar distracts 1 mm for every full
turn of 360°.
13. Apply a knee extension bar constructed from Ilizarov Rehabilitation and Follow-Up During
parts. This includes a post proximally and a twisted Lengthening
hinge distally. Off of each of these, insert a post and fix
with a 20- or 30-mm socket. Connect a threaded rod Femoral lengthening requires close follow-up and intensive
between the two posts. The knee extension bar should be rehabilitation to identify problems and maintain a functional
placed in maximum extension of the knee and locked extremity. Clinically, the patient is assessed for hip and knee
down so that the rod length cannot be changed. The range of motion, nerve function, and pin-site problems.
patient uses the bar full time at night and half time dur- Radiographically, the distraction gap length, regenerate bone
ing the day. It is easily removable for exercise. quality, limb alignment, and joint location are assessed. If the
14. Incisions are closed and sterile dressings applied around lengthening rate is 1 mm per day, then follow-up is usually
the pins. Consider Botox to the quadriceps muscles to every 2 weeks. If there is delayed healing of the regenerate
diminish muscle spasm. Final radiographs are taken bone, or if knee motion is decreasing, then the rate is often
prior to leaving the operating room. decreased. If the lengthening rate is decreased to 0.75 mm or
0.5 mm per day then the follow-up can be increased to 3 or 4
weeks, respectively. Rarely, if there is concern for premature
consolidation and the lengthening rate is increased, then fol-
Box 22.12. Lengthening Tips and Tricks low-up is more frequent.
• A knee arthrogram is helpful in young children to Patients are allowed to weight-bear as tolerated with their
visualize the posterior condyles or center of external fixator. Physical therapy is begun within 1 or 2 days
rotation. after surgery and continues daily throughout the distraction
• The first half-pin or the hinge pin sets up the rest of and consolidation phase. In the author’s opinion, the amount
the pins. Spend extra time getting the first one per- of therapy is directly related to a better functional result and
fectly right, and the rest will follow. faster rehabilitation after removal. During distraction, patients
• For smaller bones, use the cannulated wire tech- have one to two formal physical therapy sessions daily (45–
nique to ensure proper placement of pins. Eccentric 60 min each). In addition, at least two home sessions (30 min
cortical drilling can lead to fractures or pin pullout. each) are recommended to the parent. Patients undergoing
• Use off-axis pins for stronger fixation if possible. lengthening are at their best immediately after surgery and
Make sure that the osteotomy is completed and have difficulty with muscle tightness and range of motion only
distracts appropriately without too much resistance after lengthening has started. It is not until the consolidation
before leaving the operating room. phase that the usual orthopedic pattern of rehabilitation and
recovery occurs. Thus, lengthening should be thought of as a
406 D. Paley et al.
prolonged procedure that lasts until the end of the distraction painless bending of the femur or as a completely displaced
period, and not even be considered in the absence of therapy. fracture. The use of a spica cast does not reduce the fracture
The majority of therapy time is spent obtaining knee flex- rate. A study, performed by the author in 1999, showed a
ion and maintaining knee extension. Knee flexion should be fracture rate of 34 % after CFD lengthening, compared to
maintained as close to 90° as possible. It is important to start 9 % for other etiologies [84]. The regenerate bone should
pushing the extremes of passive knee flexion (especially show no gaps, the interzone should be closed, and based on
prone) during the 1-week latency period after surgery. Once AP and lateral views there should be evidence of cortical-
distraction begins, there is more resistance from muscle ization on at least three sides of the lengthened regenerate.
spasm. If knee flexion begins to diminish (<75°), then length- Since this refracture rate was so high and unacceptable,
ening should be stopped or slowed to rehabilitate the knee Paley started prophylactic rodding of the femur with a Rush
better, sometimes waiting for improvement before resuming rod in 2000. The fracture rate dropped to almost zero in the
lengthening. Function should never be sacrificed for length, senior author’s personal experience and somewhat higher in
and preserving the knee joint and motion is paramount. A a multi-surgeon series [53]. In the senior author’s personal
knee extension bar is used at night and part-time during the series, the rate of infection after such prophylactic rodding
day to prevent a knee flexion contracture [42]. Posterior sub- was 4 %. These were easy to eliminate by removal of the rod
luxation can occur with a fixed flexion deformity and is sus- and insertion of an antibiotic cement rod [74]. This was con-
pected clinically based on a change in the shape of the front sidered a reasonable trade-off: trading 34 % fracture rate for
of the knee. The patella becomes very prominent and the 4 % infection rate.
tibia appears depressed (ski hill sign).
Weight bearing is encouraged throughout the lengthening
as long as the patient has normal proprioception. In younger Surgical Technique for Prophylactic
children, walking without support can be achieved immedi- Rodding of Femur at Time of External
ately because the fixator is very stiff relative to the patient’s Fixator Removal
weight. When the child is older and heavier, crutches or a
walker is preferred until there is sufficient consolidation. 1. The external fixator is removed under general anesthesia,
During the distraction phase, therapy is focused on passive ensuring that the patient is deeply asleep (to avoid invol-
range of motion exercises to maintain hip and knee range of untary muscle spasms which could lead to fracture of the
motion. During the consolidation phase, the priority shifts to regenerate at the time of removal). Slightly loosen all of
active range of motion and strengthening to regain any motion the pins while they are still supported by the frame, which
lost during distraction. Hip abduction and extension exercises is especially important in hydroxyapatite-coated pins.
help prevent adduction and flexion contractures that can lead Remove the middle pins while the fixator is still on. The
to hip subluxation. Release of the adductors, rectus femoris, most distal and proximal pins are removed while an assis-
and TFL during lengthening may need to be considered to tant provides bimanual support of the thigh above and
allow further lengthening and to prevent hip subluxation. below the lengthening zone.
After the lengthening regenerate is healed and the exter- 2. After prepping the thigh, curettage any loose granulation
nal fixator removed, recovery is limited by two factors: joint tissue from the pin holes. The pin holes in the bone are
range of motion and muscle weakness. In most cases, joint also curetted in the event of osteolysis or infection. Cover
motion is regained faster than muscle strength. The three and seal the pin holes with an adherent plastic dressing
antigravity muscles are the limiting factor to full recovery: (e.g., Tegaderm, 3 M, Minneapolis, MN). In the event of
the hip abductors, quadriceps, and gastro-soleus muscles. To a severe infection, the surgeon must weigh risks of a frac-
minimize disability, these muscles should have resistance ture and loss of length versus hardware infection and sec-
training throughout the lengthening program to prevent atro- ondary surgeries.
phy from prolonged external fixation and lengthening. Other 3. Prep and drape the lower limb free, being careful not to
modalities such as electrical stimulation or aquatic therapy manipulate the leg. A bump may be placed under the
may be helpful. Please also refer to Chap. 12 for more details ischium to raise the femur for lateral viewing on image-
regarding physical therapy in such patients. intensifier radiography.
4. Insert a 1.8-mm bayonet-tipped Ilizarov wire at the tip of
the greater trochanter. Advance the wire by tapping, rotat-
Fixator Removal and Rodding of Femur ing the curved tip of the wire to bounce off the inner
(See Figs. 22.9 and 22.10) cortex. Use the drill only if the wire will not advance.
A slight bend in the wire may be necessary to direct
The external fixator can be removed once the regenerate the wire. The wire is passed through the center of the
bone is fully healed radiographically. The biggest risk after intramedullary canal and regenerate bone, all the way to
removal is fracture of the femur, which can manifest as the distal femoral physis if possible. A second wire of
equal length can help measure the length by inserting it to Specific Complications and Their Treatment
the greater trochanter and measuring the difference in for Congenital Femoral Deficiency
length of the two wires. Lengthening
5. Drill over the wire with a cannulated drill bit. The 3.2-
mm, 4.8-mm, and 6.4-mm drill bits correspond to the Nerve Injury
1/8″, 3/16″, and 1/4″ Rush rods, respectively. Long can- Nerve injury from surgery or distraction is unusual with fem-
nulated drills are available from Pega Medical (Montreal, oral lengthening. To avoid peroneal nerve injury from the
Canada) and are preferred since they slightly overdrill pins, the posterolateral pin should not enter posterior to the
above the designated size making rod insertion easier. biceps tendon. During distraction, if the patient complains of
They are also longer and can reach the full length of pain in the dorsum of the foot or asks for frequent massage
almost any femur. of the foot, this is most likely referred pain from stretch
6. Insert an appropriate length Rush rod. As an alternative, a entrapment of the peroneal nerve. More advanced symptoms
stainless steel flexible nail can be used instead, but they include dysesthesia in the distribution of the peroneal nerve
are more difficult to insert and direct. The end of the Rush or extensor hallucis longus weakness. A nerve conduction
rod is better designed for directing the implant tip. study may show evidence of nerve injury, but most cases will
Recently, a lockable rod (the SLIM, Pega Medical, be negative since too many fibers are conducting normally.
Montreal, Canada) has been developed for this purpose Quantitative sensory testing using the pressure-sensitive sen-
with a tip similar to the Rush rod but with a threaded sory device (PSSD), if available, is the most sensitive test to
proximal part similar to the Fassier-Duval nail (Pega assess for nerve involvement [75]. Near-nerve conduction
Medical, Montreal, Canada). using very fine electrodes is also very accurate.
7. After rodding, the patient goes home with sterile, dry If a nerve problem is identified early, it can be treated by
dressings covering the pin sites and a 10-day course of slowing the rate of distraction. However, if symptoms persist
oral antibiotics, usually a first-generation cephalosporin. or motor signs develop, then peroneal nerve decompression
Weight bearing and physical therapy are restricted for should be performed [76, 77]. The timing of nerve decom-
1 month. Gentle range of motion of the knee is allowed at pression affects the rate of recovery of neuropraxia, and an
home. early decompression leads to faster recovery. The peroneal
nerve should be decompressed at the neck of the fibula,
including transverse fasciotomy of the anterior and lateral
compartment and release of the intermuscular septum
Box 22.13. Fixator Removal Tips and Tricks between these compartments [68] (see Fig. 22.16). When
• Do not remove fixator until three sides have peroneal nerve decompression was performed on patients
corticalized. undergoing limb lengthening, intraoperative findings
• Use a rush rod or intramedullary device to prevent included hemorrhage, nerve flattening, narrowing of the
fracture through the regenerate bone. nerve at the entrance of the fascial tunnel, and reduction of
• Slightly turn or “crack” all the half-pins while in the the perineural vascularization at the site of compression.
fixator prior to removing the frame. The hydroxy- These findings are typical of nerve entrapment and not of
apatite bond can be quite strong. stretch injury. In addition, no relationship was found between
• Curettage out any hypertrophic granulation tissue. nerve injury and the amount or percent of lengthening.
Loose pins warrant more thorough curettage of the
bone. Poor or Failed Bone Formation
• It may not be possible to direct a thin Ilizarov wire Hypotrophic regenerate formation requires slowing of the
all the way down the femur. Drill just the proximal distraction rate. The rate can be slowed to ¾, ½, or ¼ mm per
portion and then proceed to the Rush rod. day. If the regenerate bone does not improve, a decision
• If needed, put a small bend in the Rush rod in line needs to be made to continue lengthening. If the defect in the
with the bevel to augment guided insertion. distraction gap does not fill, it will need bone grafting.
Hold off on physical therapy for a month after Bisphosphonate infusion (e.g., zoledronic acid) can be used
fixator removal. to prevent bone resorption while permitting bone formation
[78, 79].
408 D. Paley et al.
Complete failure of bone formation is very unusual. Hip Subluxation/Dislocation

Partial defects, especially laterally, are common. There is often mild or moderate acetabular dysplasia in
Dynamization of the fixator and bone growth stimulators patients with CFD. The pattern is different than DDH; the
(e.g., Exogen™) can be used. Resection of the fibrous tissue femoral head is usually uncovered laterally rather than
in these defects and cancellous bone grafting may become anterolaterally. If the CE angle is less than 20°, the hip joint
necessary to reduce the external fixation time and prevent is considered at risk for dislocation [59]. The orientation of
fracture after frame removal. the sourcil should also be horizontal. If it is inclined superi-
There are two methods of bone grafting the regenerate: orly, then the hip joint is potentially unstable even with a
1. Autogenous cancellous bone grafting. The distraction gap normal CE angle. It is always safer to err on the side of per-
should be opened and the fibrous tissue excised, leaving forming a pelvic osteotomy prior to femoral lengthening
behind any regenerate bone. Once all of the fibrous tissue than to end up with a hip subluxation.
has been removed, cancellous autogenous bone graft can Hip joint subluxation or dislocation during lengthening is
be harvested and inserted. In most children, this is a dreaded complication. It is diagnosed radiographically.
obtained from the iliac crest. In young children there may The earliest sign is a break in Shenton’s line or increased
not be enough bone from the standard anterior iliac crest medial head-teardrop distance indicates subluxation of the
approach. More bone graft can be obtained by splitting hip. The hip usually has an adduction and flexion contracture
the tables of the iliac crest and taking graft from above the and may also exhibit stiffness with flexion and extension.
acetabulum. This allows harvesting of a large cache of Hip subluxation does not usually occur if there is adequate
bone that is normally not available for grafting, providing coverage of the femoral head, but they can occur if there are
enough bone to fill the distraction gap. adduction and flexion contractures.
2. Cortical allograft. This technique is used when the dis- If hip subluxation occurs, distraction must stop. The
traction gap is very long and exceeds the available autog- patient should be taken to the operating room to reduce the
enous cancellous bone graft. The technique is credited to hip. Release the adductor longus and gracilis, and the TFL
Wasserstein [80], originally from Latvia and later from and rectus femoris if needed. Abduction should reduce the
Germany. He described inserting a slotted allograft into a hip subluxation. If not, the distraction gap should be short-
distraction gap to create early structure and bridging of ened to loosen the hip joint. The external fixator should be
the gap. A non-slotted allograft can be used instead, sta- extended to the pelvis with or without a flexion-extension
bilized with an intramedullary nail. BMP-2 can be added hinge. Pelvic fixation consists of at least two anterolateral
for both cancellous and cortical grafts. pins between the two tables above the anterior inferior iliac
spine followed by two off-axis lateral pins. The femur should
Incomplete Osteotomy and Premature be in 15–20° of abduction to the pelvis to maintain the reduc-
Consolidation tion. A hip extension bar should be added to prevent flexion
If there is a lack of separation of the osteotomy site after a contracture with a hinge. Lengthening may resume with cau-
week of distraction, it may be due to an incomplete osteot- tion (Figs. 22.21, 22.22, and 22.23).
omy or a periosteal hinge that will not separate. Continued If a pelvic osteotomy is performed after lengthening, care
distraction can lead to an acute separation of the bone ends, must be taken not to force the hip into the acetabulum. Since
which is usually very painful and may have an audible pop. the femoral head is osteoporotic, it can be easily crushed
The pain will continue unabated until the bone is acutely with attempts at reduction. If an acute reduction is quite dif-
shortened by a few millimeters. It is important to advise the ficult, then it may be safer to do a gradual reduction with the
patient of this possibility. If the bone does not separate, or if external fixation if the external fixator is still in place. A hip
the patient or parents wish to avoid a painful separation, a dislocation that occurs after frame removal should be treated
repeat osteotomy at the same site should be performed. with an open reduction, capsulorrhaphy, and femoral short-
Overabundant bone formation may lead to premature ening and pelvic osteotomy. The femur should be osteoto-
consolidation. Watch for a mismatch between the distraction mized after the capsulotomy and the femoral head reduced
performed and the radiographic distraction. Increasing the dis- into joint. The bone ends of the femur are overlapped to
traction rate for a few days may prevent a premature consolida- determine the amount of shortening required. It is essential
tion in such cases. Patients should be checked weekly during to shorten. There must be no tension on the femoral head if
this time. If premature consolidation occurs, a repeat osteotomy it is to remain in the acetabulum. It is important to preserve
should be performed at a new location, as a repeat osteotomy the superior capsule; if released, it will lead to recurrent dis-
through the original site is more likely to lead to failure of bone location. If the hip is difficult to stabilize despite all of these
formation. Leave the fixator in tension, as the osteotomy will measures, it can be tethered in place using a suture anchor in
separate easier when cut under tension. After the osteotomy, the the cotyloid notch, with the suture passing through the fovea
tension in the frame should be reduced to normal. and out the lateral neck. This creates a mobile tether that
prevents dislocation while limiting motion. This technique lata and biceps. Anterior or anteromedial subluxation can
was developed by the senior author and has been used since result from extension contractures and patella alta.
2002 (see Fig. 22.23). A more proximal femoral lengthening is less likely to
cause knee subluxation, but it often has narrower and less
Knee Subluxation/Dislocation well-formed regenerate bone, leading to higher fracture risk.
Most cases of CFD usually have hypoplastic or absent cruci- To prevent knee subluxation, the distal fascia lata should
ate ligaments in the knee. The tendency towards flexion con- be released and the external fixator should span the knee and
tracture during lengthening predisposes the tibia to posterior include the tibia. If the fascia is not released, then the knee
subluxation, which is the most common. Posterolateral or can begin to subluxate after only 2–3 cm of lengthening.
external rotatory subluxation may occur with a tight fascia Without tibia fixation, the knee will often start to subluxate
after 4 cm of lengthening. The spanning fixator also protects
the knee cartilage and physis from increased forces. An artic-
ulated hinge can be used to allow the patient to maintain
knee range of motion. Subluxation may be prevented by
keeping the knee in full extension throughout lengthening,
but it will result in a stiff knee. In the knee, posterior or ante-
rior subluxation can be monitored on a lateral knee radio-
graph in full extension [81]. If the knee begins to subluxate,
the rate of lengthening may need to be decreased as well as
more intensive physical therapy for stretching.
If the knee subluxates or dislocates after removal of the
external fixator, it should initially be treated by aggressive
physical therapy, traction, and splinting. Physical therapy
measures include pulling the knee forward and rotating it
inwards during knee extension and flexion range of motion.
If the knee remains subluxated despite an adequate trial
of several months of therapy, it requires a version of the
Superknee procedure called a “rescue knee.” This involves
lengthening of the biceps femoris and the iliotibial band if it
is intact. In some cases, the Langenskiöld is done to reduce
the patella into joint. If there is a fixed flexion deformity, the
femur must be shortened proximally to avoid creating a quad-
Fig. 22.21 The MRS articulated across both the hip and the knee with
riceps lag. In the most severe cases, a posterior capsulotomy
fixation to the pelvis and tibia, respectively. Both the knee and hip
external fixation hinge axes correspond to the knee and hip center of is required. If the knee also has an extension contracture, this
rotation axes in the frontal plane can be combined with a Judet quadricepsplasty (Fig. 22.24).
Fig. 22.22 MRS device in place

on a 2-year-old boy being
lengthened for CFD. The external
fixator spans the hip and knee
with a hinge connection to the
femoral rail. This construct is
shown with the hip and knee in
extension (a), hip flexion (b), and
hip and knee flexion (c)
410 D. Paley et al.
Fig. 22.23 Radiographs of a 2-year-old boy with CFD Paley type 1b cotyloid notch through the femoral head and secured over a washer
subtrochanteric type deformity (a). Following Superhip and Superknee laterally. This washer can just be seen protruding at the upper end of
reconstruction including Dega pelvic osteotomy (b) at age 2. the plate. A periacetabular triple osteotomy (according to Paley’s mod-
Lengthening of the femur at age 3 years, using the MRS device articu- ification, Fig. 22.12) was performed and secured with screws. The hip
lated across to the tibia (c). Radiograph after 4 cm of lengthening dem- remains stable and mobile. Illustration of the open reduction, shorten-
onstrating early lateral subluxation of the hip. Note the increased ing, and tethering technique (k–t). After the pelvic osteotomy, capsu-
femoral head-tear drop space (d). Extension of the external fixator to lotomy, and subtrochanteric osteotomy the femoral head can be
the pelvis with articulated spanning fixation (e). The femoral head is reduced into joint. The quadrilateral plate is drilled (k). The perfora-
well reduced and stabilized permitting continued lengthening up to tion seen from the inside of the pelvis (l). The femoral head is drilled
8 cm. Radiograph at the time of removal of the external fixator with through the fovea (m). After drilling both the femur and the acetabu-
Rush rodding to prevent fracture. Note that the hip appears well lum (n). The suture anchor is introduced into the medial wall of the
reduced and stable (f). Radiograph 3 months later showing dislocation acetabulum (o). The author’s current preference is to use the Juggerknot
of the hip joint (g). Three-dimensional CT scan showing that the femo- (Biomet, Warsaw, IN). This has a soft anchor made of nonresorbable
ral head is dislocated posteriorly (h, i). Note on the axial view (i) that suture material that bunches up when pulled back through the hole (p)
the posterior wall of the acetabulum is hypoplastic. Thus, the acetabu- locking on the inside of the quadrilateral plate of the pelvis. A Keith
lum is retroverted despite excellent superior and lateral coverage from needle is used to pull the suture through the femoral head and neck and
the previous Dega. This confirms the statement that the Dega osteot- greater trochanter (q, r). The suture is tied over a washer to secure the
omy cannot improve the posterior coverage of the hip. A “rescue hip” reduction of the femoral head. The hip remains mobile but not dislo-
procedure was performed. The femur was shortened 2 cm and fixed catable. The distal femur can be overlapped on the proximal femur to
with a plate. An open reduction of the hip was performed. To tether the determine how much shortening is required (s). The distal femur is
femoral head into the acetabulum a suture anchor was used from the shortened and fixed to the plate
Limb Malalignment of the femur through a pin hole or the regenerate. After frame
Limb length equalization should be based on full-length removal, fractures can occur through pin holes or the regener-
standing radiographs. Limb alignment is assessed for the ate bone, usually at the mid-regenerate area or host bone junc-
femur and tibia both separately and in combination. The tion. The incidence of all these type of fractures associated
joint orientation of the knee should be measured using the with CFD lengthening was 34 % compared to 9 % for non-
malalignment test [82]. congenital femoral lengthening [83–85]. In many cases, this
Axial deviation from lengthening (procurvatum and val- was despite the use of a spica cast after removal.
gus for distal femoral lengthening and procurvatum and By prophylactically rodding the femur at the time of
varus for proximal lengthening) is identified and corrected at removal, the risk of refracture is much lower [53]. There is
the end of the distraction phase, when the regenerate bone is still a concern for intramedullary infection, so proper irriga-
still malleable. When there is malalignment of the femur and tion, debridement, and curettage before nail insertion are
tibia, the femoral malalignment is corrected to a normal dis- warranted. Patients are given intraoperative antibiotics and a
tal femoral joint orientation. The femur is not over- or under- 10-day course of oral antibiotics.
corrected to compensate for the tibial deformity. The tibia Prophylactic rodding permits continuation of knee mobility
should be corrected separately, either during the same after removal. To protect from osteoporotic stress fractures
treatment or at a later treatment. Residual malalignment after through the tibial pin holes we hold formal physical therapy for
the external fixator is removed and can usually be addressed a month but permit the patient gentle range of knee motion.
with hemiepiphysiodesis or repeat osteotomy. Rodding also permits weight bearing with a removable spica
cast right after frame removal. Fractures may still occur after
Fractures rodding if the rod is too small or too short. The appropriate
Fractures associated with limb lengthening can occur through- diameter rod should be used in each case. In most small chil-
out and after the lengthening process. Fractures can occur dren the 1/8″ (3.1 mm) Rush rod is used. In larger children the
through pin holes or be pathologic secondary to disuse osteo- 3/16″ (4.65 mm) rod is preferable and in older children the ¼″
porosis. A fall on a frame can cause a femoral neck fracture or (6.2 mm) Rush rod is chosen. In general, the Rush rod is passed
a pin hole fracture, usually the second most proximal pin. as distal as possible to the edge of the distal femoral physis.
Physical therapy manipulation can cause flexion buckle frac- Alternatively, a prophylactic submuscular plate can be
tures of the proximal tibia or distal femur. An unexpected used when the pin sites are too infected to allow safe rodding
muscle spasm during frame removal can cause flexion failure (Fig. 22.25).
Fig. 22.24 Thirteen-year-old girl with CFD undergoing lengthening knee. AP and lateral radiographs of the knee (d) after a Superknee pro-
over nail using Orthofix LRS device articulated across to the tibia to cedure was performed including the Paley reverse MacIntosh proce-
prevent knee subluxation (a). After the nail was locked and the fixator dure, which in this case was secured with two medial suture anchors in
removed, the knee dislocated by an external rotatory subluxation the medial femoral condyle. Because she was skeletally mature an
including dislocation of the patella. Note that this radiograph is a true Elmslie-Trillat transfer of the tuberosity was performed instead of a
AP of the femur but an oblique of the externally rotated tibia (b). This Grammont. Note that the tibia is congruently reduced to the femur. The
radiograph is a true AP of the tibia but an oblique of the femur, which is patella is back in place using the Langenskiöld procedure. This surgery
relatively internally rotated to the tibia (c). AP and lateral radiographs was performed by the author in 1994. The patient maintains excellent
of the knee after a Superknee procedure was performed to “rescue” the stable and painless range of motion to the present date (e, f)
Fig. 22.25 Significant pin-site osteolysis after 8-cm femoral lengthen- contamination of the bone by the pins the risk of intramedullary infec-
ing in a 3-year-old boy after previous Superhip and Superknee proce- tion with Rush rodding was considered to be too high in this case. To
dures. The pin sites are debrided including curettage of the bone, at the prevent fracture a submuscular locking plate was used instead. This
time of removal of the external fixator (a, b). Lateral radiograph show- avoids contact with the pin sites
ing the holes in the bone that make it at high risk of fracture. Due to the
Joint Stiffness and Contracture and hip to be more normally oriented and accept more axial
The knee can develop both flexion and extension contrac- loading. The response to the anatomic change is ossification
tures during lengthening. Although transfixing pins or wires of the proximal femur, converting the CFD from type 1b to
may contribute to difficulty in flexion, even lengthening with type 1a. This conversion usually occurs within 2 years of the
internal distractors may still lead to loss of knee flexion with Superhip procedure, and no lengthening is attempted until
increased length. However, stiffness of the knee is prevent- conversion. The first lengthening can be performed between
able. Surgical release and lengthening of specific soft tissues the ages of 2 and 4, once the femur is of adequate size. If
(fascia lata, rectus femoris) reduce the joint reactive forces initially seen in infancy, patients with type 1a CFD typically
on the knee due to lengthening. They can be done acutely undergo their first lengthening at age 2 years, whereas
with the index procedure, but the soft tissues may heal patients with type 1b CFD typically undergo lengthening
together prior to the end of distraction. A delayed release at closer to age 4 years.
about 6 weeks allows more soft tissue distraction since
the release occurs when the soft tissues are taut from
lengthening. Treatment of Paley CFD Type 2 (Fig. 22.26)
Dynamic splinting can be done by using commercially
available products or office-customized splints such as devel- In comparison to the Paley type 1b neck type, which is
oped by Bhave [86]. The custom knee device consists of two sometimes referred to as a stiff pseudoarthrosis of the fem-
fiberglass casting material cuffs for the thigh and lower leg oral neck, the Paley type 2 CFD is a mobile pseudoarthrosis
connected by cast brace hinges. The elastic forces are applied of the femoral neck. In type 2 there is a fibrous anlage of
using elastic bandages anteriorly off of various towers sus- the femoral neck present extending from the base of the
pended proximal and distal to the knee. This is a very effi- greater trochanter to the femoral head. There may be a
cient and inexpensive type of dynamic splinting. well-formed synovial joint present with a mobile femoral
Physical therapy is essential to successful CFD length- head (type 2a), partially formed synovial joint with a par-
ening. This is especially true for the knee, and one should tial fusion of the femoral head to the acetabulum at one spot
not consider lengthening a CFD case without outpatient (type 2b), or an absent or circumferentially fused femoral
PT. If the knee gets stiff in flexion despite adequate reha- head (type 2c). The defining anatomic feature that charac-
bilitation, then a quadricepsplasty should be performed. terizes all three of these is the presence of a greater tro-
Rozbruch recommends doing this at or shortly after frame chanteric apophysis, which is missing in Paley type 3.
removal [87]. He cuts the central tendon of the quadriceps Radiographically, the apophysis can be recognized when
while leaving the medial and lateral musculature intact. He the proximal end of the femur appears rounded, while in
claims that this does not lead to a quadriceps lag. This most Paley type 3 cases, the upper end of the femur appears
author prefers to do a full Judet quadricepsplasty [88] sharp and pointed. An MRI makes this distinction much
instead. With a concomitant flexion contracture, either an more definitive.
external fixator with gradual distraction or an open poste- There are three approaches for treatment for Paley type 2:
rior capsule release can be performed. (1) rotationplasty versus Syme amputation and prosthetic fit-
ting, (2) serial lengthenings with pelvic support osteotomy at
skeletal maturity, or (3) Superhip 1.5 or Superhip 2 proce-
Difference in Treatment of Types 1a and 1b dures for selective cases of types 2a and some type 2b.
In general, Paley CFD type 1a (normal ossification) has less

deformity, deficiency, and discrepancy in the hip and knee, in Superhip 1.5 Procedure for Treatment
comparison to type 1b (delayed ossification). The distinction of a Mobile Proximal Femoral Pseudoarthrosis
between types 1a and 1b should be made while the patient is with a Mobile Femoral Head with Cartilaginous
in infancy, because the natural history of type 1b is to ossify. Remnants of the Femoral Neck Present
Therefore, adult type 1b cases may appear to be severe type (Fig. 22.27)
1a cases. Most type 1a cases do not require the complex
Superhip reconstruction. In the author’s experience, approxi- This procedure was developed in 2010. It is used when there
mately half do require pelvic osteotomy before lengthening, is a type 2b with part of the femoral neck present on the
and all cases should have extension of the fixator across the femur and the femoral head. It is carried out similar to the
knee to protect the knee joint. Superhip 1, but with exposure of the anterior hip capsule. An
The strategy of treatment for type 1b is to correct all the arthrotomy is made, and the ends of the femoral neck are
associated deformities, which will allow the proximal femur identified. A blade plate is used as described previously,
414 D. Paley et al.
Fig. 22.26 An 18-year-old girl with bilateral Paley type 2a CFD. AP in a crouch gait. The goal of treatment was to make her stand and walk
radiograph of pelvis shows femoral neck remnants on either side of the erect by eliminating the flexion deformities of both hips and knees. This
pseudoarthrosis while on the left side there is no femoral neck (a). was achieved by the Superhip 1.5 on the left and, the Superhip 2 on the
Three-dimensional CT scan cuts show the femoral neck remnants from right, and the Superknee with capsulotomy bilaterally. The posttreat-
the front (b), oblique (c, arrows), and back (d) views of the right hip ment radiographs showing the new erect posture and the healed femoral
compared to a pseudoarthrosis without any neck remnant on the left (d, neck pseudoarthrosis are also seen (g). Her clinical photograph after
circle). Her standing posture is shown clinically (e) and radiographi- recovering from bilateral Superhip procedures. She now walks longer
cally (f). She has fixed hip and knee flexion contractures and very dis- distances without fatigue (h). Since she stands and walks without a
proportionately short thighs. Note that her hands reach down below her crouch gait her upper extremities hang to a more normal level than
knees. Due to the fixed flexion deformities, she fatigues when walking before
except that the cannulated chisel is introduced to each Superhip 2 Procedure for Treatment
segment independently. The blade plate is then inserted of a Mobile Proximal Femoral Pseudoarthrosis
across to stabilize the two parts. BMP-2 is used to induce the with or Without Partial Fusion of the Femoral
two parts of the cartilaginous femoral neck to ossify and Head (Figs. 22.28 and 22.29)
unite. This is referred to as “cartilage welding.” This is a
unique feature of BMP-2 that was first identified by the The same approach as the Superhip is used. The operation
senior author when treating the unossified femoral neck of remains the same until the subtrochanteric osteotomy. Before
type 1b [14] (see Fig. 22.24) [14]. performing the osteotomy, the region of the femoral neck is
Fig. 22.27 Superhip 1.5 technique. (a) This is indicated for Paley type neck are approximated. (c) The femur is osteotomized, shortened, and
2a when there is a remnant of cartilaginous femoral neck remaining on corrected as for the Superhip procedure and fixed with a blade plate.
the proximal femur and femoral head. Through the same surgical expo- BMP-2 is inserted into the superior and inferior neck across the carti-
sure of the Superhip procedure (see Fig. 22.7) the anterior capsule of laginous pseudoarthrosis. It is used to “weld” the cartilage together by
the hip is exposed by first separating the quadriceps from the gluteus inducing it to form bone. This is an off-label use of BMP-2. (d) The
medius. The capsule is then opened in line with the femoral neck. (b) forces on the femur are neutralized by a spanning external fixator from
The fibrous anlage is resected and the cartilaginous parts of the femoral the pelvis to the femur
dissected. The level of the acetabulum is identified with the area previously decorticated. The distal femur intramedul-
image intensifier and the femoral head and acetabulum are lary diaphysis is prepared with a 3.2-mm drill bit. Dental
identified. The capsule of the femoral head is opened inferi- wire is also placed around this segment underneath the soft-
orly. The femoral head is moved in the acetabulum with for- tissue pedicles, in preparation for fixation.
ceps. If it is type 2a, it will be mobile. If it is type 2b, it will The new femoral neck is then attached to the head. The
not move. If there is a cartilaginous or bony bridge, it is four threaded K-wires are advanced carefully, holding the
always located between the femoral head and the ischium. head with the dental wire. Verify that the wires are not intra-
The cartilage of the head is cut back to the ossific nucleus to articular with fluoroscopy and a Freer elevator. After the
expose bone. Small 1-mm slices are cut back until the ossific threaded K-wire fixation, carefully tighten down the dental
nucleus is widened to about 2/3 of the femoral head. Dental wire, compressing the femoral neck to head junction.
wire may be passed through the nucleus for additional The second subtrochanteric osteotomy is then made at
fixation. 45° to the shaft of the femur, removing a trapezoidal piece to
The femoral neck is made from the proximal femur and provide adequate shortening of the femur. The cut should be
greater trochanter. Posteriorly, the interval between the made with the leg internally rotated to create femoral neck
vastus lateralis and the abductors is found, and all of the anteversion. The Rush rod is then placed through the tro-
abductors are released off of the proximal femur. The vastus chanteric piece into the femoral shaft, and the dental wire
lateralis and medial femoral circumflex artery serve as the tightened down. A cannulated 4.0 screw may be placed per-
pedicle for this bony segment. The external rotators are also pendicular to the shaft cut at 45° to augment fixation if there
removed. The proximal lateral cortex is partially decorti- is adequate room. The abductor slide is performed as needed,
cated, as it will eventually be oriented inferiorly and attached and the abductors are repaired to the greater trochanter. To
to the femoral shaft. The first cut is made about 4–5 cm long, neutralize the forces on the femoral head and neck, an exter-
perpendicular to the femoral shaft, creating the new femoral nal fixator is applied from the pelvis to the tibia for 3 months.
neck. This segment is rotated 135° on its soft tissue pedicle;
the greater trochanter moves distal and lateral, and the distal
cut end of the subtrochanteric osteotomy is rotated superi- Pelvic Support Osteotomy
orly to fix to the ossific nucleus of the femoral head.
The trochanteric segment is predrilled with a 1.5-mm drill A pelvic support osteotomy is used to treat most type 2c and
in four places around its cortical periphery, and 5/64″ some type 2b cases, combined with a distal femoral lengthen-
threaded K-wires are run retrograde through the femoral ing and realignment osteotomy (Fig. 22.30). This combination
neck and set to the same length. This bone is then drilled at is called the Ilizarov hip reconstruction [89]. Pelvic support
45° to its long axis for insertion of a Rush rod. This is drilled osteotomy is usually enough to prevent proximal migration of
in a way to miss the path of the four wire holes and into the the femur during lengthening for non-congenital pathologies.
416 D. Paley et al.
Fig. 22.28 Superhip 2 (SH2) technique. (a) After the same exposure 45° with the distal femur rotated 15° internally for anteversion. The
as in the Superhip (see Fig. 22.7). The capsule of the hip is exposed femur is shortened according to the soft-tissue tethers. (e) The femur is
without taking down the quadriceps muscle. The psoas tendon is fixed using a Rush rod, tension band wire, and compression screw tech-
released from the lesser trochanter. The femoral neck anlage is resected. nique. (f) The psoas tendon is reattached. (g) The hip abductors and
The inferior capsule is opened. (b) All of the muscles inserting into the rotators are reattached. If the abductors are too short, then the apophysis
proximal femur are detached including all of the glutei, external rota- is split and an abductor slide is carried out. (h) A spanning external
tors, and psoas. The muscles that originate on the proximal femur such fixator from the pelvis to the femur to the tibia is used. In most cases
as the quadriceps are not disturbed. The quadriceps becomes the vascu- there is a fixed flexion deformity of the knee. A posterior capsulotomy
lar pedicle for the proximal femur and greater trochanter. The femur is Superknee procedure is performed and pinned with cross K-wires to
osteotomized in the subtrochanteric region. It is then mobilized and hold the extension correction. The capsulotomy and knee extension
moved distally. (c) The proximal femur is rotated 135° to form a femo- should be performed after the first osteotomy but prior to the shortening
ral neck. (d) The femoral neck is fixed to the head using threaded osteotomy of the femur. (i) MRS device used to span the hip and knee
K-wires and a cerclage wire. The second osteotomy of the femur is at joints and neutralize the forces on the femoral neck for 4 months
Fig. 22.29 (continued) contraindication for the Superhip 2 procedure. after removing the external fixator (j). Note that the Rush rod reaches to
Modification of the technique has made it a viable option in the author’s the distal femoral physis. Radiograph 2 years later showing that there
current clinical practice. The critical consideration is rotating the fused has been a lot of growth of the distal femur as seen relative to the end of
area of the femoral head to face and join to the “new” femoral neck. the Rush rod (k). Hip and knee range of motion after recovery from
Intraoperative fluoroscopic image (g); during this procedure, after fixa- Superhip 2 procedure (l, m). First lengthening of the femur with MRS
tion with threaded K-wires, cerclage wire and Rush rod are in place. and articulated fixation spanning the knee joint. The hip joint was left
After application of the spanning external fixator (h, i). Knee capsulot- free (n). The femur was successfully lengthened 8 cm with no loss of
omy and acute extension of the knee flexion contracture were carried hip or knee motion
out and temporarily fixed with cross K-wires (h, i). Final radiograph
Fig. 22.29 Standing AP and lateral radiographs of patient with Paley The greater trochanter is out of plane for this cut (d). MRI transverse
type 2b CFD (a, b). AP pelvis radiograph showing the rounded proxi- cut showing the femoral head in the acetabulum (e). Note the bridge of
mal end of the femur indicating that it has an apophysis. It is proximally bone posteriorly between the femoral head and the ischium (arrow).
migrated. There is an obvious femoral head ossific nucleus with a well- The rest of the femoral head is covered with cartilage and remains
formed acetabulum present. The arrow is pointing to the greater tro- unfused. The same can be seen on the coronal cut of the posterior femo-
chanteric apophysis (c). MRI coronal cut, showing the ossific nucleus ral head (arrow) (f). This is therefore classified as a partial fusion of the
with no bony or cartilaginous connection to the proximal femur (arrow). femoral head (Paley type 2b). Type 2b was originally thought to be a
418 D. Paley et al.
Fig. 22.30 AP radiograph (a) of 10-year-old girl with Paley type 2c tinued lengthening as long as she was able to maintain knee motion.
CFD left side (absent femoral head). She was treated by pelvic support The pelvic support angulation remodeled and the leg length difference
osteotomy (b) and distal femoral lengthening (13 cm). This is an was 10 cm at skeletal maturity (c). At age 16 she underwent a second
unusual and extreme amount for one lengthening. The only reason we pelvic support osteotomy with 10-cm lengthening (d). Final radio-
exceeded the 8-cm limit is that she was able to maintain knee range of graphic result showing limb length equalization (e). She has excellent
motion to 90° and for financial reasons was not sure if she would ever function (f) and walks with no appreciable limp. She is now 20 years
be able to afford travelling for another lengthening. We therefore con- from her original surgery
For congenital pathologies, the fixation should be extended to equal to the total amount of adduction of the hip plus 15° of
the pelvis to prevent proximal migration of the femur. In overcorrection. The proximal osteotomy should also be
young children with very short femora, the femur may be too internally rotated and extended. The amount of rotation is
small to perform both the pelvic support and the distal length- judged by the position of the knee relative to the hip in maxi-
ening osteotomies. In addition, the valgus component of the mum adduction. The amount of extension depends on the
pelvic support osteotomy may remodel straight. amount of hip fixed flexion deformity.
The proximal osteotomy is performed at the level at The level of the distal osteotomy is determined by the
which the proximal femur crosses the ischial tuberosity in intersection of the proximal and distal mechanical axes.
maximum cross-legged adduction. The amount of valgus is The proximal mechanical axis passes through the proximal
Fig. 22.31 (a) CFD Paley type 3A. Note the initial knee flexion and underwent her fourth femoral plus tibial lengthening of 12.5 cm. She
extension for an arc of motion of 70°. Parents refused to have a Syme’s had a final tibial lengthening of 2.5 cm to correct deformity and equal-
or a rotationplasty. She was treated at age 7 with a lengthening of the ize her leg lengths (d). Her total lengthening was 50 cm. She maintains
femur and tibia for a total of 12.5 cm (b). At age 10 she underwent the 70° of knee motion and good ankle and hip motion. She has excellent
same treatment again and achieved another 12.5 cm of lengthening. At clinical function, has no pain, and walks with minimal limp. She is now
age 14 when she was skeletally mature she underwent a pelvic support 18 years following her first procedure
osteotomy together with a 10-cm femur lengthening (c). At age 18 she
formal osteotomy site and is perpendicular to the horizontal thetic reconstruction surgery with either rotationplasty or a
line of the pelvis. The distal axis is from the center of the Syme amputation. Limb reconstruction surgery has a role in
ankle to the center of the knee. Varus correction can be made these cases and can equalize an LLD. Because the number of
through the distal osteotomy. The external fixator must still these patients treated this way is small, the ultimate func-
be extended to the tibia with hinges, as previously discussed. tional result for these cases is still not predictable.
Treatment of CFD Type 3 Limb Reconstructive Surgery for Type 3
Deficiency of the proximal femur with absent femoral head, Limb reconstructive surgery is most applicable to type 3a
greater trochanter, and proximal femoral metaphysis results cases that have functional knee range of motion (Fig. 22.31).
in a mobile pseudoarthrosis and a very short femoral rem- Many of these patients have knee and hip flexion contrac-
nant. The Paley type 3a CFD has a mobile knee with at least tures. These are treated by soft tissue releases, using the
45° of motion, usually with a 45° knee flexion deformity. same approach as described above for type 1 CFD. The fas-
The type 3b has a stiff knee with less than 45° of motion and cia lata is reflected proximally, and the quadriceps and
usually has a greater flexion deformity. The most predictable abductors are elevated off the proximal femur. The psoas
and reliable treatment option in these cases remains pros- tendon is absent, but the rectus and sartorius are present.
420 D. Paley et al.
Fig. 22.32 Rotationplasty for CFD type 3. (a) Brown rotationplasty. lateral fusion position. (b) Paley-Brown rotationplasty. A Chiari oste-
The femur is fused to the side of the pelvis. It is difficult to align the otomy is used to fuse to the femur. The ischium is notched to make
femur perpendicular to the pelvis. The hip protrudes laterally due to its room for the sciatic nerve
Any fibrous femoral anlage is resected, and frequently, some Prosthetic Reconstructive Surgery for Type 3
of the cartilaginous femoral anlage may need to be trimmed.
The proximal femur is freed from these attachments, includ- Prosthetic fitting is possible without any surgery. However,
ing the hip capsular remnants, permitting it to move proxi- this can be difficult with a hip and knee flexion contractures,
mally without a soft-tissue tether. This is important for the as well as a foot not in equinus. Surgical releases as described
acute correction of the knee contracture. The long lateral above may help with prosthetic fitting. A Syme or Boyd
incision is extended distally and the peroneal nerve is decom- amputation may also help create a residual limb that is fitted
pressed and protected. Because the femur is so short, it is easily. In some cases, even a pelvic support osteotomy may
advisable to explore the peroneal nerve and follow it proxi- be considered to decrease limp and stabilize the hip joint.
mally to the hip joint region prior to significant release. This The other approach to prosthetic reconstruction is with
will prevent injury to the sciatic nerve, as it passes very near rotationplasty [49, 90]. Torode popularized the Van Nes
the dissection around the hip capsular remnant. rotationplasty for patients with CFD. They used a long
The lateral approach to knee flexion contracture release of oblique incision. The goal was to fuse the residual knee and
the posterior capsule is performed, and the knee joint is fully rotate the limb 180°, such that the foot points backward and
extended. A 2-mm Steinmann pin may be drilled across the the ankle can function as a knee joint. This was most appli-
knee joint from the femur into the tibia to maintain full knee cable to cases in which the ankle was already at the level of
extension. During the same operation, a monolateral fixator the opposite knee joint. Brown [44] modified the rotation-
is placed from the pelvis to the femur and tibia. The most plasty approach, using a racquet-like incision, performing
important and strongest pelvic pin is one from the anterior the rotation between the femoral remnant and the pelvis
inferior iliac spine oriented towards the greater sciatic notch, (Fig. 22.32a). He then fused the femur to the lateral aspect
using the cannulated drill technique. Another pin from the of the ilium, thus converting the knee into a hip joint and
lateral side in the supra-acetabular region is attached to the ankle into a knee joint. This provided improved hip sta-
the fixator. One pin is used in the distal femur and two in the bility over that provided by the Van Nes rotationplasty.
tibia. The fixation is kept in place for 6 weeks to maintain However, one problem with the Brown method is excessive
knee and hip extension. Once fixation is removed, range of shortening of the hip muscles and lateralization of the hip
motion exercises are started to regain knee motion. and lower limb. It is also challenging to adjust the align-
After the above procedures, the femur can be lengthened. ment correctly.
The external fixator is placed from the pelvis to the femur to Paley modified the Brown technique in 1997 by adding
the tibia. The femur is lengthened up to 8 cm. The knee joint a Chiari osteotomy of the pelvis and fusing the femoral rem-
is distracted, but since no knee or hip hinges are used when nant to the cancellous roof of ilium (Figs. 22.32b and 22.33).
the femur is too short, there is little need for physical therapy The detached muscles are not removed and are instead
during this first lengthening. The goal of this first lengthen- transferred distally as much as possible. Care is taken to
ing is to convert a type 3a femur to a type 2b femur. The rest appropriately shorten the knee muscles so that they can func-
of the treatment is the same as a type 2b: serial lengthenings tion adequately as hip flexors and extensors. The fascia lata
and eventually a pelvic support osteotomy (Fig. 22.31) com- connected to the gluteus maximus is reattached to the tibia to
bined with a final lengthening. Since the predicted discrep- serve as a hip abductor. Posterior capsule release of the knee
ancy ranges between 30 and 40 cm, at least four lengthenings is performed as the knee is often contracted as much as 90°.
and an epiphysiodesis are required to equalize limb lengths. The peroneal nerve is decompressed to prevent injury and
Fig. 22.33 (a) Preoperative radiograph of a 10-year-old boy with CFD fusion to the pelvis and the closure of the distal femoral physis. Sitting
Paley type 3A. Note how the knee is fully flexed and the hip is rotated posture with excellent hip flexion and active “knee extension” (via
into a frog leg position. Standing AP (b) and lateral (c) radiographs ankle plantar flexion) (e), and “knee flexion” (via ankle dorsiflexion)
after Paley-Brown rotationplasty using the prosthesis. The ankle is at (f). Active extension (g) and flexion (h) of the knee wearing the
the level of the opposite knee. Closeup radiograph (d) showing the prosthesis
allow greater rotation. A supramalleolar osteotomy may be The senior author has recently developed a new rotation-
performed to correct the internal rotation deformity of the plasty variant, which for labeling purposes will be called the
tibia. Epiphysiodesis is done with the same screws used for Paley rotationplasty (Fig. 22.34). The steps are the same as in
fixation of the femur to the pelvis. This modified Paley- the Brown, but instead of fusing the femur to the pelvis, the
Brown rotationplasty provides good functional results with femoral segment is fixed to a mobile femoral head. This gives
better hip function and stability than the Van Nes and the better hip reconstruction with active abduction motion pres-
Brown rotationplasties. ent. Even if the femoral head is fused to the acetabulum, as in
422 D. Paley et al.
Fig. 22.34 (a) Paley rotationplasty. The femoral remnant is fused to femoral head was fused to the femoral head (d). He has excellent active
the femoral head, which is made mobile in the acetabulum. (b) 2-year- hip flexion (e) and abduction (f) of the hip joint. Hip flexion is usual but
old boy with type 3b. (c) Paley rotationplasty was performed and the hip abduction is not usually possible after a Brown rotationplasty
Fig. 22.35 Twelve-year-old girl

with CFD Paley type 3c (a). She
had a rotationplasty performed
with insertion of the proximal
medial tibia into the acetabulum
(b). This gave her a stable hip
with excellent function of the hip
and knee joints
type 2b, it can be freed up similar to the Superhip 2 and then

fused to the femoral remnant (see Fig. 22.34). Finally, in cases Summary
where there is no knee joint present (type 3c), the proximal
tibia or fused distal femoral remnant can be fused to the resid- CFD is a spectrum of congenital deficiency, deformity, and
ual femoral head. If there is no femoral head, then a roof can discrepancy of the femur that involves not only the osseous
be created by a Chiari osteotomy or by creating a depression part of the femur, but also the surrounding musculature, liga-
into the pelvis (Fig. 22.35). This is stabilized by a suture ligaments, and joints. All of these components must be addressed
ment tether technique as previously shown (see Fig. 22.23). if one is to be successful in the treatment of CFD. Though the
definitive origin of CFD is still not known, it is a problem a newly recognized syndrome? Am J Med Genet A. 2014;164A(2):
287–90. doi:10.1002/ajmg.a.36273. Epub 2013 Dec 5.
that can affect the function of a patient for his or her entire
14. Paley D, Harris M, Chiari C. Can BMP2 Combined with the
life. Depending on the severity, there are several treatment SUPERhip Procedure Lead to Ossification of the Unossified
strategies, reconstructive surgeries, and prosthetic options, Femoral Neck & Lower Recurrence of Coxa Vara in Severe
all of which can improve a patient’s gait, function, and qual- Congenital Femoral Deficiency? Presentation. EPOS 32nd Annual
Meeting, Athens Greece, April 2013 and LLRS, New York, NY,
ity of life. As each deformity is different, so also is each
July 2013. Abstract in J Child
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to their individual needs and cultural expectations. Advances miological comparison of the naturally occurring condition with that
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16. Zhang Y, Wray AE, Ross AC. Perinatal exposure to vitamin A dif-
nologies have expanded the reconstructive indications and
ferentially regulates chondrocyte growth and the expression of
options in lieu of amputation. Though more research of aggrecan and matrix metalloprotein genes in the femur of neonatal
newer treatments and their long-term outcomes are needed, rats. J Nutr. 2012;142(4):649–54.
there are now more options and hope for a brighter future for 17. Aitken GT. PFFD vs: PFFD—definition, classification and man-
agement. 1969. Limb Development and Deformity: Problems of
a child with CFD.
Evaluation and Rehabilitation. Springfield, IL: Charles C. Thomas;
1969.
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Bisphosphonate rescue in distraction osteogenesis: a case series. 87. Khakharia S, Fragomen AT, Rozbruch SR. Limited quadriceps-
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shortened lower extremities using cylindrical allografts. Clin Epub 2009 Jun 25.
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Fibular Hemimelia: Principles
and Techniques of Management 23
John E. Herzenberg, Lior Shabtai, and Shawn C. Standard
forefoot ray deletion (44 %). Equinovalgus is much more

Introduction common than equinovarus. Roux and Carlioz [7] demon-
strated absence of the anterior cruciate ligament in 95 %
Congenital fibular deficiency or fibular hemimelia (FH) is a of the cases. In conclusion, FH can present with a broad
rare congenital disorder in which the fibula is partially or spectrum of severity, from mild cases to very severe
completely absent. It is the most common congenital long manifestations.
bone deficiency, and the incidence in the USA is between 7.4 The treatment of FH changes according to the severity of
and 20 cases per million live births (up to 1 per 50,000 live deformity and the associated skeletal manifestation with a
births) [1–3]. It is primarily associated with lower extremity broad spectrum of choices: from simple contralateral epi-
anomalies such as femoral shortening, hypoplastic lateral physiodesis in mild cases to limb reconstruction and length-
femoral condyle, genu valgum, anteromedial bowing of the ening in more severe cases, the latter composed of multiple
tibia, limb length discrepancy (LLD), absent or deficient surgical procedures during the patient’s childhood and ado-
anterior cruciate ligament (ACL) or posterior cruciate liga- lescence. The common alternative to extensive lengthening
ment (PCL), ball-and-socket ankle joint, equinovarus or is amputation and prosthetic rehabilitation [5, 8–16]. The
equinovalgus foot, tarsal coalition, and absence of lateral challenge in limb lengthening is to achieve normal limb
rays of the foot (Fig. 23.1). Upper extremity manifestations alignment and length with a functional, painless, plantigrade
may include syndactyly and ulnar hemimelia [4, 5]. foot. This undertaking is often characterized by repeated sur-
Rodriguez-Ramirez et al. [6] examined the prevalence of gical procedures and a high rate of complications and
associated congenital osseous anomalies in patients with sequelae such as pin tract infections, residual LLD, delayed
fibular hemimelia and found that lateral femoral condyle union, ankle and knee stiffness, refracture, knee subluxation,
hypoplasia was the most common associated anomaly and residual foot deformities. Deciding which treatment to
(93 %) followed by ball-and-socket ankle joint (80 %), apply (lengthening versus amputation) can be challenging
congenital short femur (72 %), tarsal coalition (51 %), and for the surgeon and the family [8–12].
Parents must be aware of all the options and the associ-
ated potential complications before making treatment deci-
sions. Fortunately, very little can be done during the first year
of life in most cases (unless the child has an associated club
foot), so there is ample time to meet with the parents, discuss
J.E. Herzenberg, M.D., F.R.C.S.C. (*) • S.C. Standard, M.D. the options, and have them carefully consider which option
International Center for Limb Lengthening, Rubin Institute for
Advanced Orthopedics, Sinai Hospital of Baltimore, is best for their family and their child.
Schoeneman Building, 2nd Floor, 2401W. Belvedere Ave, The aim of this chapter is to discuss in detail the princi-
Baltimore, MD 21215, USA ples and techniques of management of lengthening recon-
e-mail: [email protected]; struction for fibular hemimelia by understanding and
[email protected]
addressing each deformity in this complex, multifaceted dis-
L. Shabtai, M.D. order. A detailed description of the amputation options
Department of Pediatric orthopedics, Tel Aviv Sourasky
Medical Center, Dana Children’s Hospital, Tel Aviv 64239, Israel (Syme or Boyd) are covered in the amputation chapter
e-mail: [email protected] (Chap. 13) by J.A. Herring.

428 J.E. Herzenberg et al.
Fig. 23.1 AP (a) and lateral (b) view radiographs show 10-month-old mild genu valgus and ankle valgus. AP (e) and lateral (f) view radio-
infant with fibular hemimelia, concurrent congenital femoral defi- graphs of a 12-year-old patient with fibular hemimelia who has genu
ciency, a typical anterior bow of the tibia, and equinovalgus foot defor- valgus, mild leg length discrepancy, a ball-and-socket ankle joint, and
mity. AP (c) and lateral (d) view radiographs show a 2-year-old child talocalcaneal coalition. Reprinted with permission from the Rubin
with fibular hemimelia and congenital femoral deficiency as well as Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Box 23.1. Typical Deformities Associated with FH

Classification
• Tibial shortening
Several classifications exist for fibular hemimelia. The earli-
• Equinovalgus foot
est published classification is the Coventry and Johnson [17]
• Missing all or part of fibula
classification from 1952 (Fig. 23.2). They divided patients
• Missing one or more lateral rays with FH into three types:
• Talocalcaneal coalition
• Anteromedial bowing of tibia Type I: Partial unilateral absence of fibula; normal or slight
• Cruciate ligament insufficiency bowing of the tibia with some shortening of the limb; the
• Genu valgum foot is normal or slightly deformed.
• Hypoplasia of lateral femoral condyle Type II: The fibula is completely or almost absent; anterior
• Limb length difference bowing of the tibia with skin dimpling; deformed ankle
• Syndactyly joint; deformed foot with absent rays.
• Ball-and-socket ankle joint Type III: Includes type I or type II but is associated with
• Equinovarus foot other congenital deformities or bilateral involvement.
The Achterman and Kalamchi classification, from 1979

[18], is the most commonly used (Fig. 23.3). They divided
the patients into two groups with one subdivision:
23 Fibular Hemimelia: Principles and Techniques of Management 429
Type I Type II Type III
Fig. 23.2 Coventry and Johnson classification of FH: Type I: partial uni- tibia with skin dimpling, deformed ankle joint, and deformed foot with
lateral absence of fibula, normal or slight bowing of the tibia with some absent rays. Type III: includes type I or II associated with other congeni-
shortening of the limb, and the foot is normal or slightly deformed. Type tal deformities or bilateral involvement. Reprinted with permission from
II: fibula is completely or almost entirely absent, anterior bowing of the the Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Normal
Type IA Type IB Type II
3-year-old child
Fig. 23.3 Achterman and Kalamchi classification of FH: Type I: I-B: 30–50 % partial fibular absence, fibula does not articulate with the
incomplete fibular deficiency, subdivided into I-A and I-B. Type I-A: talus. Type II: complete fibular deficiency, with or without a tiny distal
proximal fibular epiphysis is distal to the level of the tibial growth plate, fibular remnant. Reprinted with permission from the Rubin Institute for
distal fibular growth plate is proximal to the dome of the talus. Type Advanced Orthopedics, Sinai Hospital of Baltimore
Type I: Incomplete fibular deficiency; Type I is subdivided 4. Foot: Numbers of rays.

into: Type I-A: The proximal fibular epiphysis is distal to
the level of the upper tibial growth plate and the distal Birch et al. [20] in 1998 presented a new classification
fibular growth plate is proximal to the dome of the talus; system based on the clinical status of the foot and the magni-
and Type I-B: 30–50 % fibular shortening and the fibula tude of limb shortening as a percentage of the contralateral
does not articulate with the talus. limb on radiographs in order to anticipate the extent of defor-
Type II: Complete fibular deficiency, with or without a small mity at maturity and to recommend the appropriate treatment
distal fibular remnant. required (Fig. 23.5). In 2011, Birch later [5, 21] modified the
treatment proposed: fewer amputations and more reconstruc-
The Stanitski classification, from 2003 [19] (Fig. 23.4), is tion procedures. Cases are divided into two categories:
a morphologic description that divides FH into four catego-
ries based primarily on radiographic presentation: Type 1: Functional foot, in which the foot has at least three rays
and can provide a stable weight-bearing platform. Type 1 is
1. Fibula: Normal, partially absent, or completely absent. divided into four subtypes according to the percentage of
2. Ankle joint: According to the morphology of the tibiota- total limb shortening compared with the contralateral side.
lar joint: horizontal, valgus, or ball-and-socket joint. Type 1A: <6 % leg length inequality; the treatment is
3. Tarsal bones: Tarsal coalition present or absent. orthosis or contralateral epiphysiodesis.
Fig. 23.4 Stanitski classification Fibula

of FH according to four
parameters: 1. Fibula: normal, Normal Type I Type II Type III
partially absent, or completely (Nearly normal) (Small) (Absent)
absent. 2. Ankle joint: according
to the morphology of the
tibiotalar joint: horizontal,
valgus, or spherical (ball-and-
socket joint). 3. Tarsal bones:
tarsal coalition present or absent.
4. Foot: numbers of rays.
the Rubin Institute for Advanced
Orthopedics, Sinai Hospital of
Baltimore
Tibiotalar joint
H V S
(Horizontal - (Valgus) (Spherical)
Normal)
Presence of tarsal coalition
S C
(Without) (With)
Number of rays
5 4 3 2 1
Type 1B: 6–10 % leg length inequality; the treatment is Type 2: Nonfunctional foot. Type 2 is subdivided into Types
epiphysiodesis ± lengthening. 2A and 2B according to the functionality of the upper
Type 1C: 11–30 % leg length inequality; the treatment is extremity.
one or two lengthening procedures ± epiphysiodesis or Type 2A: Upper extremities are functional; therefore, the
extension orthosis. treatment proposed is early amputation.
Type 1D: >30 % leg length inequality; the treatment pro- Type 2B: Upper extremities are nonfunctional, and so
posed is more than two lengthening procedures or foot amputation is contraindicated, as the foot must act
amputation or extension orthosis. as a replacement for the upper extremity.
Fig. 23.5 Birch classification of

At least three rays and functional upper extremity
FH. Type 1: Functional foot, foot
with at least three rays that can Type 1A Type 1B Type 1C Type 1D
provide a stable weight-bearing
surface. Type 1 is divided into
four subtypes according to the
limb length difference compared
with the normal side. Type 1A:
<6 % limb length inequality;
Type 1B: 6–10 % limb length
inequality; Type 1C: 11–30 %
limb length inequality; Type 1D: Overall length
>30 % limb length inequality. (pelvic crest
Type 2: Nonfunctional foot to tibial plafond)
(fewer than three rays). Type 2 is
compared with
subdivided into Types 2A and 2B
contralateral side
according to the functionality of
the upper extremity. Type 2A:
Functional upper extremity; Type
2B: Nonfunctional upper
extremity. Reprinted with
permission from the Rubin > 30%
Institute for Advanced
Baltimore 11–30%
6–10%
< 6%
Contralateral
side
Two or fewer rays

Functional Non-functional
upper extremity upper extremity
Type 2A Type 2B
A more recent classification proposed by Paley [16] is

Box 23.2. Classification Schemes Used in North surgically oriented (reconstruction, not amputation)
America for FH (Fig. 23.6). He classified FH into four types, based on the
• Coventry and Johnson ankle pathology:
• Achterman and Kalamchi
• Stanitski Type 1: Stable normal ankle.
• Birch Type 2: Dynamic valgus ankle.
• Paley Type 3: Fixed equinovalgus ankle. Type 3 is divided into four
subtypes according to the location of the valgus deformity.
Fig. 23.6 Paley classification of

FH. Type 1: Stable normal ankle. Type 1: Stable normal ankle Type 2: Dynamic valgus ankle
Type 2: Dynamic valgus ankle.
Type 3: Fixed equinovalgus
ankle. Type 3 is further LDTA = ADTA =
subdivided according to the 85 – 90° 80 – 85°
etiology of the valgus into 3A
(ankle), 3B (subtalar), 3C
(combined ankle and subtalar),
and 3D (talar). Type 4: Fixed
equinovarus ankle (clubfoot).
Baltimore Type 3: Fixed equinovalgus ankle
3A: Ankle type 3B: Subtalar type
ADTA≥90° ADTA < 90°
3C: Combined ankle and

3D: Talar type
subtalar type
ADTA≥90° ADTA < 90°
Type 4: Fixed equinovarus ankle Ossified bone

(clubfoot type)
Non-ossified bone
ADTA≥90°
Type 3a: Ankle type. and a supramalleolar reorientation osteotomy. For Type 3,
Type 3b: Subtalar type. soft tissue lengthening (peroneal tendons and tendo Achilles),
Type 3c: Combined ankle and subtalar type. resection of the fibrous anlage and interosseous membrane,
Type 3d: Talar body type. and a reorientation osteotomy is recommended. This has
Type 4: Fixed equinovarus ankle (clubfoot). been nicknamed the “Super-ankle procedure” and will be
described later in the chapter. The osteotomy site varies
For Type 1, tibial lengthening is recommended, along according to whether the deformity is classified as Type 3a,
with tendo Achilles lengthening. For Type 2, tibial lengthen- 3b, 3c, or 3d. Type 3a needs a supramalleolar osteotomy,
ing is recommended, along with tendo Achilles lengthening type 3b needs a subtalar osteotomy, Type 3c needs both
supramalleolar and subtalar osteotomy, and Type 3d, which maturity allows the surgeon to propose various treatment strate-
is very rare, may be treated with an opening wedge osteot- gies. For example, if the Multiplier Method predicts an adult
omy of the body of the talus. Type 4 (clubfoot type) is treated height for a boy to be 5′7″ (170 cm) with an LLD of 6 cm, then
initially by applying Ponseti casts and performing an Achilles most families would choose limb lengthening as opposed to epi-
tenotomy followed by a “Super-ankle procedure” at age physiodesis. However, if the same boy had a predicted height of
12–24 months. The casting treatment typically converts the 6′2″ (188 cm) with an LLD of 6 cm, then epiphysiodesis would
foot position from equinovarus to equinovalgus, which can become a more attractive option. For the reconstruction option,
then be addressed with the Super-ankle procedure. While the the family needs to be told if it will be necessary to perform foot/
more severe Type 3 cases should also be considered candi- ankle reconstruction and how many lengthening procedures are
dates for amputation, Paley’s classification is geared towards anticipated. External fixation is the mainstay of lengthening in
reconstruction, with the subtype designation dictating the young children with FH [21]. In skeletally mature patients, it is
specific reconstructive procedure. possible to use the newer magnetic telescopic intramedullary
(IM) nails for tibial lengthening [25, 26]. Combinations of
lengthening and epiphysiodesis can also be done to minimize
the number of lengthening procedures required.
Box 23.3. Paley Classification of FH The initial clinical exam should include a complete ortho-
• Type 1: Normal ankle pedic examination of the child to look for associated anoma-
• Type 2: Dynamic valgus ankle lies. In the lower extremity, the number of rays and position
• Type 3a: Fixed equinovalgus ankle, ankle type of the foot and ankle are noted. Specifically, the ankle is
• Type 3b: Fixed equinovalgus ankle, subtalar type examined to determine if it is mobile and well aligned or
• Type 3c: Fixed equinovalgus ankle, combined ankle/ contracted into equinovalgus or equinovarus. The range of
subtalar type motion and stability of the hip, knee, and ankle are assessed.
• Type 3d: Fixed equinovalgus ankle, talar body type The knee joint is often somewhat unstable (ACL or PCL
• Type 4: Equinovarus type (clubfoot) deficiency) and may be in excessive valgus alignment.
If the involvement of the hip and knee is severe (as in
combined FH and congenital femoral deficiency), then the
overall reconstruction plan is either concurrent hip/knee
reconstruction or sequential reconstruction. Most commonly,
Clinical Assessment of a Child with FH the significantly involved hip and knee would be addressed
first between the ages of 18 and 24 months (this is discussed
In our practice, a patient with fibular hemimelia is usually at length in Chap. 22). The ankle reconstruction would then
seen during the first year of life. In less than one-third of typically occur 6–12 months after the hip and knee recon-
cases, the diagnosis has already been made using prenatal struction, although in some cases, it may be feasible to per-
ultrasonography [22]. During the initial visit, full-length form simultaneous hip, knee, and ankle reconstruction
standing anteroposterior (AP) and lateral view radiographs (Super-hip, Super-knee, and Super-ankle procedures).
are obtained to evaluate the exact configuration of the skel- Even if the femur is not involved or minimally involved,
etal anatomy of the lower legs, to determine the amount of there still may be instability of the knee due to an absent
LLD, and to look for concurrent deformities such as con- ACL. In such cases, the preoperative full-length standing lateral
genital femoral deficiency. The child’s height predication view radiograph shows anterior subluxation of the tibia on the
based on the long limb should be recorded. Based on the femur (Fig. 23.8). Consideration should be given to perform an
radiographic LLD (or the clinically measured LLD), the ulti- intra-articular ACL substitution procedure prior to tibial length-
mate LLD at maturity can be predicted. We use the Multiplier ening. If the PCL is also deficient, then an extra-articular PCL
Method [23, 24] to predict the ultimate LLD and ultimate reconstruction should be performed at the same time.
overall height (based on the long leg) at skeletal maturity.
This is easily done with the Multiplier mobile app (available
for free on the Apple iTunes store for the Apple iOS platform Radiographic Assessment of a Child with FH
and Google Play for the Android platform).
These two predictions (LLD and height) allow for formula- The initial radiographic assessment should include plain
tion of a multistage treatment plan for the family. The family films: full-length standing AP view radiograph depicting
should leave the first visit with a clear picture of the pros and both legs with a lift under the short leg (Fig. 23.7) and a full-
cons of the two main treatment arms: amputation versus recon- length standing lateral view of the affected leg with the knee
struction. Knowing the predicted adult height (based on the cur- in maximum extension (Fig. 23.8). The typical appearance in
rent length of the long leg) and the predicted LLD at skeletal severe cases is a mild valgus deformity of the knee secondary
Fig. 23.8 Full-length standing lateral view radiograph shows a typical

Fig. 23.7 Full-length standing AP view radiograph shows a typical child with FH who has an apex anterior bow of the tibia and congenital
child with FH who has knee valgus and tibial valgus bow. Reprinted instability of the knee due to the lack of functional intra-articular liga-
with permission from the Rubin Institute for Advanced Orthopedics, ments (ACL/PCL). Reprinted with permission from the Rubin Institute
Sinai Hospital of Baltimore for Advanced Orthopedics, Sinai Hospital of Baltimore
to a hypoplastic lateral femoral condyle. This creates a patients with FH have a talo-calcaneal coalition, which may
mechanical lateral distal femoral angle (mLDFA) of less than not be evident on the initial radiographs obtained during
85°. Additional valgus may come from an apex anteromedial infancy (Figs. 23.13 and 23.14) but become more obvious as
bow in the tibia. A dimple on the skin is usually visible over the coalition ossifies with age.
the apex of this bow (Fig. 23.9). A lateral view radiograph of In addition to plain films for cases of Paley Type 3 FH, it
the foot may show overlap of the talus and calcaneus or a can be useful to obtain a magnetic resonance imaging (MRI)
talo-calcaneal tarsal coalition. If the talus and calcaneus are evaluation of the foot and ankle just prior to performing the
on top of one another, the ankle valgus is typically from a tilt Super-ankle procedure. Since much of the hindfoot and dis-
in the distal tibial plafond. The talus and calcaneus may be tal tibia is unossified during the first 2 years of life, it can be
stacked on top of each other on the lateral view, which points difficult to accurately classify the exact Paley Type 3 sub-
toward the ankle as the source of valgus (Fig. 23.10). If the type. In such cases, an MRI can help differentiate between
talus and calcaneus are overlapped on the lateral view Type 3a, b, and c, thus allowing better preoperative planning
(Fig. 23.11) or side by side on the AP ankle (Fig. 23.12) view (Figs. 23.15 and 23.16). Also, during surgery an arthrogram
giving the appearance of a “double barrel shot gun,” then the of the ankle joint can help delineate the valgus orientation at
valgus component is more likely subtalar in origin. Most the ankle joint (Fig. 23.17).
Fig. 23.9 (a, b) Clinical photos

show a child with moderate
deformity secondary to FH. The
typical anterolateral bow,
equinovalgus foot, and dimple at
the apex of the tibial bow are
shown. (c, d) Clinical photos
show a child with severe
deformity secondary to
FH. Reprinted with permission
from the Rubin Institute for
Advanced Orthopedics, Sinai
Hospital of Baltimore
Fig. 23.10 Lateral view radiograph of the foot shows the talus and Fig. 23.11 Lateral view radiograph of the foot shows the talus and
calcaneus stacked on top of each other, suggesting the hindfoot valgus calcaneus overlapped on top of each other, suggesting the hindfoot val-
is supramalleolar in origin. Reprinted with permission from the Rubin gus is subtalar in origin. Reprinted with permission from the Rubin
Institute for Advanced Orthopedics, Sinai Hospital of Baltimore Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Fig. 23.12 AP view ankle radiograph shows the talus and calcaneus Fig. 23.14 Lateral view radiograph (same child depicted in Figure 23.13)
side by side, suggesting the hindfoot valgus is subtalar in origin. shows the classic “C” sign of the talocalcaneal coalition and a concurrent
Reprinted with permission from the Rubin Institute for Advanced talonavicular coalition. Reprinted with permission from the Rubin
Orthopedics, Sinai Hospital of Baltimore Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Principles of Treatment
One must understand that the initial reconstruction is a very

complex set of procedures that is unique for each child. The
deformity related to fibular hemimelia is the combination of
contracted soft tissues and abnormally formed joints of the
ankle and foot. The treatment of FH should address several
issues such as limb shortening, lower leg deformity, and an
abnormally positioned foot with or without absence of lat-
eral rays. The goals of treatment are to create a lower limb
with a stable hip, knee, and ankle by:
1. Correcting the foot (usually equinovalgus) into a planti-

grade position.
2. Equalizing lower limb lengths by skeletal maturity.
3. Correcting lower limb malalignment.
In many of the more severe cases, it may not be possible

to improve the limited ankle mobility. Furthermore, the treat-
ment itself (multiple lengthening procedures or Super-ankle
reconstruction plus multiple lengthening procedures) may
result in further loss of ankle motion compared to the amount
present initially. The success of reconstruction is not neces-
sarily determined by the final ankle motion. The amount of
Fig. 23.13 AP view radiograph of the tibia, ankle, and foot in a typical final ankle motion is usually predetermined by the amount of
child with FH shows a ball-and-socket ankle with a talocalcaneal coali- motion initially present at the ankle joint.
tion. As an infant, the talocalcaneal block is cartilaginous, but the talo-
The amount of LLD predicted by the end of growth does
calcaneal coalition becomes more radiographically evident as the child
ages and the bones ossify. Reprinted with permission from the Rubin not necessarily determine whether a successful reconstruc-
Institute for Advanced Orthopedics, Sinai Hospital of Baltimore tion is possible. There are potentially no set limits to the
Fig. 23.15 (a–c) Sequential

MRI scans of the left lower
extremity in a patient with fibular
hemimelia show that the valgus
originates in the supramalleolar
region (Paley Type 3A—ankle
type). Reprinted with permission
cm lengthening treatments. Better yet, two 5-cm lengthening

treatments combined with one 5-cm epiphysiodesis may
yield the optimum outcome.
For children with Paley Type 3 (fixed equinovalgus defor-
mity), the decision to lengthen during the first reconstructive
surgery is determined by the amount of ankle motion present.
If the ankle is intrinsically stiff, then a 4-to-5-cm lengthening
is performed at the same surgical setting as the Super-ankle
procedure. If the ankle is relatively mobile, then another
option is for the initial surgery to concentrate on positioning
the foot and ankle in a stable or corrected position while con-
currently correcting the bowing in the lower leg with a fixator
for a minimal amount of lengthening (just enough to provide
correction of the apex anteromedial bow). This strategy may
help preserve ankle mobility because the wires used for the
Super-ankle procedure cross the ankle joint for approxi-
mately only 3 months for this limited lengthening instead of
6 months for a more typical 4-to-5-cm lengthening.
The number of subsequent lengthening treatments is deter-
mined by the overall predicted lengthening goal. The subse-
Fig. 23.16 MRI scan of the right lower extremity in a patient with quent lengthening treatments are performed at intervals of 4–6
fibular hemimelia shows that the hindfoot valgus originates in the sub- years apart for a total of up to three or even four lengthening
talar joint (Paley Type 3B). Reprinted with permission from the Rubin treatments for the most severe grades of FH. The first length-
Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
ening usually achieves a 4 to 5 cm gain in length. Subsequent
lengthenings can achieve between 5 and 7 cm of length each.
amount of overall lengthening that can be performed, espe- The amount of length gained during a single lengthening
cially when divided into small steps of approximately 5 cm treatment is determined by the rate and quality of regenerate
for each treatment. However, the overall predicted LLD does bone formation and by motion and stability of the joints
help when estimating the number of lengthening treatments above and below the lengthening site. Bone formation is bet-
that will be needed to equalize the limb lengths before skel- ter in the proximal metaphysis rather than in the diaphysis.
etal maturity. Dividing the amount of lengthening into This is another reason why it may be preferable not to per-
smaller amounts might be less traumatic for the muscles, form a large amount of lengthening at the time of the initial
nerves, and adjacent joints than trying to perform a heroic Super-ankle procedure. Development of a knee flexion con-
lengthening during one treatment. For example, if the pre- tracture during lengthening may dictate the need to stop fur-
dicted discrepancy is 15 cm, this may be better addressed ther lengthening. The amount of lengthening will also be
with three 5-cm lengthening treatments instead of two 7.5- limited if the patient has poor regenerate bone quality or
Fig. 23.17 (a) Supine full-

length radiograph of an
18-month-old girl with left
fibular hemimelia shows typical
equinovalgus of the foot and
ankle. (b) Intraoperative AP
ankle arthrogram of the same
patient shows the distal tibia as
the source of the ankle valgus.
Baltimore
stiffness of the joints with loss of motion. In patients younger All children (and adults) with FH have a certain degree of
than 6 years of age, the maximum length typically gained tightness of the Achilles tendon, which will be made worse
during a single lengthening is 5 cm. The total amount of with limb lengthening. Therefore, we recommend perform-
lengthening in a younger patient is limited to avoid exces- ing a triceps surae lengthening procedure at the time of tibial
sive pressure on the growth plates that can cause premature lengthening. We recommend an open Vulpius procedure
closure of the growth plates and loss of potential natural (Fig. 23.18) and a prophylactic anterior compartment fasci-
growth. Older patients can often tolerate gaining more length otomy (Fig. 23.19). The fasciotomy decreases the risk of
in a single treatment (between 5 and 7 cm). compartment syndrome (a known complication of tibial oste-
otomy). An additional benefit of anterior compartment fasci-
otomy (especially if the longitudinal incision in the fascia is
Box 23.4. FH Treatment Options
combined with a short cruciate incision [transverse]) is that it
• Amputation
allows the anterior compartment musculature to bulge out,
• Lengthening with external fixator
thus creating the impression of a larger diameter calf. This is
• Lengthening over nails
desirable, as all patients with FH have a smaller ipsilateral
• Lengthening with internal telescopic nails
calf due to combined bone and soft tissue hypoplasia.
• Foot/ankle reconstruction for dynamic valgus
The fixator should be extended to include the foot to pre-
• Super-ankle for rigid equinovalgus
vent equinus deformation of the ankle during lengthening.
Once the lengthening is complete, the foot frame may be
removed provided there is no flexion contracture of the knee.
Surgical Techniques for FH We favor circular external fixators over monolateral external
fixators for their superior ability to correct deviations that
Lengthening for Paley Type 1 may occur during lengthening (typically procurvatum and
valgus) and for the ability to insert fixation pins and wires
Mild cases of FH may be lengthened using standard limb from multiple directions, creating a more stable construct.
lengthening methodology, which is described in Chap. 7 on Circular frames are generally well tolerated in the tibia and
limb lengthening by Dr. Feldman and coauthors. are easy to bridge across the ankle to the foot. Figure 23.20
Fig. 23.18 Open Vulpius procedure is recommended for all Paley horizontally to the opposite edge (medially) of the tendon using deep
Type 1 and 2 FH lengthening procedures. (a) Posterior leg can be retraction. (e) The median raphe (central tendon) of the soleus muscle
divided into five anatomic levels. (b) Cross-sectional view of anatomic is located and released. GSR gastrocnemius–soleus recession, GT gas-
level 2 with steps to perform gastrocnemius–soleus recession numbered trocnemius tenotomy, m muscle, n nerve, TAL tendo Achilles lengthen-
sequentially. The broken lines represent the tendon portion of the gas- ing, v vein. Figure and legend reprinted with permission from the
trocnemius and soleus muscles and the median raphe of the soleus American Podiatric Medical Association. Lamm BM, Paley D,
muscle. (c) After midline vertical incision, vertical division of the Herzenberg JE. Gastrocnemius soleus recession: a simpler, more lim-
superficial fascia, and horizontal release of the gastrocnemius tendon, ited approach. J Am Podiatr Med Assoc 2005; 95:18–25
then the soleus tendon is released. (d) Soleus tendon is also released
Incision
Proximal
extension
of fasciotomy
Ant.
Lat.
a
Skin
incision
d
Fasciotome
advanced
Distal proximally
b extension
Small incision of fasciotomy
created in fascia and
distally
under guidance
of touch
c
Longer end of
fasciotome inserted
under fascia
Fig. 23.19 Prophylactic anterior compartment fasciotomy is recom- Fasciotome is then inserted under the fascia. (d) Fasciotome is advanced
mended for all FH lengthenings. This decreases the risk of post- proximally, and care is taken not to invade the muscle. Note that the
osteotomy compartment syndrome and also causes a bulge of the opposite index finger follows the leading edge of the fasciotome. Ant.
anterior compartment musculature, increasing the circumference of the anterior compartment, Lat. lateral compartment. Figure and legend
calf (a positive effect for a hypotrophic calf). (a) Small midline anterior reprinted with permission from the Rubin Institute for Advanced
longitudinal incision is made just lateral to the tibial crest. (b) Fascia is Orthopedics, Sinai Hospital of Baltimore
exposed using blunt dissection, and a small incision is created. (c)
shows a standard FH lengthening with external fixation. In locked proximally. The osteotomy must be proximal enough
pure external fixation cases, it is typical to use three pins/ (and the nail long enough) so that at the end of lengthening,
wires proximally, use two pins/wires in the distal segment, there is at least 5 cm of nail distal to the osteotomy (for sta-
and include foot fixation. A wide space should be left between bility). An important technical point is to drill holes in the
the proximal and distal rings to allow for better recruitment anterior aspect of the tibia prior to reaming to vent the canal
of soft tissue into the lengthening. and to encourage the reamings to exit anteriorly to stimulate
For skeletally mature patients in whom there is no longer new bone formation at the lengthening site. The external fix-
a viable proximal tibial growth plate, it is attractive to use IM ator that is applied for the LON technique can be simple,
nails, either in the form of Lengthening over Nail (LON) or with two points of fixation at either end. The rate of length-
in the form of implantable IM telescopic nails. The prototype ening is adjusted according to bone formation. At the end of
nail, and the only one FDA approved and marketed in the lengthening, the patient returns to the operating room for dis-
USA currently, is the PRECICE system (Ellipse Technologies, tal locking and frame removal (in that order, to prevent short-
Irvine, CA). This telescopic nail is magnetically powered, ening). The bulk of the consolidation then takes place under
accurately controllable, and can go both in forward and in the protection of the nail, without need for an external fix-
reverse (in case of over lengthening or in case of poor bone ator. In order to control the foot during IM lengthening or
formation). With the LON technique, a standard trauma nail LON, a temporary extra-articular ankle arthrodesis screw
(8.5- to 10-mm diameter) is inserted and over reamed by may be inserted from the back of the calcaneus into the distal
2 mm to allow the bone to slide over the nail. The nail is tibia [26]. This screw is removed at the end of lengthening to
Fig. 23.20 Full-length standing AP view radiographs of a 12-year-old achieved (c), and after full correction of distal femoral valgus with a
girl with FH before treatment (a), after distal femoral hemiepiphysiode- normalized mechanical axis (d). Reprinted with permission from the
sis and application of circular external fixation for lengthening (b), after Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
removal of external fixation once lengthening and consolidation were
allow mobilization of the ankle. Figure 23.21 shows an FH Lengthening Plus Ankle Realignment
lengthening over nail to decrease duration of treatment time for Paley Type 2
with the external fixator (also discussed in Chap. 8).
The technique for self-lengthening telescopic nails is sim- In this subset of patients, the recommendations for lengthen-
ilar to that described above for LON, except that the nail is ing are largely identical to those described for Type 1.
locked proximally and distally and no external fixator is However, in these cases, the ankle has a flexible valgus
needed. Blocking screws (Poller screws) can be used to pre- deformity. This will worsen with limb lengthening as the
vent mechanical axis deviations during lengthening. peroneal tendons tighten. For such cases, we recommend
Figure 23.22 shows an FH lengthening with a PRECICE intramuscular lengthening of the peroneal tendons and bony
intramedullary lengthening nail, including the extra-articular realignment of the ankle (Fig. 23.23).
screw technique to stabilize the ankle (IM lengthening nails
are also discussed in Chap. 9).
Lengthening Plus Super-Ankle Reconstruction
for Paley Type 3
Box 23.5. Lengthening for Moderate FH For the rigid equinovalgus type of FH, we use a modified
• Lengthen Achilles/Vulpius Paley Super-ankle approach. This surgery can be performed
• Anterior compartment fasciotomy when a child is as young as 1 or 2 years of age. The goal is to
• Transfix foot during lengthening realign the foot relative to the distal tibia and to make it plan-
• Options: External fixation, LON, telescopic nails tigrade. It is an extra-articular reconstruction, as the ankle
joint and its ligaments are not opened.
Fig. 23.21 (a) Preoperative full-length standing AP view radiograph lateral view tibial radiograph (g) after locking of the intramedullary nail
shows a 16-year-old girl with left congenital femoral deficiency and and removal of the external fixation device for the consolidation phase.
fibular hemimelia prior to tibial lengthening with lengthening over nail (h, i) Final full-length standing AP (h) and lateral (i) view radiographs
(LON) technique. (b, c) One week postoperative AP (b) and lateral (c) show complete healing of the tibial lengthening segment. After three of
view radiographs of the left tibia show the LON technique using an the four cortices had healed, the distal locking screws were removed for
intramedullary tibial nail and concurrent classic Ilizarov circular exter- dynamization to improve the anterior cortical thickness. Reprinted with
nal fixation device. (d, e) AP (d) and lateral (e) view radiographs of the permission from the Rubin Institute for Advanced Orthopedics, Sinai
left tibia after completion of the distraction phase and prior to external Hospital of Baltimore
fixation removal. (f, g) Full-length standing AP view radiograph (f) and
Surgical Technique for Type 3a (Figs. 23.24 the anteromedial bow. This resected intermuscular septum
and 23.25) should be placed in a sterile specimen cup and covered with
saline, as it may be recycled for repair and elongation of the
Positioning Achilles tendon as needed. (Paley’s original technique
Supine position, bump under the ipsilateral sacrum, and tour- described resection of the anlage up to the proximal tibia and
niquet control. decompression of the common peroneal nerve through a
separate, proximal incision. We feel that this step is
Surgical Approach unnecessary.)
Longitudinal (straight) incision starts laterally at the mid- Next, the sural nerve is identified and protected. The
calf and ends just above the sole of the foot. Peroneal ten- Achilles tendon and posterior tibial neurovascular bundle are
dons (sometimes only one is present) are cut with a identified through this posterolateral approach. Be careful
Z-technique and later repaired in the lengthened configura- not to damage the posterior tibial nerve, which is juxtaposed
tion. Distal anlage of the fibula, if present, may be left in to the Achilles tendon. The flexor hallucis longus is identi-
place. Anterior compartment fasciotomy is performed, and fied as a guide to the anatomy of the posterolateral corner.
the superficial peroneal nerve is protected. The anterior com- The posterior tibial nerve is decompressed as far as can be
partment muscles are dissected off of the cartilage analage of seen into the tarsal tunnel distally through this posterolateral
the fibula and off of the intermuscular septum. approach. The Achilles tendon is very short and can be made
The intermuscular septum is resected from the ankle longer for purposes of Z-lengthening by scraping the distal
proximally up to the mid portion of the tibia at the apex of muscle belly proximally to expose more of the tendon.
Fig. 23.22 Case example of a 14-year-old girl with fibular hemimelia applied magnetic field. The PRECICE is also reversible and controllable
and congenital femoral deficiency who underwent lengthening with the to a very accurate degree. (e, f) Full-length standing AP (e) and lateral (f)
PRECICE. (a, b) Preoperative full-length standing AP (a) and lateral (b) view radiographs obtained at the completion of lengthening. (g, h) Final
view radiographs show that the child had an 8-cm limb length discrep- full-length standing AP (g) and lateral (h) view radiographs obtained
ancy and concurrent genu valgus. (c, d) Full-length standing AP (c) and after nail removal. Note the equal limb lengths and the correction of the
lateral (d) view radiographs depict lengthening with the PRECICE mag- mechanical axis. Reprinted with permission from the Rubin Institute for
netic intramedullary telescopic nail. This device contains a small mag- Advanced Orthopedics, Sinai Hospital of Baltimore
net, which will gradually lengthen when activated by an externally
Fig. 23.23 Illustration shows

bony realignment of the valgus
ankle for cases of Paley Type 2.
Orthopedics, Sinai Hospital of Supra-
Baltimore malleolar
dome Valgus
osteotomy deformity
planned corrected
30°
Despite this, it is often necessary to fashion a tendon graft cases, the surgeon may elect to perform a closing wedge
(from the resected intermuscular septum) to obtain sufficient osteotomy and remove the entire segment between the two
elongation of the Achilles tendon. The foot is then placed osteotomies, thus performing a closing trapezoidal wedge
into the maximum equinus and valgus position, and two 1.8- osteotomy. This is only done when the diaphyseal deformity
mm diameter Kirschner wires are inserted from the plantar is in very close proximity to the ankle.
surface of the heel, across the calcaneus and talus, and across
the ankle joint into the distal tibia about 5–10 mm. This pins Frame Application
the ankle in the maximum valgus/equinus position. At this Six months after the initial procedure, the tibia can be
stage of the operation, these pins do not cross the intended lengthened with an Ilizarov external fixator through an oste-
site of the supramalleolar osteotomy. otomy made at the apex of the anteromedial bow.
Alternatively, the Ilizarov frame may be applied immedi-
Osteotomy ately and used to correct the apex anteromedial bow with
Next, a subperiosteal dissection is performed of the distal minimal lengthening (1–2 cm). This frame is removed after
tibia, being careful not to damage the perichondrial ring of healing of the diaphyseal corticotomy is complete, and a
the growth plate. Mini-Hohmann retractors are placed ante- long leg cast is applied for 3–4 weeks followed by mobiliza-
riorly and posteriorly. An oscillating saw is used to make an tion and rehabilitation of the ankle to try to regain as much
oblique cut from posterolateral to anteromedial, but the motion as possible.
anteromedial cortex intact is left intact. The osteotomy is
levered open from the posterolateral direction with an osteo- Surgical Technique for Type 3b (Figs. 23.26
tome or laminar spreader. This maneuver corrects the and 23.27)
equinovalgus position of the hindfoot. The osteotomy is held
open with a cortical allograft (small piece of fibular allograft Positioning and Surgical Approach
works well) and the two Kirschner wires are passed across The positioning and approach are identical to what is
the osteotomy and up into the distal half of the tibia, stopping described previously for Type 3a.
just short of the anteromedial bow in the apex of the diaphy-
sis. The Kirschner wires are bent outside the skin of the heel Osteotomy
and can be incorporated into the frame (to be applied shortly) However, instead of a supramalleolar osteotomy, an oblique,
or simply bent and left outside the skin for removal when the U-shaped osteotomy of the subtalar region is performed. The
frame is removed. The foot is now corrected and plantigrade cartilaginous anlage is discarded, divided, or dissected
relative to the distal half of the tibia. The tourniquet can be around. After the osteotomy is complete (need to be able to
let down for the closure. The Achilles and peroneal tendons fully mobilize the hindfoot), the Kirschner wires are initially
are repaired in their lengthened configuration, and the inci- inserted the same way with the foot in the maximally uncor-
sion is closed, over a small drain if necessary. At this point, rected position (equinus and valgus). Then the distal half of
if desired, the operation can conclude and a long leg cast can the calcaneus and foot is translated medially and angulated
be applied. The cast and pins are removed in 6 weeks, and to make the Kirschner wires now face directly upwards
then the patient can receive physical therapy for the ankle. If toward the talus. This reduces the valgus lateral translation
necessary, the tibial diaphyseal bow is corrected through a of the foot, which is dorsiflexed to create an open wedge
mid-diaphyseal osteotomy. In severely shortened, angulated posteriorly for bone graft placement to correct the equinus
a AP
Peroneal
longus
tendon c
divided Extent of
proximally membrane
and anlage
b resection
Peroneal
brevis
tendon
divided
distally
h
j
Tendons
sutured Achilles
tendon repair
augmented
with resected
membrane
PRN
i
K-wires
advanced
k
Fixator
applied
l
Mid-diaphyseal
opening wedge
osteotomy m
performed Gradual
alignment and
lengthening
completed
Fig. 23.24 (a–m) Super-ankle surgical technique used to treat supramalleolar type fibular hemimelia (Paley Type 3A—ankle type). Reprinted with
permission from the Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Fig. 23.25 Case example of an 18-month-old boy with Paley Type 3A and lengthening. (g, h) Immediate postoperative radiographs obtained
fibular hemimelia. (a, b) Preoperative AP (a) and lateral (b) view radio- after the completion of the Super-ankle procedure. (i, j) Postoperative
graphs depict the typical equinovalgus foot and ankle deformity along with radiographs obtained 2 weeks after Super-ankle procedure show correction
the anterior tibial bow. (c–e) Intraoperative fluoroscopic images show the of the midshaft tibial deformity along with limited bone lengthening. (k, l)
distal tibial osteotomy portion of the Super-ankle procedure (comprehen- AP (k) and lateral (l) view radiographs obtained at the time of external fixa-
sive reconstruction). (f) Intraoperative fluoroscopic image shows the appli- tion removal (3 months after Super-ankle procedure). Note the complete
cation of the pediatric Ilizarov external fixator with the hinges positioned at correction of the tibia and the ankle. Reprinted with permission from the
the apex of the deformity on the convex side to produce both straightening Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
c
a Extent of
Peroneal membrane
longus and anlage
tendon resection
divided
proximally
b
Peroneal
brevis
tendon
divided
distally
Site of mid-
diaphyseal
osteotomy
d e
Path of Acute translation
Achilles and angulation
tendon Site of subtalar osteotomy
Z-lengthening osteotomy performed
h
Tendons
sutured
f
Bone i
graft Achilles
inserted tendon
repair
augmentated
with resected
membrane PRN
g
K-wires inserted
j
Fixator
applied
k
Mid-diaphyseal l
opening wedge Gradual
osteotomy alignment and
performed lengthening
completed
Fig. 23.26 (a–l) Super-ankle surgical technique used to treat subtalar type fibular hemimelia (Paley Type 3B). Reprinted with permission from
the Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Fig. 23.27 Case example of a 6-year-old boy with left fibular hemime- with a laminar spreader instrument. (e) Fluoroscopic image of fibular
lia and residual hindfoot valgus secondary to a deformity in the subtalar allograft wedge position with the surgeon’s thumb maintaining medial
region (Paley Type 3B). The osteotomy is performed through the talo- translation of the calcaneal fragment and initial wire fixation. (f, g)
calcaneal coalition. (a, b) AP (a) and lateral (b) view fluoroscopic Final fluoroscopic views of the subtalar osteotomy with medial transla-
images show residual hindfoot valgus after a previous supramalleolar tion and varus angulation of the calcaneal fragment stabilized with fibu-
osteotomy at 2 years of age. (c) Fluoroscopic image shows a guidewire lar allograft wedges and percutaneous wire fixation. Reprinted with
inserted into the region of the subtalar coalition demonstrating the permission from the Rubin Institute for Advanced Orthopedics, Sinai
oblique osteotomy. (d) The subtalar osteotomy is completed with an Hospital of Baltimore
osteotome and translated medially with concurrent varus angulation
position of the calcaneus. The smooth wires are driven across Osteotomy
the osteotomy into the talus. In some cases, sufficient pur- Both a supramalleolar osteotomy and a hindfoot subtalar
chase is available in the talus, but for very young children, coalition osteotomy are performed, opened (supramalleolar),
the wires should be driven across the ankle joint into the dis- translated, angulated (subtalar), and held open with bone graft
tal tibia for better purchase. At this point, the tourniquet is let and transfixion Kirschner wires. Intraoperative arthrography
down, and the closure proceeds as described above, includ- of the ankle joint helps to determine the degree of ankle val-
ing external fixator application and corticotomy of the mid- gus and whether it needs to be corrected or if only a subtalar
diaphyseal deformity. osteotomy is sufficient. If necessary, the tibial diaphyseal bow
is corrected through a mid-diaphyseal osteotomy. In severely
Surgical Technique for Type 3c (Fig. 23.28) shortened, angulated cases, the surgeon may elect to perform
a closing wedge osteotomy and remove the entire segment
Positioning and Surgical Approach between the two osteotomies, thus performing a closing trap-
The positioning and approach are identical to what is ezoidal wedge osteotomy. This is only done when the diaphy-
described previously for Type 3a. seal deformity is in very close proximity to the ankle.
i.
h. Supramalleolar
Acute bone graft
supramalleolar inserted
opening wedge
osteotomy
performed
j.
K-wires advanced
f.
e. Subtalar
Acute subtalar bone graft
osteotomy inserted
completed
along with
translation
and angulation
g.
K-wires inserted
Fig. 23.28 Super-ankle surgical technique used to treat combined ankle/subtalar type fibular hemimelia (Paley Type 3C). Reprinted with permis-
sion from the Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Surgical Technique for Type 3d (Fig. 23.29)

Box 23.6. Super-Ankle Procedure for Paley
Types 3a, b, and c Diagnosing Type 3d
• Lengthen peroneal tendons This subtype is extremely unusual and can be recognized in
• Lengthen Achilles younger children through MRI evaluation or in older chil-
• Resect fibular anlage and intermuscular septum dren through computed tomography. The hallmark of this
• Osteotomy: supramalleolar or subtalar or both deformity is a wedge-shaped talar body.
• Transfixion wires from sole of foot into tibia
• External fixator to correct diaphyseal anteromedial Positioning and Surgical Approach
bow The positioning and approach are identical to what is
described previously for Type 3a.
Fig. 23.29 Surgical technique

used to treat talar body type
fibular hemimelia (Paley Type
3D). Reprinted with permission
Opening Bone
wedge graft
osteotomy
Wire
Osteotomy be attributed to hypoplasia of the lateral femoral condyle and/

The opening wedge procedure is similar to the technique or to the tethering effect of the residual scar-like fibular band
described for the Type 3b (subtalar type), except the osteot- called the fibular anlage, if present. After lengthening, the
omy does not pass completely through the medial side. Cozen phenomenon may also account for some of the rebound
valgus that can be seen post-tibial lengthening (Fig. 23.31).
Surgical Technique for Type 4 We have observed cases of relapse of ankle valgus several
Initial Nonsurgical Treatment years after the Super-ankle procedure. After the initial cor-
This subtype represents less than approximately 15 % of FH rection of the foot and ankle with the Super-ankle procedure,
cases; therefore, treatment must be individualized. In the the valgus deformity can recur with the appearance that the
author’s experience, the initial treatment is nonsurgical, fol- child is walking on the medial malleolus. This recurrence
lowing Ponseti principles. It is unclear why the Ponseti can be addressed with a revision surgery that includes acute
method is effective in correcting the varus deformity, as the osteotomy of the distal tibia and/or the hindfoot bones.
Ponseti technique works through the subtalar joint. In our Usually the leg is placed in a cast for 4–6 weeks for healing
experience, all of these children have a coalition of the subta- and then the child is allowed to wear regular shoes.
lar joint. After casting and tenotomy, the deformity is con-
verted to a more typical equinovalgus type, which can then be
corrected using the Super-ankle approaches described above. Post-surgical Care
However, we have seen many children treated elsewhere with
preliminary open posteromedial release for Type 4 FH. Lengthening and deformity correction of the tibia begins 7
days after the surgery at a rate of 0.25 mm three times daily.
Physical therapy is the most important aspect of the length-
Additional Procedures ening process. Without proper physical therapy, the length-
ening goals will not be achieved and major complications
During tibial lengthening, valgus deformity of the diaphysis, will occur. Therefore, physical therapy requirements are
if present, should be corrected. Distal femoral valgus can be very strict. The patient undergoing tibial lengthening must
corrected with screw-plate devices for growth modulation, attend physical therapy sessions 5 days per week while at the
which is discussed in greater detail in Chap. 4 (Fig. 23.30). same time performing an exercise and stretching program at
Once corrected, the screw-plate devices are removed, but home. Normally, the foot is included in the frame, so all
rebound valgus is commonly observed. After tibial lengthen- physical therapy efforts are directed towards the knee and
ing, the valgus position of the lower extremity can recur due the toes. The knee tends to develop a flexion contracture.
to abnormal growth at the physis of the distal femur and/or the The toes also tend to develop flexion contractures. Night
proximal tibia. This progressive valgus deformity in FH may splints for the toes can be fabricated by the occupational
Fig. 23.30 (a) Full-length

standing AP view radiograph of
the same child shown in
Fig. 23.25 at 3-year follow-up
with genu valgus secondary to
hypoplasia of the lateral femoral
condyle and supination deformity
of the forefoot. (b) Full-length
standing AP view radiograph of
the patient 10 months after distal
femoral hemiepiphysiodesis and
tibialis anterior tendon transfer to
the dorsum of the foot. Reprinted
with permission from the Rubin
Baltimore
therapists as a forefoot splint to support the toes. The physi- distraction rod to stretch out the knee flexion contracture.
cal therapy continues at this level of intensity for the dura- Please refer to Chap. 12 for more details regarding physical
tion of the distraction phase (2–3 months depending on the therapy for limb lengthening.
planned amount of lengthening). Younger patients can put as
much weight as they can tolerate on the operated leg. Older
and larger patients are kept at 50 % weight-bearing during Outcomes
the lengthening phase.
Normally, we include the foot in the tibial frame, so there The treatment of FH is still a challenge. The goal is to achieve
is initially no physical therapy for the ankle. Once the length- normal limb alignment and length with a functional planti-
ening is completed (provided the knee is not contracted), it is grade foot. This undertaking is often accompanied by
recommended that the foot wires be removed to allow reha- repeated surgical procedures and a high rate of complica-
bilitation of the ankle. During the lengthening with the foot tions such as pin tract infections, residual LLD, delayed
included in the frame, the therapist may direct all efforts to union, ankle and knee stiffness, refractures, knee sublux-
preventing knee flexion contracture. Nighttime splinting in ation, residual foot deformities, and even mild to severe
extension is also helpful. If despite best efforts, the patient depression.
develops a knee flexion contracture, then it is possible to McCarthy et al. [8] compared the final outcomes after
extend the tibial frame to the femur in the form of a cast reconstruction and amputation and found that children
brace and use that as leverage to stretch the knee contracture. after early amputation were more active, had less pain, and
This can be done by building a thigh cuff from fiberglass cast were more satisfied with the results of treatment. These
material, using waterproof cast padding, and incorporating a children also had fewer procedures with fewer complica-
ring into the thigh cuff, which is then linked to the proximal tions. The reconstruction group had a total of 21 complica-
tibial ring with two hinges at the knee joint and a posterior tions and each patient underwent a mean of 7 procedures.
Fig. 23.31 (a) Full-length standing AP view radiograph of a 6-year- from the proximal tibia is also evident, possibly from a Cozen-type phe-
old boy obtained before he underwent tibial/fibular lengthening and nomenon. (d) Full-length standing AP view radiograph obtained after
distal femoral hemiepiphysiodesis. (b) Full-length standing AP view distal femoral guided growth plate removal and hemiepiphysiodesis of
radiograph after 6 cm of lengthening. Note the mild knee flexion con- the proximal tibia. (e) Full-length standing AP view radiograph
tracture but normalized medial proximal tibial angle. (c) Full-length obtained 8 months after proximal tibial hemiepiphysiodesis shows con-
standing AP view radiograph obtained 6 months after external fixation tinued correction of the recurrent valgus. Reprinted with permission
removal. Note the correction of the distal femur and the normalized from the Rubin Institute for Advanced Orthopedics, Sinai Hospital of
lateral distal femoral angle. However, progressive recurrent genu valgus Baltimore
In this study, the children in the lengthening group were procedures. All patients developed minor complications
older and therefore treated later, making treatment more such as pin-tract infections. At last follow-up (average 15
difficult. Also 50 % of the children were treated with the years), all patients were ambulatory and mobile with accept-
Wagner method, which is associated with more complica- able limb lengths and limb alignment.
tions [27, 28]. Oberc and Sulko [13] published a study of 31 patients.
Before 2000, there are several papers with the same poor Only nine (29 %) were treated with the elongation method.
outcomes in which the authors [12, 27, 29] suggested that In these 9 patients, there were 12 elongation and 39 surgical
amputation is a more effective method of management than procedures in total. The complication rate was 78 %, and
limb lengthening in severe fibular hemimelia cases. During there were satisfactory results in five of nine patients.
the last decade, there was a tendency toward a reconstruction El-Sayed et al. [14] reported the results of 157 patients
and lengthening treatment even for more severe cases with with Achterman and Kalamchi type-II FH. All patients
more favorable results. underwent elongation, and 12 patients underwent concomi-
Catagni et al. [10, 30] treated 32 patients with type III FH tant femoral lengthening (average 13.6 cm). There were 107
according to the modified Dalmonte classification. In this minor complications, which were treated without change to
study, the mean number of surgical procedures was six and the final outcomes and 19 major complications in which the
the complication rate was 82 % but most of the complica- desired lengthening was not achieved.
tions resolved except for two patients who ultimately under- El-Tayeby and Ahmed [31] performed ankle reconstruc-
went amputation. The outcomes were satisfactory in 17 of 32 tion prior to elongation in 13 patients. All patients had a sta-
patients. ble ankle without valgus deformity or subluxation after a
Changulani et al. [9] reported the results of eight patients, mean follow-up of 18.6 months and showed good alignment
in which each patient underwent an average of three surgical in all cases.
Zarzycki et al. [32] treated ten patients with fibular hemi- 12. Naudie D, Hamdy RC, Fassier F, Morin B, Duhaime M. Management
of fibular hemimelia: amputation or limb lengthening. J Bone Joint
melia type II according to the Achterman and Kalamchi clas-
Surg Br. 1997;79(1):58–65.
sification. The mean lengthening was 23 % of the preoperative 13. Oberc A, Sułko J. Fibular hemimelia: diagnostic management,
length. Equal limb length and functional foot positioning principles, and results of treatment. J Pediatr Orthop B.
were achieved in four patients, and the complication rate was 2013;22(5):450–6.
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Başbozkurt et al. [33] reported his experience treating FH Achterman-Kalamchi type II patients. J Pediatr Orthop B.
in five patients. At last follow-up, three of five patients had 2010;19(1):55–60.
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17. Coventry MB, Johnson Jr EW. Congenital absence of the fibula.
outcome of this debate, there will always be families that J Bone Joint Surg Am. 1952;34A(4):941–55.
refuse amputation; therefore, reconstructive lengthening 18. Achterman C, Kalamchi A. Congenital deficiency of the fibula.
techniques should be available. For cases with more normal J Bone Joint Surg Br. 1979;61-B(2):133–7.
19. Stanitski DF, Stanitski CL. Fibular hemimelia: a new classification
function of the foot and ankle and with at least four rays, the
system. J Pediatr Orthop. 2003;23(1):30–4.
results of reconstruction should be fairly predictable and the 20. Birch JG, Lincoln TL, Mack PW. Functional classification of fibu-
need for amputation in these moderately involved patients lar deficiency. In: Herring JA, Birch JG, editors. The child with a
may be minimized. limb deficiency. American Academy of Orthopaedic Surgeons:
Rosemont, IL; 1998. p. 161–70.
21. Hamdy RC, Makhdom AM, Saran N, Birch J. Congenital fibular
deficiency. J Am Acad Orthop Surg. 2014;22(4):246–55.
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5. Birch JG, Lincoln TL, Mack PW, Birch CM. Congenital fibular ankle equinus. Clin Orthop Relat Res. 2008;466(12):3003–10.
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and a proposed classification system based on clinical deformity. for partial or total absence of the fibula. J Bone Joint Surg Am.
J Bone Joint Surg Am. 2011;93(12):1144–51. 1990;72(9):1391–9.
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Orthop. 2010;4(6):519–24. 32. Zarzycki D, Jasiewicz B, Kacki W, Koniarski A, Kasprzyk M,
10. Catagni MA, Radwan M, Lovisetti L, Guerreschi F, Elmoghazy Zarzycka M, Tesiorowski M. Limb lengthening in fibular hemime-
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317–9. 46–53. Turkish.
Tibial Hemimelia
24
Dror Paley and David Y. Chong
Tibial hemimelia may be diagnosed on prenatal ultrasound

Introduction as early as 16 weeks of gestation [9]. There have been many
studies on the genetic inheritance of tibial hemimelia.
The first case of tibial hemimelia was reported in 1841 by Multiple reports describe parent to child transmission (Nutt
Otto [1] in the German literature. However, he incorrectly [8], Jones [47]) as well as families with multiple siblings
described it as fibular hemimelia, and Burckhardt later rec- affected (Aitken [10], Emami [11]). Autosomal dominant
ognized his mistake in 1880 [2, 3]. Thus, the first correct heritance has been implicated by Clark [12], Cowell [13],
reported complete case of tibial hemimelia is credited to and Lenz [14, 15]. Autosomal recessive inheritance has also
Billroth in 1861 [4, 5]. been described (Fried [16], Mahjlondji [17], and McKay
Tibial hemimelia often appears as a shortened leg with [18]). An interesting breeding trial of Galloway cattle with
knee and ankle deformity. Most often there is a knee flexion tibial hemimelia concluded that tibial hemimelia was caused
contracture, though multidirectional instability may also be by homozygosity of a single autosomal recessive gene with
present due to a lack of collateral ligaments. A patella and variable expressivity and pleiotropic effects on various body
quadriceps mechanism may be present or absent and may systems [19].
have limited function if present. There may be cutaneous man- Tibial hemimelia has been found to be associated with
ifestations such as dimples over the head of the fibula or the several syndromes. Werner’s syndrome, or tibial hemimelia–
free end of the tibia. The tibia may be shortened, dysplastic, or polysyndactyly–triphalangeal thumb syndrome [20]
absent; it may also present as an anlage invisible to radio- (THPTTS) is an autosomal dominant disorder which is now
graphs. The fibula can be normal or dysplastic, and often sub- thought to be a variant of triphalangeal thumb-polysyndactyly
luxated or dislocated proximally and/or distally. The ankle is syndrome (TPTPS), which have both been mapped to chro-
often in varus and equinus, and the foot may be adducted and mosome 7q [21]. It has also been proposed that deletion on
supinated. The medial side of the foot may be missing rays. chromosome 8q, contiguous with Langer-Giedion syndrome,
or type II tricho–rhino–phalangeal syndrome (TRPS II) may
also be responsible for tibial hemimelia [22]. Case reports
Genetics have also linked tibial hemimelia and other limb anomalies
to CHARGE syndrome, a pattern of congenital anomalies
The incidence of tibial hemimelia is reported to be one in one including eye, nose, ear, heart, and genital defects [23, 24].
million [6, 7]. In 1941, there were 79 published cases [8]; Most cases of CHARGE syndrome are due to a mutation of
since then there have been several hundred more reported. the CHD7 gene (chromodomain helicase DNA-binding pro-
tein 7), located on chromosome 8q, and CHD7 is known to
be expressed by the developing limb bud mesenchyme [25,
D. Paley, MD, FRCSC (*)
St. Mary’s Medical Center, Paley Advanced Limb Lengthening 26]. In addition, tibial hemimelia has been linked to tibial
Institute, 901 45th St. , Kimmel Building, West Palm Beach, hemimelia-diplopodia syndrome [27], tibial hemimelia–split
FL 33407, USA hand and foot syndrome [28], and tibial hemimelia–micro-
melia–trigonal brachycephaly syndrome [29].
D.Y. Chong, MD Tibial hemimelia can be unilateral or bilateral. It is esti-
Department of Orthopedic Surgery, University of Oklahoma Health
mated that 30 % of cases are bilateral [30]. It does seem to
Sciences Center, 940 NE 13th St, Suite 2009/MRI 2000, Oklahoma
City, OK 73104, USA favor the right side for unknown reasons. Spiegel noted that
e-mail: [email protected] all 11 of his unilateral cases affected the right side, and in his

456 D. Paley and D.Y. Chong
review, about 72 % of unilateral cases in the literature around and inserted on the posterior capsule of the ankle.
affected the right side [31]. Tibial hemimelia has also been Abductor hallucis muscles were found in all specimens, even
associated with multiple congenital anomalies. In in feet without medial rays. No discrete plantar fascia was
Schoenecker’s case series of 57 patients, 34 (60 %) had asso- found. All specimens had subtalar coalitions, and some had
ciated anomalies [32]. Similarly, Launois and Kuss found midfoot coalitions. The talus articulated with the distal
24/41 (59 %) cases had associated anomalies [33]. medial fibula on its posterolateral side with a vertical and
In the ipsilateral limb, additional deformities may be sup- sagittal orientation with only one plane of rotation.
pressive or duplicated. Reported anomalies include congeni-
tal femoral deficiency, bifid femur, missing patella or
quadriceps mechanism, knee hyperextension or flexion, Box 24.1. Introduction to Tibial Hemimelia
clubfeet, missing toes, syndactyly, supernumerary or dupli- • Incidence is approximately one in one million.
cated great toe, and a “mirror foot” [34–38]. Other reported • Autosomal dominant and recessive inheritance has
limb deformities include radial dysplasia, lobster claw defor- been reported, with variable expression.
mity, hand syndactyly, polydactyly, triphalangism, missing • Defects on chromosome 7q and 8q have been impli-
fingers or toes, hip dysplasia, hip dislocation, coxa valga, cated, as well as the CHD7 gene.
hemivertebrae, and myelomeningocele [39–42]. Associated • Common associated deformities: congenital femo-
deformities that have been reported include deafness, cleft ral deficiency, bifid femur, absent patella, mirrored
palate, pseudohermaphroditism, cryptorchidism, and hypo- foot, and polydactyly.
spadias [30]. • A dimple is often seen on the proximal fibula.
• Anterior and deep posterior compartments of the
leg are most deficient, with tendon remnants still
Pathoanatomy attached to the foot.
• The talus tends to follow the fibula in the deformity.
Evans and Smith [43], in an anatomic examination of the leg,
found both an absence and duplication of some muscles. He
described some muscles as being essentially functionless
and attached to only one bone. He postulated a mesoblast
origin and not likely a mechanical or traumatic source, con- Classification
firming what Hovelacque and Noel had found in mouse
embryos in 1909 [44]. Frantz and O’Rahilly described a classification system for
Turker et al. [45] dissected five Jones type Ia specimens. congenital skeletal limb deficiencies in 1961 [46]. The
They found ipsilateral toe anomalies in most cases, ranging authors described amelia, hemimelia, phocomelia, and
from four to eight digits. The affected leg had smaller girth, other limb deficiencies. Hemimelia was further described
and a dimple was consistently found where the skin was teth- as being complete, partial, or paraxial. They are then
ered over fibula. They found intact saphenous and lesser divided into terminal (distal) and intercalary (middle) defi-
saphenous veins, as well as sural and superficial peroneal ciencies, and further subdivided into transverse and longi-
nerves in the subcutaneous tissues. The deep peroneal and tudinal deficiencies.
posterior tibial arteries were found associated with their The Jones classification (Fig. 24.1) was proposed in 1978
respective nerves, but the posterior tibial bundle was found and was based on radiographic criteria. Type I deficiencies
to be short and acting as a tether. Lateral and superficial pos- had no visible tibia and were subdivided into two groups: the
terior compartment muscles were generally intact with nor- Ia group had a hypoplastic distal femoral epiphysis and the
mal insertions. Gastrocnemius and soleus muscles had Ib group had normal ossification of the distal femoral epiph-
confluent Achilles tendon, which inserted on the medial side ysis, suggesting the presence of a proximal tibial epiphysis.
of the calcaneal tubercle. The anterior and deep posterior Type II deficiencies had a visible proximal tibia but had a
compartments did not have a discrete boundary. Tendons had distal tibia deficiency. Type III deficiencies had a visible dis-
anomalous courses and sometimes split. No identifiable pos- tal tibia, but had a proximal tibia deficiency. Type IV defi-
terior tibial or anterior tibial muscle belly was found, but all ciencies had a shortened tibia with significant distal
specimens had a tendon inserting medially on the midfoot tibiofibular diastasis [47].
that tethered the foot in supination. Three specimens had an Kalamchi and Dawe, in their study of 21 cases in 1985,
anomalous tendon inserting onto the neck of the talus. In proposed a modification to the Jones classification and the
addition, all specimens had a flat cable of tendon-like struc- results of their treatments for each type [48]. Type I deficien-
tures on the anterior border of the fibula with minimal mus- cies were defined as a total absence of the tibia. This group had
cle associated proximally. This unknown structure wrapped knee flexion contractures, greater than 45° and no active quad-
24 Tibial Hemimelia 457
Fig. 24.1 Jones classification of tibial hemimelia
riceps function. These patients also had proximal migration of fibulae. Type 7 was complete agenesis with a single fibula.
the fibular head and hypoplasia of the distal femur. Type II He then assigned a score to determine the functional ability
deficiencies were defined as distal tibial aplasia, with a proxi- of the limb, where higher scores indicate less impairment of
mal tibia present. There was active quadriceps function and the limb. The tibia (0–22 points) and presence of an anlage
had knee flexion contractures between 25° and 45°. They had (+10 points) weighed heaviest in importance, and the patella
more normal development of the distal femur, with normal (0–3 points) was given slightly extra weight. The rest of the
metaphyseal width and normal epiphyseal ossification, indi- limb was scored from 0 to 2 points, and included the hip
cating at least a proximal tibial anlage was present. There was joint, distal femur, fibula, foot, and muscle function over the
also less proximal migration of the fibula. Type III deficiencies hip, knee, and ankle joints. Five classes were defined based
were defined as distal tibia aplasia with diastasis of the tibio- on the score, indicating the degree of deficiency and diffi-
fibular syndesmosis. They had normal knee joints and good culty of reconstruction.
quadriceps function. Usually the talus was subluxated proxi- The rarity of tibial hemimelia lends itself to other vari-
mally with a prominent distal fibula. ants, which still may not fit any classification scheme. One
Paley proposed a classification in 2003 based on the Jones reported case which does not fit any current classification is
classification [49]. The Paley classification (Fig. 24.2) is that of the intercalary deficiency, where the central portion of
modified in this manuscript and described in more detail the tibia is missing but the proximal and distal tibia is intact
below. The classification is oriented around the treatment for [52]. The authors in this case were able to transfer the fibula
each type. into the defect with success.
The most recent classification scheme was published by
Weber in 2007 [50]. Previous classifications had not taken
into account the cartilaginous anlage. He also added a few
types of tibial hemimelia which were previously unclassifi- Box 24.2. Jones Classification
able by the previous schemes and previously published by • Type I: No visible tibia
case report [51]. Weber’s new classification took into account – Ia: Hypoplastic distal femoral epiphysis
the entire affected leg, from hip to foot. He proposed seven – Ib: Normal distal femoral epiphysis ossification
types of increasing severity, with subgroups based on (possible cartilaginous proximal tibia)
whether the cartilage anlage was present (a) or not (b). Type • Type II: Distal tibia deficiency
1 was tibial hypoplasia but intact proximally and distally. • Type III: Proximal tibia deficiency
Type 2 was distal diastasis. Type 3 was distal aplasia. Type 4 • Type IV: Shortened tibia with distal tibia-fibula
was proximal aplasia. Type 5 was bifocal aplasia proximally diastasis
and distally. Type 6 was complete tibial agenesis with double
Fig. 24.2 Paley classification of tibial hemimelia
of the proximal fibula to the intercondylar notch, and side-to-

History of Treatment side synostosis of the fibula to the tibia both proximally and
distally. Sulamaa and Ryoeppy [41] performed side-to-side
Early Treatments opposition and recommended knee disarticulation for type I
deformity.
In 1877, Albert first published about the centralization of the In the current literature, the treatment of choice for tibial
fibula, fusing the femur and fibula [53]. In 1905, Myers [54] hemimelia has historically been biased towards amputation,
proposed a fibula-femoral arthroplasty, which was further though it is dependent upon the classification of the defor-
developed by Brown in 1965. In the case of a partial absence mity. In addition, in some communities, the option of
of the tibia, there are early published reports of attempted amputation is not accepted culturally, and patient families
synostosis between the tibial remnant and fibula, either side- wish to pursue limb salvage. With complete absence of the
to-side or end-to-end (Bade [55], Nove-Josserand [56]). tibia (Jones type Ia), most literature points towards amputa-
Fraser and Robarts in 1914 reported a case with an interca- tion [32, 42, 48, 67, 69], while less severe deformities have
lary defect in which they transplanted the contralateral fibula several case reports of salvage options [59–61]. The pres-
with reported success [57]. In 1929, Putti [58] was one of the ence of a tibia anlage and an active quadriceps mechanism
first to give directives on treatment, describing different are important factors for reconstruction, as well as preven-
approaches to eight different cases: fusing the fibula to the tion of knee flexion deformities [62]. Their absence may be
talus in extreme equinus to increase the leg length, transfer indicated by the absence of a patella, which can be difficult
to find in young children. Thus, current imaging options such patients developed progressive knee flexion deformities and
as MRI and ultrasound are a useful method of determining needed multiple secondary procedures, leading the authors
the presence of the patella or tibia anlage [63]. to recommend early disarticulation of the knee and pros-
thetic fitting as the treatment of choice. However, the results
were considered to be failures due to the knee flexion
Brown Procedure (Fibular Centralization) deformities.
Simmons et al. (1996) [70] revisited the Brown proce-
Brown first published his surgical procedure of fibular trans- dure, publishing his results on five patients and seven limbs.
fer and centralization under the femur in 1965 [64]. This pro- All cases had Jones type Ia deformities, though one case was
cedure was done for patients with complete absence of the found to be a 1b during surgery and thus the rudimentary
tibia (Jones type I). He also recommended a Syme-type proximal tibia was utilized for fixation. All patients had knee
amputation of the foot. In 1972, Brown published his 15-year flexion and varus deformities. They also had equinovarus
follow-up, in which 40 of 56 patients were available for deformities of the foot and underwent Syme amputations.
review [65]. Eighteen required secondary surgery due to a An anterolateral knee incision was used, and the biceps ten-
knee flexion deformity. Of the remaining 22, 1 was nonam- don and distal insertion of the patellar tendon released.
bulatory, and all but 2 were wearing braces while ambula- Femoral or fibular shortening was performed as needed
tory. He recommended attachment of the patellar ligament to along with peroneal nerve decompression. The fibula was
the fibula, preoperative traction, as well as femoral shorten- centralized underneath the femoral condyles and the knee
ing and soft tissue releases as needed to gain extension. He stabilized in extension with a Steinmann pin. The patellar
also recommended surgery before age 1 for maximal ambu- tendon was reattached to the fibula and along leg spica cast
latory and fibular articulation potential. Inferior results were was applied. Subsequent procedures included split thickness
noted with an absence of quadriceps function. skin grafting, and heel pad release for the Syme amputation,
Jayakumar and Eilert (1979) [66] reported six cases and one quadricepsplasty for lack of knee flexion. All ambu-
treated with the Brown procedure. All patients received lated with a PTB socket with thigh extension. Average range
either a Boyd or Syme amputation. Clinical outcomes were of knee motion was 57° and only two patients achieved
graded on several criteria of knee joint function, requiring 10–80° of knee flexion. However, all patients were satisfied
(1) at least 10–80° of active range of motion, (2) <5° varus/ and none went on to knee disarticulation. They recom-
valgus instability, and (3) no flexion contracture. Only two mended narrowing indications for reconstruction to those
patients achieved these criteria, and they had 3+ quadriceps patients with grade 3+/5 active quadriceps, younger than 1
power. One patient had ipsilateral congenital femoral defi- year of age, no fibular bowing, and ambulatory potential.
ciency and underwent a femoral-fibula arthrodesis. Three Wada et al. (2006) [71] published nine cases of limb
patients had “poor” results from knee flexion contractures reconstruction in Jones type I and II cases. They performed
and two went on to knee disarticulations. The authors con- four tibiofibular synostosis and five Brown procedures, with
cluded that strong active quadriceps function of at least 3+ concurrent foot centralization procedures and subsequent
was necessary for Brown reconstruction to have superior lengthening as needed. The tibia-fibular fusion patients had
results to an amputation. good results with preserved knee function. Of the Brown
Loder and Herring (1979) [67] reported nine cases of procedures, four had poor quadriceps function and one had
Jones type I deficiencies treated with the Brown procedure. persistent knee instability. All five had unsatisfactory func-
All had preoperative knee flexion contractures. Initially, five tional results based on Jayakumar’s criteria [66]; however,
of nine had good results with active range of motion and no they were all household ambulators with only one patient
flexion contractures. However, only one patient maintained needing an orthosis.
active quadriceps power, and all progressively developed Hosny (2005) [72] published a case series of 6 patients
knee flexion contractures. Despite secondary procedures, with tibial hemimelia. Amputation was not accepted in his
outcomes were also poor due to knee instability and poor community and they presented at an older age (3.5–13 yo).
range of motion. At final follow-up, five had knee disarticu- Jones type I cases were treated with gradual distraction of the
lations and the remaining cases had poor range of knee fibula with an external fixator, followed by a Brown proce-
motion (10–45°). dure 1 month later. Type II cases were treated with gradual
Epps and Schneider (1989) [68] published on three cases distraction followed by fixation of the proximal fibula to the
of Brown procedures, and noted that their results deterio- proximal tibia. Fibula lengthening was subsequently per-
rated over time due to progressive flexion contractures, and formed but had limitations due to knee flexion contractures
needed secondary procedures. In 1991, Epps et al. [69] that resulted. Femoral lengthening was then performed at a
reported on 14 patients (20 knees) who underwent central- later stage. Hosny reported all patient’s families were satis-
ization of the fibula for Jones type I tibial hemimelia. All fied with the outcome, with all the type II patients ambulating
independently with minimal (5°) to no knee flexion amputation so that the patient would treat it as a “congenital
contractures and active ROM greater than 90°. The type I amputation,” allowing for better adaptation to their prosthesis
patients could walk with a KAFO and walker and had limited and rehabilitation. For many of these patients, their socio-
ROM (10–35/40°) but could perform ADLs at home without economic status also required a quicker and more definitive
any pain. solution.
Overall, most authors did not report good outcomes with Spiegel et al. (2003) [31] described some potential com-
the Brown procedure and recommended knee disarticulation plications and sequelae of amputation in patients with tibial
rather than reconstruction as the best option for total absence hemimelia. They treated nine Jones type I deficiencies with
of the tibia. Many of the poor outcomes were due to progres- knee disarticulation without any subsequent complications.
sive knee flexion contractures, knee instability, and poor Five type II deficiencies were initially treated with distal
range of motion, as previously defined by Jayakumar [66]. amputation (Chopart or Syme). They developed prosthetic
However, for some patients in whom amputation is not an irritation from the proximal fibula from the varus malalign-
option, a limb that is weight bearing though less functional ment and prominent fibular head. Three patients subse-
may be considered a success. Certainly the presence of a quently underwent tibiofibular synostosis. One patient had
strong quadriceps, patella, and proximal tibia or anlage progressive varus deformity and difficulty with prosthetic
seems to favor reconstruction, and tibiofibular synostosis has fitting that improved after hemiepiphysiodesis. Overall, the
generally met with good results. In addition, the use of an authors felt unsure about the best course of action for type II
external fixator prior to reconstruction can help overcome deficiencies. Four type III deficiencies were treated with
soft tissue contractures. Syme or Chopart amputations and did well.
Amputation Tibiofibular Synostosis
Knee disarticulation has been described for treatment and In the presence of a tibial anlage (Jones type Ib) or a proxi-
remains a salvage option for failed Brown procedures, as mal tibia (Jones type II), most authors have reported good
seen in many of the studies above. Kalamachi (1985) [48] results with tibiofibular synostosis. Kalamachi [48] reported
treated three children with the Brown procedure, and all ten patients with Jones type II tibial hemimelia that were
went on to subsequent knee disarticulations. The failure was treated with tibia and fibula synostosis. Three of these uti-
attributed to knee flexion contractures and no active quadri- lized a modified Brown procedure, fusing the fibula with the
ceps function, leading the authors to recommend early dis- cartilaginous tibia anlage. They found good stability and
articulation of the knee without attempt of reconstruction. adequate range of motion with only mild (20–30°) flexion
Alternatively, if the femur was severely hypoplastic, a fem- contractures. Due to leg length discrepancies and foot defor-
orofibular arthrodesis was performed to effectively lengthen mities, half had concurrent Boyd amputations and did well
the femur, creating a longer lever arm for improved pros- with a prosthesis.
thetic fitting. Schoenecker [32] reported 8 of 15 Jones type II limbs
Similar results and conclusions were drawn by Schoenecker successfully treated with tibiofibular synostosis. Twelve
et al. (1989) [32]. Of a series of 57 patients and 71 limbs, 61 were treated with a Syme amputation and were functional
limbs eventually had some type of an amputation. Brown pro- below-knee amputees, one had a knee disarticulation, one
cedures were performed on 14 Jones type Ia limbs. Ten was nonoperative, and only one patient, who also had a tib-
required secondary procedures due to knee instability or flex- iofibular synostosis, retained the foot. They recommended
ion contractures. Based on their results, they recommended tibiofibular synostosis for Jones Ib and II with concurrent
evaluating for a proximal tibia anlage to differentiate Jones distal Syme or Boyd amputations.
types 1a and 1b, and that a type Ia should be treated by knee
disarticulation due to their less-than-satisfactory results with
the Brown procedure. Distal Tibia and Ankle Stabilization
Fernandez et al. (1998) [38] have also been a proponent of
early amputation. Twenty-two cases of tibial hemimelia For Jones type II deficiencies, distal tibia aplasia leads to an
were reported, of which 17 patients (and caretakers) accepted unstable ankle. These have historically been treated with sta-
surgery. All ten Jones type I cases underwent knee disarticu- bilization of the ankle in the form of arthrodesis and possible
lation. Three Jones type II and III cases had distal amputa- amputation. In Kalamachi’s [48] series, three cases had
tions. Only the remaining four Jones type IV cases had essentially normal knees, but the talus was subluxated proxi-
conservative treatment. The author recommended early mally with a varus foot and prominent distal fibula. These
were treated with calcaneo-fibular fusions and Boyd amputa- converted into a tibial plateau. The soft tissues were gradually
tions. Schoenecker [32] treated seven Jones type limbs with distracted with an external fixator to avoid the need to shorten
Syme or Chopart amputations to function as below-knee the femur or fibula. The patella is then brought down with
amputees. Tibiofibular synostosis was performed in two of crossing “visor” flaps from the capsule and chondrodesed to
these patients. a centralized fibula. A Z-plasty of the quadriceps tendon was
Jones type IV deficiencies have a shortened tibia with sig- used to gain length. A hinged external ring fixator was used
nificant distal tibia and fibula diastasis. Tokmakova et al. to stabilize while allowing knee range of motion and weight
(2003) [73] felt that the treatment of choice was reconstruc- bearing. A second procedure was done to achieve chon-
tion of the ankle mortise, as their patients were independent drodesis between the distal fibula and the talus. To our
ambulators with stable ankles and plantigrade feet. knowledge, there have been no publications of results of this
In Schoenecker’s series of ten Jones type IV limbs, one procedure, though Weber did further describe and refine his
limb had a Syme amputation and nine limbs had ankle joint procedure in 2006 [79].
reconstruction and salvage of the foot. Of these nine, five had
a Syme amputation within 3 years due to leg length discrep-
ancy. He recommended ankle reconstruction and leg length
equalization, similar to Fernandez [38]. Box 24.3. Treatment Options
• Outcomes for the Brown procedure (centralization
of the fibula) depend on the presence of a quadri-
Limb Lengthening ceps mechanism and a mobile knee without flexion
contractures.
Limb lengthening is also an option and adjunct for the treat- • Progressive knee flexion contractures and knee
ment of tibial hemimelias [74]. Hootnick et al. (1977) [75] instability are common reasons for failure of the
followed the natural history of tibial hemimelia and found Brown procedure.
that the leg length discrepancy remained proportional over • The presence of a proximal tibia or its anlage has
time. Thus, they were able to calculate a final predicted leg had good results from synostosis with the fibula.
length discrepancy to help a family decide on lengthening • For some parents, a quicker and more definitive
versus Syme amputation. They recommended amputation knee disarticulation may be a more attractive
for predicted discrepancies greater than 8.7 cm. However, option, allowing a child to adapt early to a
with newer technology and a better understanding of the prosthesis.
biology, staged reconstruction and limb lengthening may • Distal tibia deficiencies can be treated with distal
allow the trained surgeon to address more severe cases of fusion or synostosis or Syme-type amputation.
congenital tibial deficiency, preserving more lower limb • Advances in limb lengthening allows for limb
function than what has been reported previously. length equalization.
Desanctis et al. (1990) [76] published three cases of • The Weber patelloplasty converts an existing patella
Jones type II deficiencies treated with staged reconstruction. into a proximal tibia.
They corrected the foot deformity with serial casting and
posteromedial soft tissue release, followed by tibiofibular
diaphyseal reconstruction, alignment of axis of the foot and
leg, and limb lengthening using the Ilizarov technique. Javid New Horizons and Strategies
(2000) [77] reported on a lengthening after centralization of for Reconstruction in Tibial Hemimelia
the fibula in a Jones IIb deformity. They found that the fib-
ula had delayed new bone formation, and compression-dis- The deficiency in tibial hemimelia is a spectrum of pathol-
traction technique with an Ilizarov device had to be utilized ogy, ranging from a congenitally short tibia with relative
to promote osteogenesis. Devitt reported good results with fibular overgrowth to complete absence of the tibia. It is to
differential lengthening of two cases of Weber type I defi- be distinguished from fibular hemimelia, where there is a
ciency [51]. progressive deficiency of the fibula, ranging from a congeni-
tally short tibia with relative fibular hypoplasia to complete
absence of the fibula. An important distinction between these
Weber Patelloplasty two conditions is that the foot and ankle are always in varus
with tibial hemimelia and almost always in valgus with fibu-
The Weber patelloplasty was published in 2002 [78] and lar hemimelia. The progression of deficiency of the tibia is
described a surgical procedure in which the patella was from distal to proximal, contrary to fibular hemimelia, where
the progression of fibular deficiency is from proximal to

distal. Tibial hemimelia should be distinguished from proxi- Box 24.4. Paley Classification of Tibial Hemimelia
mal deficiency of the knee, which is most commonly associ- (See Fig. 24.2)
ated with TAR (thrombocytopenia absent radius) syndrome. • Type 1: Congenital short tibia with relative over-
Proximal deficiency of the tibia ranges from simple genu growth of the fibula, distal tibial plafond intact
varum to a ball and socket, complete absence, or congenital • Type 2: Congenital deficiency of the tibial plafond
fusion of the knee joint. Therefore, in order to prevent with diastasis of the tibia and fibula and relative
confusion, tibial hemimelia should be classified as an incre- fibular overgrowth
mental deficiency from distal to proximal. • Type 3: Congenital deficiency of tibia with proxi-
Most classifications in orthopedics classify pathology from mal and distal physis of tibia present; distal tibial
the less severe to the more severe (e.g., Garden classification of physis more dysplastic than proximal; relative fibu-
hip fractures [80], Berndt and Harty classification of talar lar overgrowth
dome osteochondral fractures [81], Herring and Catterall clas- • Type 4: Absent distal tibia; proximal tibia present;
sifications of Perthes [82, 83], and Kalamchi classification for relative fibular overgrowth
fibular hemimelia [84]). The Paley classifications [85] for con- • Type 5: Complete absence of tibia, fibula present
genital femoral deficiency and fibular hemimelia (FH) follow
a similar format. Some well-accepted published classifications
follow the opposite format, starting with the most severe or
deficient cases (e.g., Pappas classification for CFD [86],
Kalamchi classification of tibial hemimelia [48], and Jones Paley Classification with Subtypes
classification for tibial hemimelia [47]). The Jones classifica- and Description of Common Features
tion for tibial hemimelia has been the most widely used, but it
is in reverse order of deficiency. Furthermore, the Jones type Type 1: Valgus proximal tibia (genu valgum), overgrowth of
III is so rare that the senior author has only seen two cases of it fibula proximally, plafond present and normal.
in 27 years of practice and more than 300 cases of tibial hemi- Type 2: Tibial plafond missing. Medial and lateral mal-
melia. The complete absence of the proximal tibia in the Jones leolus present; distal fibula (lateral malleolus) normally
III makes its treatment similar to the Jones I where the proxi- aligned to lateral talus, internally rotated with foot around
mal tibial is missing. One consideration was to add this sub- the tibia. Talus is positioned between the tibia and fibula due
type to the Paley type 5 group. I have chosen not to do this to absence of tibial plafond. Relative fibular overgrowth
since the treatment is the same whether the distal tibia is pres- present. Foot follows the fibula. The diastasis can extend as
ent or not. The treatment at the knee still depends on the pres- a cleft between the tibia and fibula.
ence or absence of the patella and whether the fibula is Type 3a: Well-formed distal tibial physis and separate
autocentralized or not. The treatment of the foot does not from proximal growth plate of tibia. Plafond present but dys-
change since the foot is in severe equino-varus as if this reman- plastic. Major relative overgrowth of fibula.
ant of distal tibia was not present (Figure 24.3). Finally, the Type 3b: Delta Tibia: proximal and distal growth plates
Jones classification also lacks description of several types of connected through bracket epiphysis. Malorientation of ankle
tibial deficiency that involve both the proximal and distal phy- and knee joints ends of tibia. Major relative overgrowth of
ses, such as the Delta tibia type [87] or types that have a normal fibula.
appearing tibia beside a longer fibula. The Jones classification Type 4a: Delayed ossification of part, or all, of the tibia.
also does not consider the presence or absence of a patella. Ankle joint present, but distal tibial physis absent. Major
The Weber Classification [50, 79] changed the order back to relative overgrowth of fibula.
increasing severity of deficiency. It also included types with a Type 4b: Diaphyseal deficiency of tibia. Complete
proximal deficiency (type 3). Similar to the Pappas classifica- absence of distal tibia at level of diaphysis; proximal tibia
tion, it splits tibial hemimelia into a large number of types (total has atrophic, pointed bone end often covered by separate
of 7). The Weber also subdivides into types that will eventually skin pouch. Major relative overgrowth of fibula.
ossify and change into other types (types 3, 4, 5, 6, 7). The Type 4c: Epiphysis of proximal tibia present but absent
Weber scoring system, combined with the large number of sub- proximal physis. Knee joint present. Delayed ossification of
divisions, makes this classification challenging for practical use. epiphysis. Significant relative overgrowth of fibula.
Dissatisfied with the existing classifications and prior to Type 5a: Complete absence of tibia: a remnant of distal
Weber’s publication, Paley proposed a new classification in tibia may be present in rare cases; patella present; flexion
2003 [49]. Each type and subtype has a different surgical treat- contracture of knee.
ment. The progression of deficiency is from least to most, and Type 5b: Complete absence of tibia; no patella present;
the classification assumes that tibial hemimelia is a progres- flexion contracture of knee; fibula may be (i) auto-centralized
sive spectrum of deficiency from distal to proximal. or (ii) dislocated.
Limb Reconstruction Surgery option and should be considered as the most tried and proven
method of treatment. However, advances in the treatment of all
Since Brown introduced centralization of the fibula, many types of tibial hemimelia offer new options with excellent func-
attempts to reconstruct the knee in the most severe types (Jones tional results as an alternative to amputation. The rest of this
I, Paley 5) have been made. These have been met with poor manuscript will focus on the reconstructive options for tibial
results, as previously discussed. Similarly, poor results of hemimelia according to Paley type and subtype.
reconstruction for Jones types II and IV and Paley types 2, 3,
and 4 have led most surgeons to conclude that through-knee
amputation for Jones type I, through- or below-knee amputa- Paley Type 1 (Fig. 24.4)
tion for Jones type II, and Syme’s amputation for Jones type IV
are the best treatment for each type of tibial hemimelia. In light These patients tend to have bilateral involvement. The condi-
of the advances in modern prosthetics, this is definitely a good tion is often familial (autosomal dominant). The tibias are
Fig. 24.3 (a) Jones type III tibial hemimelia. This would be classified classification. (c) Magnetic resonance imaging (MRI) confirming that
and treated as a Paley type 5 despite the remnant of distal tibia. (b) Long there is no cartilaginous proximal tibia. (d) Another MRI cut confirm-
x-ray of both legs showing the bilateral nature of this rare type. The left ing absence of the proximal tibia
leg is a Paley type 3a and cannot be classified using the Jones
Fig. 24.4 (a, b) Right and left AP radiograph of Paley type 1 TH side fibula is not transported distally. (d) Final standing radiograph showing
showing valgus tibial deformity and overgrowth of fibula. (c) good alignment bilaterally. Proportions of femur to tibia restored closer
Lengthening and valgus correction of tibia and fibula with TSF. The to normal
short and the knees are in valgus. The valgus is usually from avoids the problem of a knee flexion contracture that can
the proximal tibia, but can also be from the distal femur. If result from transporting the fibula distally. If the proximal
treated when the physes are open, the valgus can be corrected fibula is producing a noticeable bump that is bothersome to
using temporary hemiepiphysiodesis. In adulthood, the val- the patient, then the tibia can be lengthened without cutting
gus can only be treated by osteotomy. The biggest complaint the fibula, transporting the fibula distally.
of these patients is mesomelic disproportion and short stat-
ure. Therefore, the treatment is usually bilateral and includes
tibial lengthening for stature in addition to correction of the Paley Type 2 (Fig. 24.5, 24.6)
valgus angulation. In regard to the overgrown proximal end
of the fibula, which often articulates with the side of the The diastasis of the tibia with the fibula starting with the
femur, I prefer to leave it alone and not pull it distally. This absence of the tibial plafond (Fig 24.4) may extend up the
Fig. 24.5 (a) AP and (b) lateral radiographs of Paley type 2 TH. The of the ankle joint is performed. A suture washer system is used to com-
distal fibula is more anterior than the tibia on the lateral. The distal fib- press the diastasis. An intramedullary wire is placed to hold the foot in
ula goes with the talus and is more hypertrophied than the distal tibia. place under the tibia and the external fixator is left in place for 3 more
(c, d) TSF device applied to foot and tibia for distraction of contracture months. (g, h) Epiphysiodesis of the distal fibula is performed to pre-
of foot and to move the talus under the distal tibia. (e, f) After the talus vent distal overgrowth of the fibula. The final result is a plantigrade
is under the tibia, the ankle joint is opened and a biologic arthroplasty stable foot with some motion of the ankle
tibia. The tibia may have a varus bow to its diaphysis and in and calcaneus causes it to follow the foot distally. This
rare cases there can be a skin cleft between the two bones moves the fibula from its relatively overgrown proximal
(Fig. 24.5). The foot always remains with the fibula. The foot position down to the normal station.
is in equinovarus and internally rotated relative to the knee. Once the foot is located under the distal tibial epiphysis, a
The talus is proximally migrated relative to the distal tibia. planned second stage surgery can be carried out. Under gen-
The talus is at the correct level relative to the distal fibula. eral anesthesia, the distal ring and wires are removed. The
Since the ankle joint is to be reconstructed, the Achilles ten- pin sites are covered by Betadine soaked gauzes and the leg
don is not cut but a gastrosoleus recession may be performed. is prepped and draped free. A transverse incision is made on
The foot is repositioned by gradual distraction of the foot the medial side at the level of the tip of the medial malleolus.
from the tibia using a circular ring external fixator. To pre- The tibiotalar joint is opened, and the distal tibia and proxi-
vent epiphysiolysis of the proximal and distal fibula, a 1.5- mal talus are exposed. The tibialis posterior tendon is often
mm wire is drilled retrograde into the fibula and up the found dislocated between the tibia and fibula, where the
fibular diaphysis to exit through the proximal fibular plafond should have been. It is moved out of this location
epiphysis. The wire is brought through the skin proximally and retracted postero-medial to the tibia. The distal end of
and then bent backwards on itself to form a hook. A small the tibial cartilage is carved with a knife to the concavity of
proximal incision is made and the wire is pulled back into the the tibial plafond, matching the convexity of the dome of the
fibular head to lock into the proximal epiphysis. Distally, the talus, creating a biologic arthroplasty. A wire, perpendicular
wire is also bent 180°, then shortened and buried under the to the sole of the foot, is passed through the dome of the
skin. This creates a temporary epiphysiodesis of the proxi- talus, through the epiphysis of the distal tibia, and continues
mal and distal fibula. proximally into the tibial diaphysis. If the tibia has a varus
One ring is applied to the proximal tibia with one wire diaphyseal bow to it, a percutaneous osteotomy should be
and two half pins. The second ring is applied to the foot made at the apex of this bow with an acute angular correc-
with three wires. The equinovarus deformity is corrected tion, straightening the tibia. The wire is advanced up the tibia
by gradual distraction of the foot away from the knee, repo- to stabilize this osteotomy.
sitioning the talus under the distal tibial epiphysis. Since The tibiofibular diastasis is treated next. This is fixed by
the fibula is overgrown relative to the tibia, it does not need using a syndesmotic suture system such as the Arthrex-
to be fixed to the distal ring. Its association with the talus Tightrope™ or the Biomet-Ziptite™. The syndesmotic
Fig. 24.6 (a) Paley type 2

variant: Clinical photo showing
there is a cleft associated with the
distal tibio-fibular diastasis.
Notice that the foot goes with the
fibula. There is also tibial
hemimelia on the other side
Paley type 5. (b) Radiograph
showing the diastasis and cleft.
(c) Radiograph of the TSF
external fixator applied to the
foot and tibia at onset of
correction. Notice the 90°
equinus contracture requiring the
rings to be at 90° to each other.
(d) AP and lateral radiographs
after the correction is completed.
The talus was centralized under
the distal tibia. The syndesmotic
repair was done using the Ziptite
suture-washer implant. The two
washers can be seen on the x-ray.
The cleft was surgically closed.
(e) Final AP and lateral
radiographs showing the foot is
plantigrade and the talus is
centered under the tibia. The
fibula is lateral to the talus.
The cleft and diastasis are closed.
(f) Final clinical photo from the
front showing that the cleft is
closed
suture with its two washers is used to reduce and compress for weight bearing. I prefer to leave both of these in place
across the diastasis. The incision is then closed, and the foot for 6 more months. This serves several purposes: preven-
ring is reapplied with three new wires. This helps ensure that tion of fracture of the now osteoporotic tibia and fibula, sta-
the foot remains in a plantigrade position. bilization of the ankle joint to prevent recurrence of equinus,
The external fixator is left in place for 3 more months. and retardation of the faster-growing fibula to prevent
The fibular wire as well as the transarticular tibial wire recurrent relative overgrowth. Six months later, both wires
should be left in place even after fixator removal. The tran- should be surgically removed. A solid AFO is used until the
sarticular wire can be advanced into the calcaneus to allow wires are removed, after which the patient is placed into an
articulated AFO with a plantarflexion stop. Physical ther- Paley Type 3b (Fig. 24.8)
apy is initiated after the transarticular ankle wire is removed.
This type is often associated with syndromes (e.g., Werner’s
dysplasia). There is usually duplication of toes. This is often
a familial condition (autosomal dominant) and bilateral. The
Box 24.5. Surgical Tips bracket epiphysis can be oriented in any direction and does
• Due to often aberrant anatomy, we recommend a not always correspond to the deformity seen. The fibula is
mini-open incision for the Achilles tenotomy. much longer than the tibia. The treatment in these cases is to
• A single 1.5-mm intramedullary wire protects consider the direction of the bracket in planning the surgery.
the fibular physes at both ends and prevents To interrupt the bracket, the cartilage of the epiphysis and
epiphysiolysis. physis is cut, and an osteotomy is performed through the
• After the ankle arthroplasty, pass the wire antegrade bone at the same level. To allow for acute correction of the
through the center of the dome of the talus, then deformity, part of the fibula must be resected. The acute cor-
pass it retrograde through the distal tibia. rection is accomplished by an opening wedge osteotomy on
• A suture system is used to reconstruct the syndes- the side of the bracket, with or without a partial closing
mosis between the tibia and fibula. wedge on the opposite side. The correction can be done with
or without lengthening at the same time. With lengthening,
it is done with the external fixator extending to the femur.
Without lengthening, fixation is obtained with axial retro-
grade wires entering through the foot and crossing the knee.
Paley Type 3
The presence of a proximal and distal growth plate defines Paley Type 4
type 3. As such, the knee is usually mobile albeit often unsta-
ble due to absence of cruciate ligaments and depression or In this type, there is a knee joint present. The degree of defi-
deficiency of part of the tibial plateau. The ankle plafond is ciency of the proximal tibia varies, but the knee is present
present but often dysplastic, and thus does not have much and functional. The foot is in very severe equinovarus. The
motion despite its presence. The presence of the plafond dif- lack of a distal tibia makes a mobile ankle joint not possible.
ferentiates it from type 2. Ankle diastasis is not typical, but Articulating the talus with the distal fibula in a biologic
some degree may be present depending on the severity of arthroplasty has met with recurrent deformity and failure.
dysplastic changes of the tibial plafond. Fusion of the talus to the distal fibular epiphysis is the goal
of treatment for this type. Transfer of the fibula to the distal
end of the tibia is also part of the reconstructive goal if no
Paley Type 3a (Fig. 24.7) cartilaginous anlage exists.
The foot is in equinovarus, and the tibia is also deformed.

The circular external fixator is applied, and gradual correc- Paley Type 4a (Fig. 24.9)
tion of the bony deformity is carried out together with the
foot, similar to the correction described for Paley type 2. At The salient feature in this type is the delayed ossification of
times, the foot deformity exceeds the bony deformity. After part or all of the tibia. When part of the tibia is affected, it is
the lengthening and deformity correction of the tibia is com- always the distal part. An MRI is useful to image the articu-
pleted, the distal tibia pins can be separated from the foot lations between the tibia and the femur and the tibia and the
ring and connected to the proximal tibial ring. The foot and ankle joint. Based on these, a decision can be made as to
ankle are then freed to move independently with the distal whether the deformity of the tibia needs to be corrected in
ring. Once all of the deformities are corrected by distraction, order to reorient the ankle to the knee. Since the fibula is
the bone is allowed to consolidate and eventually the exter- longer than the tibia, two options can again be considered for
nal fixator is removed. In these cases, the fibula can be managing the fibula: (1) resection to create a pseudarthrosis
treated in one of two ways: (1) resection of the mid-diaphy- of the fibula, or (2) lengthening of the tibia relative to the
sis of the fibula, in an attempt to create a fibular nonunion, or fibula. The ankle joint, while present in these cases, is not
(2) distraction of the tibia without fixation of the fibula, to functional. The goal is to create a plantigrade foot with a
pull the fibula down to its correct station at the knee and stable ankle. This usually requires distraction of the foot
ankle. The external fixator should always be extended to the through the ankle joint, and then a secondary arthrotomy as
femur to stabilize the knee joint. described for type 2 deficiencies, to best match the articula-
Fig. 24.7 (a, b) AP and lateral of Paley type 3a TH. (c) Lengthening of Hemi-plateau elevation to treat valgus. (g) Hemi-plateau elevation of
tibia with distal transport of fibula. (d) Recurrent deformity of tibia lateral tibial plateau to correct the valgus and stabilize the knee. (h)
(valgus). (e) Arthrogram of knee showing depressed lateral plateau. (f) Second lengthening. (i) Final result after second lengthening
Fig. 24.8 (a) A 6-month-old boy with Werner’s syndrome. Mother, and pinned retrograde from the foot. (h) After correction and amputa-
one sibling, and grandmother have it, too. Note the syndactylized mir- tion of extra toes. (i) Lengthening of tibia with external fixator to femur,
ror hands and eight toes. (b, c) Delta left tibia Paley type 3b. The tibia, and foot. (j) TSF device for lengthening. (k) Radiograph after
bracket epiphysis is facing anteromedial. (d) Cartilage of the bracket lengthening. He will require serial staged lengthening procedures in the
epiphysis intact (asterisk). (e) Cartilage of bracket epiphysis cut with future to equalize leg length discrepancy by skeletal maturity
osteotomy. (f) Fibulectomy. (g) Leg straightened by osteotomy of tibia
tion between the talus and tibia. The syndesmosis may or foot and up the tibia. In most cases, ossification of the anlage
may not need to be fixed with the suture technique described is already seen by 3 months after BMP implantation surgery.
for type 2. Biologic arthroplasty may also be necessary. Lengthening is usually done at 1 year after BMP insertion.
The unossified portion of the tibia will eventually ossify
after many years. To accelerate this process, bone morpho-
genic protein (BMP) can be inserted into the cartilage. This is Paley Type 4b (Fig. 24.10)
an off-label use of INFUSE (Medtronic, Memphis,
Tennessee). The basis of its use in tibial hemimelia comes This is the most common type 4 seen. The proximal tibial
from the senior author’s experience with congenital femoral epiphysis, physis, and metaphysis are well formed, a patella
deficiency, using BMP in a region of delayed ossification in is present, and active and passive knee motion are present
the femoral neck [88]. Ossification of the tibia facilitates through a normal range. The fibula is overgrown and
lengthening and deformity correction of the tibia through proximally migrated and the foot is in extreme varus and, to
bone. If sufficient parts of the tibia are bony, an osteotomy a lesser extent, equinus. The goal of treatment is to correct
can be made through the bony portion and pins placed in the the foot deformity, fuse the talus to the end of the fibular
bony portion. If an insufficient portion of the tibia is ossified epiphysis distally, and transfer the fibular diaphysis to the
to allow for external fixation, then open surgery is performed distal end of the tibia proximally. This makes a one-bone leg.
to acutely realign the foot with a tibial osteotomy, combined While this can be accomplished in one stage, it is safer, eas-
with resection of part of the fibular diaphysis. To ossify the ier, and more length preserving to do this in two stages.
tibial anlage (nonossified portion of the tibia), BMP is inserted The first stage involves applying a circular external fix-
into drill holes in the cartilage. Stabilization of the osteotomy ator to the femur, tibia, and foot. The knee is locked in exten-
is achieved with retrograde axial Kirschner wires through the sion by inserting an axial wire through the distal tip of the
Fig. 24.9 (a) Preoperative AP radiograph showing Paley type 4a tibial a plantigrade position. The fibula was also acutely shortened. BMP was
hemimelia with severe equino-varus. Note the non-ossified region of the inserted into the anlage via drill holes. The tibia and fibula were gradually
distal tibia. (b) Preoperative clinical photo showing the severe equino- lengthened through a separate proximal osteotomy. The distraction gap can
varus deformity of the foot. (c) Preoperative lateral radiograph showing the be seen. A femoral external fixator (to protect the knee) with hinges, is con-
non-ossified region of the distal tibia. (d) MRI showing a cartilage anlage nected to a tibial external fixator for lengthening. (f) Final long standing AP
is present articulating with the ankle joint. (e) A distal tibial acute angular radiograph showing the bone is straight and the distal part is ossified. In
correction combined with shortening was performed to correct the foot to future additional lengthenings will be required for leg length equalization
Fig. 24.10 (a) AP radiograph of bilateral TH with Paley type 4b on the the distal fibular epiphysis and the fibula was transferred to the tibia to
left and 2 on the right. (b) Lateral radiograph of left leg Paley type 4a form a one-bone leg. On the right side the treatment discussed in
with severe equinovarus. (c) Lateral radiograph of right leg Paley type Fig. 24.4 was carried out to achieve a stable plantigrade foot. The dias-
2 with severe equinovarus. (d) Application of TSF to left foot and femur tasis on the right was also corrected. (f) Lateral of left foot. The fusion
to correct the equinovarus deformity and bring the foot under the talus. at the ankle may not be complete. The foot equinus has partially
A temporary epiphysiodesis wire is in place to prevent epiphysiolysis of recurred. (g) The right foot is plantigrade and maintains some range of
the fibula during distraction. (e) After correction the foot was fused to motion after the biologic arthroplasty
tibia, crossing the knee and inserting into the femur. The ately inserted in the same track to protect the fibula from
external fixation pins are in the femur only (two half pins at fracture. The fibula is often osteoporotic at this stage and
the femoral neck level and one wire at the ring level) and not without an intramedullary wire, it can easily break upon
in the tibia. This avoids having a contaminated pin site in the manipulation of the foot or leg. The cartilage at the end of the
operative field at the time of the second stage surgery. Wires fibula is incised and pared back until the ossific nucleus of
are used in the foot (three in the calcaneus and foot and one the fibula is reached. If there is no ossific nucleus, then BMP
transverse in the talus). The foot is then distracted away from can be inserted to get the epiphysis to ossify. The talus is also
the tibia to correct its deformity and to gain length. This cre- incised parallel to the sole of the foot to the level of its ossific
ates a large space between the talus and the end of the tibia. nucleus. The two ossific nuclei are then aligned, and the wire
The goal is placing the talus distal to the lateral malleolus. To is advanced from the proximal end through the talus and sole
prevent epiphysiolysis of the fibula during distraction, a tem- of the foot to hold the foot plantigrade to the fibula.
porary epiphysiodesis wire is inserted into the fibula to pro- A longitudinal anterior incision is made over the tibial
tect its two growth plates (as described for type 2). diaphysis and extended distally. Fasciotomy of the anterior
Once the foot is in the corrected position, the second stage compartment is carried out. The anterior compartment mus-
surgery is carried out. The first step is to remove the foot ring cles are elevated off of the tibia, and a path is then dissected to
and cover the pin sites with an occlusive dressing to mini- the fibula. The fibula is exposed subperiosteally. The wire in
mize contamination during surgery. After the leg is prepped the fibula is backed out distally to the level of the planned
and draped free, a transverse lateral incision is made over the osteotomy. The fibula is then cut at the level of the distal end
distal tip of the fibula. The distal epiphysis of the fibula and of the proximal tibia. The fibula is then shifted over to the
the dome of the talus are exposed. The capsular connections tibia. The tibia can either be split longitudinally to accept the
between them are cut to mobilize both bones relative to each end of the fibula inside of it, or it can be cut transversely to
other. A small incision is made proximally over the fibular allow end-to-end apposition of the two bones. In both cases,
wire. The fibular epiphysiodesis wire is cut either proximally the fibular wire is advanced across the proximal tibia up to the
or distally and then removed. A new straight wire is immedi- joint line of the knee. The wire in the foot is the same wire in
the fibula that is advanced across into the tibia. The external with physical therapy. In the future, lengthening of the one-
fixation wires in the foot are reinserted and connected to a bone leg can be carried out without crossing the knee joint.
ring. Struts are now used between the upper femoral ring to
the distal foot ring. The external fixator maintains the align-
ment of the foot and knee to achieve fusion of the tibia and Paley Type 4c (Fig. 24.11)
fibula proximally and of the fibula and talus distally. This usu-
ally takes 3 months. After that, the external fixator is removed, In this type, there is only a proximal tibial epiphysis present
leaving one wire buried in the foot and fibula to protect the and no proximal tibial physis. The proximal tibial epiphysis
fibula for a few months longer. The knee motion is restored is often unossified at an early age. The foot is in severe
Fig. 24.11 (a) Radiograph of Paley type 4c. There were eight metatar- device (8 cm). (f) Third lengthening over a nail in tibia and fibula
sals. They were resected and transferred on a vascular pedicle to the leg (10 cm). (f) Fourth lengthening using ISKD internal lengthening device
to connect with the tibial epiphysis. (b) Clinical photo of the same. (8 cm). (g) Final radiographic result after four lengthenings and 32 cm
(c) Osteotomy across the three transplanted metatarsals and the fibula gained. (h) Clinical appearance and knee function 0–90° after 32 cm of
for first lengthening (6 cm). (d) After first lengthening and bony con- lengthening. (j) He is sports active, plays soccer, and rides a bicycle
solidation. (e) Second lengthening of tibia and fibula with Ilizarov
proximal tibial epiphysis. Finally, the foot wire and ring are
Box 24.6. Synostosis and Ankle Fusion Tips reapplied and connected with six struts to the proximal ring.
• The Paley type 4 lacks a distal tibia, and the goal is It is important to use an external fixator because the axial
usually to obtain a fusion of the tibia remnant and foot wire cannot maintain the foot in a plantigrade position.
the fibula proximally, and a fusion of the distal fib- The fixator remains in place for 3 months until the fibula
ula and talus distally. fuses to the tibial epiphysis proximally and the talus distally.
• The femoral ring is kept relatively high on the femur Knee range-of-motion exercises ensue after that.
to stay out of the surgical field for subsequent An alternative form of treatment in this type is possible
surgeries. when there are duplicate metatarsals, which can be trans-
• Protect the fibula with an intramedullary temporary planted into the space between the tibial epiphysis and the
epiphysiodesis wire as previously described. This is talus. The metatarsals can be taken as free grafts, but they are
also important intraoperatively while working on preferably taken as vascular pedicle grafts flipped proximally
either end of the fibula. on their pedicle. Iliac crest bone grafting between the meta-
• Once the proximal fibula is brought in line with tarsals promotes them to unite together into one-bone mass.
the proximal tibia, switch the transverse wires in Once this occurs, an osteotomy across the new tibia and the
the fibula from the distal ring to the proximal ring. fibula can be done for lengthening. The fibula remains proxi-
Then focus on correcting the ankle deformity. mally dislocated as long as it does not impede knee motion.
• Remove extraneous wires several days prior to sur- Repeated lengthenings are needed to equalize limb length
gery to minimize infection risk. discrepancy.
• A longitudinal split in the tibia like a clothespin
allows greater surface area and healing potential.
• BMP-2 can be used to promote ossification of Paley Type 5
cartilage.
• Use the external fixator and intramedullary wires to Complete absence of the tibia presents the biggest challenge
provide stability and maintain correction until for reconstruction because there is no knee joint. While ankle
fusion is achieved. fusion gives good function with little disability, knee fusion
• An arch wire can help provide compression to the leads to significant disability for sitting and climbing stairs.
talo-fibular fusion. It is preferable to avoid a knee fusion. Even if active knee
motion cannot be achieved, a mobile knee joint supported by
a brace is preferable to a knee fusion. This is not dissimilar
equinovarus, and the fibula is relatively overgrown and prox- to a paralytic knee from polio. Therefore, the following two
imally migrated at the knee. The treatment preferred is also a methods have been developed to reconstruct the knee.
two-stage surgery, with the first stage using a circular fixator
to correct the foot deformity. There is often a knee flexion
contracture present in these cases. It is also treated by dis- Paley Type 5a (Fig. 24.12)
traction of the knee and foot with the same external fixator. A
temporary fibular epiphysiodesis wire is used. A transverse If a patella is present, it can be converted to a tibial plateau.
wire can be added to the fibula. Initially this wire is con- This original idea was first published by Michael Weber [78,
nected to the distal foot ring, and the fibula and foot are both 79]. The patella is moved on a vascular pedicle from its nor-
distracted distally, bringing the fibular head under the epiph- mal position, anterior to the femur, to the distal end of the
ysis of the tibia. Once this is achieved, the transverse fibular femur. The fibula is centralized to the patella and its epiphy-
wire is detached from the foot ring and connected to the sis fused to the patella. This procedure is referred to as the
proximal tibial ring. This allows distraction of the foot defor- Weber patellar arthroplasty or Weber procedure.
mity, and the foot is pulled distally to the end of the fibula. The Weber procedure can be performed acutely (as
In order to minimize the risk of infection, the fibular wire described by Weber) with gradual correction of a flexion
is removed in the office prior to going back to surgery for the contracture of the knee joint, using a hinged external fixator,
second stage surgery. The same treatment is carried out to or following staged gradual distraction of the fibula to first
fuse the talus to the fibula as described in type 4b. At the correct the knee flexion contracture (according to Paley).
knee, the fibula is fixed to the tibial epiphysis. If the epiphysis When performed acutely, the foot deformity is gradually dis-
is unossified, then BMP is inserted into drill holes in the tracted at the same time as the gradual knee contracture cor-
cartilage. The proximal fibular epiphysis is pared down as rection. When performed after the distraction, the foot is
was done for the distal epiphysis. The intramedullary wire distracted away from the fibula as described above with a
used to fix the foot is advanced proximally to transfix the temporary epiphysiodesis wire in place. Once the femur,
Fig. 24.12 (a, b) AP and lateral radiographs of Paley type 5a TH. The fixator an intramedullary wire was used to help protect the fibula. (h, i)
foot was previously treated and remains in an equinovarus deformity. AP and lateral of the knee and tibia after healing from the Weber. Note
(c, d) AP and lateral radiographs after the Weber patellar arthroplasty how well remodeled the knee appears. Due to leaving a small fragment
was performed and the patella jointed to the fibula. The ankle was fused of the patella behind a new patella developed. The knee has excellent
in a plantigrade position for the foot. Initially there was a flexion con- active range of motion. (j, k) Clinical photos of the leg after one length-
tracture of the knee. The circular external fixator was used to correct ening. Note the active knee flexion and extension
this. (e, f) After distraction the knee is straight. (g) After removal of the
fibula, and foot are all aligned, the Weber procedure can be Weber Patellar Arthroplasty (Fig. 24.13)
performed through a longitudinal anterior incision over the
distal femur and proximal fibula. At the distal end, the ankle An anterior midline incision is made over the distal femur
fusion can be performed as described above through a trans- and proximal fibula. Medial and lateral skin flaps are ele-
verse lateral incision. vated to expose the fibula and distal femur. The patella is
24 Tibial Hemimelia
Fig. 24.13 (a) Weber patellar arthroplasty incision. (b) The underlying fascia is exposed over distal one goes proximal. (g) The quadriceps tendon is repaired and the visor flaps sutured
the patella, femur, and tibia. (c) The peroneal nerve should be exposed and decompressed and together. (h–k) Corresponding H-flaps are incised into the perichondrium of the patella and the
the biceps tendon lengthened. (d) The two visor flaps are marked out. (e) The visor flaps are fibular head and the ossific nucleus of the fibular head and the patella exposed by resecting some
incised and the posterior capsule is cut with great care to identify the vessels posteriorly so as of the overlying cartilage. (l) The patella and fibular head are mated to each other. (m) The
not to injure them. (f) The two visor flaps are flipped so that the proximal one goes distal and the H-flaps are sutured together
475
foot. After the external fixator is removed, the patient goes

Box 24.7. Weber Patelloplasty Tips into a cast for a month; when the cast is removed, they are
• Correct knee flexion contractures as much as pos- fitted for a KAFO brace. A brace is needed for many years
sible with frame distraction before surgery. A poste- until the knee is stable enough to allow walking without the
rior capsulotomy may be necessary. brace. This is usually after age 10, when adequate hypertro-
• Find and protect the peroneal nerves and the poste- phy of the joint surface and fibula has occurred.
rior tibial neurovascular bundle.
• If there are remnants of the medial or lateral ham-
strings, these can be preserved and reattached to the Type 5bi (Fig. 24.14)
fibula at the end of the operation.
• Keep the visor flap pedicles as wide as possible. If there is no patella, but the fibula is autocentralized, then
Deepen the cuts just enough to allow the mobility to there is usually a quadriceps muscle in continuity to the fib-
slide them around each other. Usually the upper ula. The distal femur is usually less dysplastic in these cases.
patella flap goes underneath. The knee still presents with a fixed flexion contracture and
• Patellas can be present in various shapes. A vertical the foot presents as with type 5a, dislocated and in extreme
patella can be easily split, such that a small remnant varus and equinus. The treatment is to distract the knee con-
is translated superiorly with the visor flap and form tracture until the fibula and femur are collinear with each
a new patella. other. The foot should also be distracted relative to the fibula
• The H-flaps should be vertical on one side and hori- to centralize it under the distal end of the fibula. This is
zontal on the other and should be determined by the accomplished with an Ilizarov device on the femur using
anatomy and shape of the patella and fibula. hinges and a circular ring fixator for the foot correction. This
• Cut down into the cartilage of the patella and proxi- permits simultaneous correction at both ends of the bone.
mal fibula until reaching the ossific nucleus. If these This is possible because the fibula is autocentralized. If the
are not present, then consider placing BMP-2 to fibula is dislocated as in type 5bii, a circular fixator is needed
promote ossification. for both knee and ankle and the correction is done sequen-
• Suture the posterior H-flap first before fixing the tially as for type 5a. Once the distraction correction at both
patella and fibula with a wire. knee and ankle is completed, a second stage surgery is per-
formed to reconstruct collateral ligaments at the knee and to
advance the quadriceps muscles onto the fibula. An ankle
fusion as described above is performed. The distal ring on the
identified. Two “visor” flaps are outlined with a marking foot is removed and then reapplied in this surgery. All wires
pen. At the medial and lateral ends, the pedicle for each near the knee are removed in the office at least 1 week prior
“visor” is kept as wide as possible. The proximal visor flap to the surgery. The external fixator is left in place for another
contains the patella. The distal visor flap is all capsular. In 3 months and then removed. A spica cast is applied for a
the Paley modification, the distal visor flap includes a small month and then an HKAFO brace is made with a hinged knee
part of cartilage of the inferior pole of the patella. This allows and solid ankle.
a new patella to form anterior to the femur. A Z-lengthening
of the quadriceps tendon is performed as part of the proximal
visor flap. If the fibula or patella is unossified, Weber Type 5bii (Fig. 24.15)
recommends using two perichondral H-flaps to get the two
cartilage surfaces to heal together. To ensure bone-to-bone If there is no patella and the fibula is dislocated, the fibula
healing, we prefer to insert BMP into drill holes in the carti- can be centralized by distraction. First, a temporary epi-
lage of the patella and fibular epiphysis. To avoid injury to physiodesis wire is inserted up the fibula and hooked over
the popliteal vessels, it is preferable to find and protect them each epiphysis at both ends. This is to protect both proxi-
before completing the posterior capsulotomy. mal and distal fibular physes. An Achilles tendon tenotomy
Since the knee and ankle were predistracted, they can is carried out. Then, one proximal and one distal circular
both be fixed by one axial wire across the knee joint. The fixator ring is applied with two half pins in the proximal
circular external fixator is used only as a holding device to femur (one up the femoral neck and one transverse at the
ensure full knee extension and a plantigrade foot. The medial level of the lesser trochanter) and one wire on the proximal
and lateral collateral ligaments should be reconstructed ring in the mid-femur. A distal ring on the foot is applied
using local tissues (biceps tendon laterally and semitendino- with three anterior posterior wires in the foot (one from
sus medially). After 3 months, the external fixator is removed posterior midcalcaneus to anterior mid-forefoot, one from
and the trans-knee wire shortened to span only the fibula and posteromedial calcaneus to anterolateral forefoot, and one
Fig. 24.14 (a, b) AP and lateral radiographs of Paley type 5b (i) prevent epiphysiolysis. (f, g) After the knee and foot are corrected, the
TH. The fibular is autocentralized on the femur. For this reason it has knee is reconstructed by open surgery to do a quadricepsplasty and to
hypertrophied. The knee joint has a fixed flexion deformity. (c) The foot reconstruct the collateral ligaments. The ankle is fused: talo-fibular
is in severe equinovarus relative to the fibula. (d, e) Hinged construct fusion. The external fixator is left on for 3 months after this second
used to correct knee contracture. A knee hinge wire is located across the surgery. It is then removed and a brace is used to support the leg. (h, i)
distal femur. Taylor Spatial Frame is used to correct foot contracture The distal fibular epiphysis is fused to the talus in a plantigrade posi-
gradually. A temporary epiphysiodesis wire is in place in the fibula to tion. The knee is well reconstructed
from posterolateral calcaneus to anteromedial forefoot) and to the knee. The transferred muscles are all connected
one transverse wire across the ossific nucleus of the talus. together with the lateral and medial muscles balancing
One wire is inserted into the fibula and connected to the each other and centralizing themselves to the iliotibial
foot ring. The spatial frame planning is done relative to the band, which acts as a central rib for the connection of all of
proximal ring for the knee joint contractures. Once the fib- these muscles. It is eventually attached to the front of the
ula is reduced at the knee and the knee flexion contracture fibula to act as the patellar tendon.
eliminated, the fibular wire is connected to the proximal Before connecting the transferred muscle, it is important
ring and the spatial frame planning repeated for the distal to anchor the fibular head to the femur. This is done by cre-
ring and foot contractures. When both ends of the fibula are ating an interosseous ligament between the femur and the
aligned to the knee and ankle, the second stage surgery can fibula. One can use allograft or the half of the biceps tendon
be performed. for this purpose. This single step prevents subluxation of the
fibula from the femur. As dislocation was the main reason
for failure of the original Brown procedure, the interosseous
Paley Centralization of the Fibula ligament prevents subluxation from occurring while permit-
on the Femur (Fig. 24.16) ting hinge flexion of the two bones. The ankle is fused as
previously described. A wire is inserted from the foot
Through a midline anterior incision, the distal femur and through the fibula and then through the knee joint. The foot
the proximal fibula are exposed. The peroneal nerve is lib- ring, which was removed at the start of the procedure, is
erated and decompressed from the fibula. The biceps ten- reapplied with the circular fixator struts. The foot is immo-
don is split with half of its tendon left attached to the fibula bilized in a plantigrade position and the knee in full exten-
to be used as a lateral collateral ligament. The other part is sion. Three months later, the external fixator is removed, but
transferred to the fibula anteriorly to rebuild a quadriceps the medullary wire crossing the ankle and knee is left in
muscle. On the medial side, the semitendinosus muscle is place. A cast is used for 1 month and then a KAFO is pre-
also transferred anteriorly. The tensor fascia lata and its pared. The wire is removed 6 months later and knee reha-
iliotibial band are also harvested and transferred. The bilitation is started. Other than amputation, the alternative
adductor magnus is also transferred. These four muscles or fall back operation for this type of tibial hemimelia would
are connected to the quadriceps muscle. The quadriceps be a knee fusion. In unilateral cases, this is a reasonable
muscle usually ends in the mid-thigh and does not continue alternative.
Fig. 24.15 (a, b) Radiograph of Paley type 5b (ii). There is a disloca- The foot is fused to the distal epiphysis of the fibula and the biceps,
tion of the fibula on the femur and the foot on the fibula. There is a semitendinosus, tensor fascia lata, and adductor magnus muscles are all
severe equinovarus of the foot and flexion contracture of the knee. (c, transferred to the knee to substitute for the missing quadriceps muscles.
d) A Taylor Spatial Frame is applied from the femur to the foot with one A temporary arthrodesis wire is placed across the knee and ankle. The
wire across the tibia. A temporary epiphysiodesis wire is in place in the external fixator was removed 3 months after the knee-ankle surgery.
fibula. (e, f) After 3 months of distraction the knee contracture is elimi- The wire across the knee and ankle is left in place for 6 months to allow
nated and the foot is reduced below the end of the distal fibula. (g, h) better loading and minimize the risk of recurrence. It is then removed
Summary achieving a functional lower extremity. For type 5a, where a

patella is present, the results of a Weber patellar arthroplasty
Through-knee amputation remains the most commonly used in the hands of experienced surgeons are also very satisfac-
procedure for complete tibial aplasia. It is certainly a reli- tory. The reconstructive options for tibial hemimelia have
able option that can be carried out by most orthopedic sur- improved greatly over the past two decades. With continued
geons. It should be seriously considered in unilateral cases. success and improvements, they may one day overtake the
In bilateral cases of Paley type 5, complete tibial aplasia amputation option. Other anatomic variants of tibial hemi-
reconstruction by one of the techniques described above melia including duplication of fibula, tarsals, metatarsals and
should be considered, especially on the side that has a patella. toes, and secondary femoral condyles, have not been dis-
Amputation is probably overused in Paley type 2, 3, and cussed. They are not included in the classification either.
4 tibial hemimelia. In the senior author’s opinion, recon- These associated anatomic variants must be considered in
structive results for these types are reliable and successful in the treatment but are beyond the scope of this chapter.
24 Tibial Hemimelia
Fig. 24.16 (a, b) Incision for acute reduction of Paley type 5b (ii). (c, d) Biceps tendon length- are identified and the posterior capsule is incised. All of the hamstrings and gastrocnemius ten-
ening, peroneal nerve decompression, and femoral shortening to treat the knee flexion contrac- dons are released. (i) The biceps tendon is used to create a cruciate ligament to tether the fibula
ture. (e) Fixation of the distal femur. (f) Reduction of fibula to femur. (g) Advancement of the to the femur. (j) The biceps is advanced and the new cruciate ligament is secured
479
remnant of quadriceps to the fibula. (h) To fully correct the knee flexion contracture, the vessels
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Congenital Pseudarthrosis of the Tibia
25
Corinna C. Franklin and Richard S. Davidson
product neurofibromin [3]. As many as 75 % of CPT cases are

Anatomy and Pathology associated with NF-1, though only 5 % of NF-1 patients
develop CPT [3]. Histologically, there is no difference
Congenital pseudarthrosis of the tibia (CPT) describes a spec- between cases with or without NF-1 [4]. Non-orthopedic
trum ranging from anterolateral bowing of the tibia to frank manifestations of NF-1 include café-au-lait spots, optic glio-
pseudarthrosis and disappearance of bone. It is a rare condi- mas, neurofibromas, malignant peripheral nerve sheath
tion, with an estimated incidence of 1/150,000–250,000 births tumors, and increased risk of cardiovascular diseases [3].
[1–3]. CPT is typically unilateral and located at the junction Other orthopedic manifestations include scoliosis and abnor-
of the middle and distal thirds of the tibia; it occurs with equal malities of the cervical spine; patients often have decreased
frequency in boys and girls [1]. Patients may present initially bone mass due to the loss of neurofibromin function [3].
with a fracture or simply with a bowed tibia; the fibula is The method by which NF-1 is related to or causative of
affected in about one-third of patients [1, 22] (Fig. 25.1). CPT is imperfectly understood. A recent study by DY Lee
The pathology of CPT involves fibrous hamartoma and et al. suggested that the fibrous hamartoma of CPT in NF-1
pathological periosteum [1]. Pathologic examination of CPT results from periosteal cells that have failed in terminal
lesions has revealed highly cellular tissue similar to fibroma- osteoblastic differentiation, arresting without completing the
tosis; the gap contained fibrous tissue as well as both fibro- differentiation process [6]. Another study by SM Lee et al.
cartilage and hyaline cartilage, and was continuous with the indicated that double inactivation of the Nf1 gene could be
periosteum [5]. This hamartomatous tissue is thought to present in some cases of CPT but is not the essential require-
interfere with bone production and callus formation, perhaps ment for development of CPT [7].
by decreasing the blood supply [2]. Pseudarthrosis of the tibia has also been described in
cases of osteofibrous dysplasia or fibrous dysplasia. Boyd
described several cases of CPT as having pathology similar
Associations to fibrous dysplasia in 1958 [8]; Campbell et al. reported on
five cases which they termed a variant of fibrous dysplasia
There is a strong association between CPT and neurofibroma- involving anterior bowing of the tibia [9]. Campanacci et al.
tosis type 1 (NF-1). Also called von Recklinghausen disease, reported on a series of 35 cases of what they termed osteofi-
NF-1 is inherited in an autosomal dominant manner and brous dysplasia, one of which had a pseudarthrosis of the
is relatively common, with an incidence of 1/3,000 [3]. tibia, and ten of whom had bowing of the tibia [10].
The NF-1 gene is located on chromosome 17 and encodes the The tibial bowing in CPT is distinctly anterolateral.
Bowing in other directions occurs in rickets (typically tibia
vara or valga) or in posteromedial bowing of the tibia [11]
C.C. Franklin, MD but is unrelated to pseudarthrosis of the tibia and treated very
Shriners Hospital for Children, 3551 North Broad St, differently.
Philadelphia, PA 19102, USA
R.S. Davidson, MD (*) Classification Systems
Department of orthopedic Surgery, The Children’s Hospital
of Philadelphia, 34th and Civic Center Blvd., 2nd Floor
Wood Building, Philadelphia, PA 19107, USA Multiple classifications of CPT have been described; these
e-mail: [email protected] include the systems of Crawford, Boyd, and Andersen.

484 C.C. Franklin and R.S. Davidson
Crawford described a classification system in 1986 based Boyd described six types of CPT in 1982; in this assess-
on the radiographic appearance of the tibia in neurofibroma- ment type 2 has the worst prognosis [13]:
tosis; in this assessment type 4 has the worst prognosis Type 1: Anterior bow and defect of the tibia, possibly associ-
(Fig. 25.2) [12]: ated with other congenital deformities.
Type 1: Anterolateral bow with a dense medullary canal. Type 2: Anterior bow and hourglass constriction of the tibia
Type 2: Anterolateral bow with a widened medullary canal with fracture typically before 2 years of age; often associ-
and a tubulation defect. ated with neurofibromatosis.
Type 3: Anterolateral bow and a cystic lesion. Type 3: CPT developing in a bone cyst, usually at the junc-
Type 4: Anterolateral bow with evident fracture, cysts, or tion of the middle and lower two-thirds of the tibia.
pseudarthrosis. Type 4: CPT originating in the same location without tibial
narrowing but with at least partial, if not complete,
narrowing of the canal.
Type 5: A dysplastic fibula with fibular or tibial pseudarthrosis.
Type 6: Intraosseous neurofibroma or Schwannoma.
Andersen described a series of 46 patients with pseudarthro-
sis of the leg in 1976, which he separated into six types [14]:
Club foot: True congenital fracture present at birth, associ-
ated with other congenital anomalies.
Cystic: Cystic lesions of the distal tibia and fibula without
angulation or fracture at birth.
Late: Pseudarthrosis developed after fracture through a scle-
rotic tibia (at 4 and 9 years of age).
Fibular: Pseudarthrosis first developed in the fibula.
Dysplastic: Dysplastic, hourglass tibia with pseudarthrosis;
associated w/neurofibromatosis.
Angulated: No radiographic evidence of dysplasia; pseudar-
throsis developed after osteotomy; associated with
neurofibromatosis.
In practice, the most relevant criterion to guide treatment
Fig. 25.1 Photograph of tibia with anterolateral bow is whether the tibia is fractured or intact.
Fig. 25.2 Crawford

classification of CPT: Type 1:
anterolateral bow with a dense
medullary canal; type 2:
anterolateral bow with an
increased medullary canal and a
tubulation defect; type 3:
anterolateral bow and a cystic
lesion; type 4: anterolateral bow
with evident fracture, cysts, or
pseudarthrosis
25 Congenital Pseudarthrosis of the Tibia 485
Imaging Bracing is also frequently used postoperatively in order to

support the surgical procedure.
The mainstay of imaging for CPT is anterior-posterior and
lateral radiographs of the affected leg. Computed tomography
(CT scanning) may be used to quantify healing at the CPT Operative Management
site. More recently, magnetic resonance imaging (MRI) has
been used to assess CPT; it has been suggested that MRI can CPT is a challenging disorder to treat. The underlying condi-
provide additional information such as the type, length, and tion makes healing difficult, and multiple procedures and revi-
structure of the pseudarthrosis, as well as the extent of the sions may be needed. Ultimately some cases may eventually
affected periosteum and subtle soft-tissue changes [15]. This be treated with an amputation. Many procedures exist for the
may assist in treatment planning and monitoring. treatment of CPT. These include bypass grafting, intramedul-
lary fixation, free fibula transfer, Ilizarov external fixation,
periosteal grafting, Masquelet’s procedure, and amputation.
Prognostic Factors
Multiple prognostic factors have been proposed and debated, Modified McFarland Bypass Graft
much of which remains controversial. Association with NF-1
was thought to indicate a poorer prognosis; however, this is Ofluoglu et al. describe a method of prophylactically treating
no longer thought to be the case, as this has not been borne an anterolaterally bowed tibia in NF1 with a posteromedially
out in the literature [1]. The relationship between age and placed allograft fibula combined with bracing until maturity.
prognosis is complex. There has been debate over when to In their series of ten, no patient had a fracture or a pseudar-
initiate surgical management, with some authors advocating throsis at final follow-up (Figs. 25.3a–c and 25.4a–f) [19].
early intervention and others urging waiting until at least age This technique is performed under tourniquet with the use
4 [4]. Boyd asserted that older children would do better after of an image intensifier. The patient is positioned supine and
surgical treatment [13]. However, Morrissy et al., reporting the lower leg is placed on the operating table such that the
on a series of 40 cases of CPT, felt that older children were tibial deformity is in the horizontal plane. The skin and soft
more likely to have a poor surgical outcome; they found that tissues are incised anteromedially from the proximal to the
patients who still required grafting at or after age 13 years distal physis; the periosteum is then resected, taking care not
did poorly [16]. to incise too close to the physes. Notches are then made in the
Tudisco et al. examined 30 patients with CPT who had tibial metaphysis proximally and distally about 2 cm from the
reached the end of skeletal growth, and found that radio- physes. An allograft fibular strut, several millimeters longer
graphic classification was correlated with prognosis.
Crawford type 2 and 4 had the worst prognosis for union,
with type 4 having the worst functional results [17]. Ohnishi
et al., reporting on a series of 73 patients with CPT, felt that
residual deformity and narrow diameter of the bone at the
CPT site would lead to refracture [18].
Finally, a more distal location in the tibia may worsen the
prognosis of CPT as this location is more difficult to control
and necessitates the involvement of the ankle and the hind-
foot in fixation [1].
Non-operative Management
In general, the treatment of CPT is surgical. Early on, patients

with anterolateral bowing may be braced in order to attempt to
delay progression to fracture and CPT [1]. At first, when a
child is younger than 3 or 4 years old, knee-ankle-foot orthoses
(KAFOs) are used in order to control rotation of the lower
extremity and prevent progression to fracture . Thereafter, a
patella-tendon-bearing clamshell orthosis is used with medial
and lateral femoral flanges, which will also control rotation. Fig. 25.3 (a–c) Modified McFarland bypass graft technique
Fig. 25.4 (a–j) CPT treated by the modified McFarland bypass graft method
than the span of the two notches, is impacted into the two Remodeling often leads to resorption of the tibia in favor of
notches under compression, taking care to place the graft on enlargement and incorporation of the fibular graft. Proximal
the concavity of the bow of the tibia to accept weight bearing. and distal metaphyseal deformity may need guided growth
Thus, this bypasses the bowed tibia. A screw, a wire, or heavy plates and screws if remodeling is incomplete.
suture is then used to fix the distal end of the graft to the tibia,
and the tibia facing the graft is decorticated. Bone graft is
then used to facilitate incorporation of the allograft. The Intramedullary Nailing
patient is then placed in a long leg cast and kept non-weight
bearing until radiographic evidence of bone healing is noted In 1956, Charnley described the use of an intramedullary
(6–8 weeks). Thereafter the patient wears a total contact nail to treat CPT; two patients were treated with Steinmann
clamshell patellar tendon-bearing orthosis (flared at the knee nails in the intramedullary canal [20]. Sofield, in 1971,
to control rotation) or a hinged KAFO until maturity [19]. reported on a series of more than 100 patients with CPT, and
long, it should be cut and stabilized with a Kirschner wire.

The bone graft is placed around the pseudarthrosis site and
secured with heavy suture. Patients are cast for about 4
months—spica initially in young children, and then above
knee. Thereafter a clamshell KAFO is used with a free knee
and locked ankle. In older children, this may eventually
be converted to a total contact ankle-foot orthosis (AFO).
Once the distal edge of the rod approaches the talar articu-
lar surface, via longitudinal growth of the tibia, a second pro-
cedure is done to advance the rod across the ankle.
The orthosis is then converted to a hinged ankle and is worn
until maturity [22].
Free Vascularized Fibula
A free vascularized fibular (FVF) graft from the contralateral

leg may be used as a strut after excision of a tibial pseudar-
Fig. 25.5 X-rays of CPT treated with an intramedullary nail throsis. This functions as a structural replacement for the
excised portion of the tibia. In the ideal case, the vascularity
of the graft allows it to heal more quickly, avoid resorption,
asserted that those treated with intramedullary fixation did and add vasculature to a scarred and dysplastic site [23].
best; he advocated fragmentation, reversal of the fragments, FVF transfer requires a specialized team with microsurgery
and fixation with an intramedullary rod (Fig. 25.5) [21]. expertise, and careful attention to detail.
Dobbs et al. describe a treatment method of CPT using a Several authors have reported on the results of FVF trans-
Peter Williams rod for stabilization after excision of the fer in the treatment of CPT. Dormans et al. reported on a
pseudarthrosis. They report a satisfactory outcome in 16 of series of 12 patients in which FVF was used to treat CPT; at
21 patients; complications included reoperation in 5, ampu- final follow-up, one patient had a persistent nonunion [24].
tation in 5, ankle valgus in 10, and leg-length discrepancy Similarly, Weiland et al. reported on a series of 19 patients
in 11 [22]. with CPT treated with FVF; of these, 18 had healed at final
This technique is performed under tourniquet with an follow-up [25]. Iamaguchi et al. present a cautionary view of
image intensifier. Iliac crest bone graft is first acquired. An this procedure; in their series of 16, 63 % required multiple
anterior incision is made longitudinally extending proxi- surgeries to achieve union, but eventually all 16 did [26].
mally and distally from the pseudarthrosis site. The pseudar-
throsis is excised down to normal, bleeding medullary
canal, which is then reamed both proximally and distally. Ilizarov External Fixation
A Williams rod is used to stabilize the tibia (implants such as
the Fassier-Duval rod may also be considered, although there The Ilizarov circular external fixator may be used in
is concern for threaded screws in the growth plate). The size CPT. Several different techniques and frame configurations
(length and diameter) of the rod is determined preopera- have been described. Paley et al. describe a series of 15
tively, based on anticipated growth. In most cases, the rod patients with 16 CPT who were treated using the Ilizarov
traverses the ankle. In children younger than 4, it will go into device. The rate of union was 94 % after one treatment and
the calcaneus; in patients who are 5–10 years old, the rod 100 % after two; there were five refractures and two residual
will end in the talus; in those older than 9 or 10 (or if the deformities [27]. Boero et al. describe a series of 21 patients
pseudarthrosis is quite proximal), the rod will not cross the with CPT treated with the Ilizarov device; at final follow-up,
ankle. The assembled rod is passed antegrade into the distal 9 had consolidated without deformity or shortening, 5 con-
tibial segment, and then out through the talus and calcaneus, solidated with axial deviations, and 7 did not consolidate
finally passing out through the heel pad, taking care to [28]. Ghanem et al. reviewed a series of 14 patients with CPT
position the foot and ankle correctly in neutral. Then, the rod treated using the Ilizarov fixator; in all instances the pseudar-
is passed back up retrograde into the proximal tibial seg- throsis site was not excised but the leg was realigned and
ment, again taking care to align the tibia properly (this may lengthened. Union was ultimately achieved in 13 cases,
require a second, proximal osteotomy). If the fibula is too 6 having required additional grafting [29] (Fig. 25.6a, b).
Fig. 25.6 (a, b) Clinic photo and

X-ray demonstrating CPT treated
with the Ilizarov device
The pseudarthrosis site may be treated by direct end-to-

end compression, by side-to-side compression, or by insert- Masquelet’s Procedure
ing one part into another; it may also be resected, with
lengthening through a proximal corticotomy, through the site Masquelet has described a procedure, called induced mem-
itself, or by bone transport [27]. Typically, a four-ring brane, for reconstruction of long bone defects which he has
assembly is used, with two rings proximal and two distal to applied to CPT [31]. This is performed in two stages. Initially,
the pseudarthrosis site; the foot may be included if the pseud- a broad debridement is done, followed by the insertion of a
arthrosis is very distal in the tibia [28]. cement spacer (made out of polymethyl methacrylate). In the
The versatility of the external fixator may also allow asso- second stage, performed 6–8 weeks later, the spacer is
ciated deformities in the ipsilateral lower extremity to be extracted but the membrane surrounding it is not removed;
treated. Lengthening as well as correction of angular defor- this is filled with iliac crest bone graft (and supplemented
mities may be performed [27, 28]. with allograft if needed). An external fixator is used, and the
fibula is often incorporated [31]. Further investigation is
required on this method as the available results in the litera-
Periosteal Grafting ture are limited (Fig. 25.9a–d).
Thabet et al. report on the results of a technique that is a

combination of several methods, using intramedullary fixa- Amputation
tion and Ilizarov circular fixation along with bone and peri-
osteal grafting. Their case series includes 20 patients, all of Amputation is usually considered an option of last resort.
whom united after the first procedure; 8 patients sustained However, some difficult cases of CPT end up requiring many
refractures, reoperation was required in 7 cases, and no complex surgeries, with long periods in which the patient is
amputations were performed [30]. unable to fully bear weight, play sports, or engage in many
This technique is performed under tourniquet and with an activities shared with their peers. In these cases, amputation
image intensifier. An anterior incision is used; the cuff of abnor- may become the most attractive option, as it would mean an
mal periosteum is incised, circumferentially dissected, and end to the frequent procedures and, with current advanced
removed. A small longitudinal split is made in the proximal prostheses, allow most sports and activities to be possible.
tibia segment, and the distal end is drilled open and then inserted Furthermore, many patients with neurofibromatosis will
into the proximal end. The same procedure, including periosteal have other conditions requiring treatment, such as malignan-
excision, is performed on the fibula. Intramedullary fixation is cies or spinal deformities, and may wish to curtail the mul-
then placed in both the tibia and fibula (static or expandable tiple surgeries involved in treating a recalcitrant CPT in
rods were both used in the referenced paper). Next, iliac crest order to keep some years of childhood free of hospital visits
bone graft is harvested, as well as the periosteum over the ilia- if possible. In younger children, an ankle disarticulation is
cus muscle. These are placed around the pseudarthrosis site, appropriate (as the malleoli may be expected to remodel),
periosteum first. Finally, an Ilizarov fixator is applied, with whereas a Syme’s amputation is indicated in older patients.
three wires and a ring proximally and the same distally; the Amputation through the pseudarthrosis itself is indicated if
frame is extended to the foot [30] (Figs. 25.7a–f and 25.8a–h). infection is present (Fig. 25.10a, b).
Fig. 25.7 Treatment for type I CPT in the lateral and anteroposterior the pseudarthrosis and (e) iliac crest bone graft is applied to the tibial and
(AP) views is shown. (a) There is longitudinal splitting of the proximal fibular docking site. (f) External fixator is applied. With kind permission
tibial and fibular fragments. (b) The bone ends are docked and (c) IM rods from Springer Science + Business Media: Clinical Orthopedics and
are inserted (Fassier-Duval telescopic IM nail with Paley modification Related Research, Periosteal grafting for congenital pseudarthrosis of the
illustrated) into the fibula and tibia. (d) Periosteal graft is wrapped around tibia: A preliminary report, 466(12), 2008, Thabet AM
Medical Management Bone Morphogenetic Protein
Several medical adjuncts to surgical treatment have been BMPs are biologic substances, several of which stimulate bone
described in the literature. These include the use of bone formation. BMP-2 and BMP-7 are commercially available in
morphogenetic proteins (BMPs), bisphosphonates, bone the USA, and have been used in procedures such as spinal fusion
stimulation, and bone marrow grafts. In all cases, further and repair of open or non-united tibial fractures in order to pro-
research is needed before firm conclusions can be drawn or mote or accelerate fusion [32, 33]. These proteins may be a use-
recommendations made. ful adjunct to CPT procedures; currently this is an off-label use.
Fig. 25.8 (a) Anteroposterior (AP) and (b) lateral view radiographs of view radiograph shows retreatment with an Ilizarov device and rerod-
a 2-year-old boy with neurofibromatosis and CP that had not been pre- ding with Fassier-Duval telescopic IM nail locked in both proximal
viously treated are shown. (c) AP view radiograph shows split of the and distal epiphysis. (g, h) Tibia remains healed 2 years later with
proximal segment with invagination of the distal segment, rodding of good remodeling. The telescopic nail has extended with growth. With
tibia from medial malleolus and of fibula through lateral malleolus, and kind permission from Springer Science + Business Media: Clinical
application of Ilizarov external fixator. (d) AP view radiograph obtained Orthopedics and Related Research, Periosteal grafting for congenital
immediately after external fixator removed shows union of the CPT. (e) pseudarthrosis of the tibia: A preliminary report, 466(12), 2008,
AP view radiograph shows refracture of the tibia 1 year later. (f) AP Thabet AM
Fig. 25.9 Masquelet’s induced membrane technique principles. neous tissue results in a containment system. (d) Principles of recon-
Elements of technique. (a) The preoperative aspect of the membrane struction of the tibia. At the first stage the PMMA spacer is applied on
just before filling up the cavity. Note the petaling of the bone extremity. the fibula. At the time of reconstruction an intertibiofibular graft is per-
(b) Morcellized cancellous bone graft; the chips should be as small as formed on both extremities. Reprinted from Orthopedic Clinics of
possible. The alveolar structure of the cancellous bone permits a fast North America, 41 (1), Masquelet AC, Begue T, The concept of induced
revascularization by the vascular buds issued from the membrane. (c) membrane for reconstruction of long bone defects, 27–37, Copyright
The cavity is filled up; the suture en bloc of the membrane and subcuta- 2010, with permission from Elsevier
A further patient required revision but went on to union. One

patient had a persistent nonunion and ultimately underwent
an amputation. No complications related to BMP were
reported. The authors concluded that the use of BMP-2 in
this setting improved outcomes [34].
In contradistinction, Lee et al. reported on a series of five
patients in which BMP-7 was used as an adjunct to surgery
but concluded that this did not promote improved bone heal-
ing in these patients [35].
Bisphosphonates
Bisphosphonates are analogues of pyrophosphate that inter-

fere with osteoclast activity, and thus slow bone resorption;
they have been used in several diseases in which bone metab-
Fig. 25.10 (a, b) X-rays of a patient who eventually underwent a
olism is disturbed or bone turnover is excessive, such as
below-knee amputation following multiple attempts at reconstruction fibrous dysplasia [36]. Birke et al. proposed that bisphospho-
as well as history of three primary malignancies and a large leg-length nates would be a useful adjunct in treating CPT, particularly
discrepancy that would have required several more surgeries as NF-1 involves deficient bone metabolism [37]. They
reported on a series of eight cases in seven patients (six of
Richards et al. reported on a series of seven patients with whom had NF-1) in whom BMP-7 and either pamidronate or
CPT in whom BMP-2 was used as an adjunct; in all cases a zoledronic acid were systemically administered as adjuncts
Williams rod was used for fixation [34]. Five patients went to CPT surgery. Primary healing was achieved in six of the
on to radiographic union, one of whom later had a fracture eight cases, with the remaining two cases eventually going on
that was retreated in the same manner and went on to heal. to heal after multiple procedures and subsequent refractures.
The authors concluded that bisphosphonate therapy was a A split is created in the proximal tibia, and the distal tibia
useful addition to CPT surgery [37]. It should be noted that canal is opened with a drill. The distal portion is inserted into
there exist limited data on the long-term effects of bisphos- the proximal split. Similarly, the distal fibula is split and the
phonate use in children or women of child-bearing age. proximal portion inserted into it. If there has been prior sur-
gery, all dead bone should be resected (the tourniquet may be
let down in order to observe which portions of the bone are
Electrical Stimulation bleeding and healthy). An intramedullary rod is then inserted
into the tibia across the site of the CPT, followed by another
In the past, several studies looked at using electrical current rod in the fibula. The ankle is not crossed.
to augment surgical treatment of CPT [38, 39]. This then fell Iliac crest bone graft is then harvested, as well as perios-
out of favor for some time. More recently, however, Cebrian teum from the underside of the iliacus muscle. The perios-
et al. published a study examining the use of pulsed electro- teum is meshed using a skin graft mesher and then placed
magnetic fields (PEMFs) in adult cases of CPT treated with around the invaginated CPT site, with the cambium layer
intramedullary nailing. They found improved healing in facing the bone; this is also done on the fibular site. The har-
those cases in which the PEMFs were used compared to vested cancellous graft is then placed around the CPT site
those in which the PEMFs were not used, and suggested that and in the space between the tibia and fibula. BMP-2 is
this is a useful addition to CPT surgery [40]. placed as well, including into the space between the tibia and
fibula, in order to promote cross-union.
Finally, an Ilizarov fixator is placed, with three proximal
Bone Marrow Grafting wires, three distal wires, and fixation of the foot, followed by
a walking ring. After union (about 12 weeks after surgery),
In 1995, Garg and Gaur published a report of one patient the fixator is removed and a long leg walking cast is placed.
with CPT in whom a percutaneous injection of harvested This is then followed by a KAFO with a free knee and solid
bone marrow cells from the iliac crest was used; this patient ankle. In patients older than 6 years, the ankle is articulated;
healed with casting (after prior excision of the pseudarthro- as the patient grows, the brace is modified, progressing to a
sis) [41]. A similar case was reported by Kitoh et al., in which total contact articulated AFO or patellar-tendon-bearing
culture-expanded osteoblast-like cells as well as autologous brace only. Once the patient is older than 10, a gator brace
platelet-rich plasma were injected into the distraction site of (with medial/lateral malleolar flanges) is used. The brace is
a CPT being treated with distraction osteogenesis, with a worn at all times (other than bathing and therapy) until skel-
good result [42]. A study of the biological basis for the use of etal maturity.
bone marrow cells in the treatment of CPT noted that this The intramedullary rod is changed as needed as the patient
was a promising strategy meriting further study [45]. grows, with zoledronic acid infusions accompanying rod
exchange surgeries. A telescoping rod may be useful in this
setting. Hemi-epiphysiodesis may also be performed as
Combined Approach needed for valgus at the ankle or knee [44].
Recently, Dror Paley has described a combined approach for

the treatment of CPT for which he has reported excellent Summary
results, though longer follow-up and confirmation of this
approach’s feasibility in the hands of other surgeons are Congenital pseudarthrosis of the tibia is a rare condition that
required [44]. This method combines pharmacologic and encompasses a wide spectrum from anterolateral bowing of
surgical management in order to maximize the chances of a the tibia to true pseudarthrosis with bone loss. The periosteum
successful outcome. in CPT is pathologic, with the presence of fibrous hamar-
Either 1 week prior to or 1 week following surgery, the toma. There is a strong association between CPT and neuro-
patient is given zoledronic acid intravenously (0.2 mg/kg over fibromatosis-1 (NF-1); it may also be associated with fibrous
30 min), followed 1 hour later by 60 mg/kg of calcium gluco- dysplasia or osteofibrous dysplasia. Multiple classification
nate intravenously over 60 minutes. The patient also is to take systems exist; the key criterion in guiding treatment is
2 g elemental calcium for 7 days and 400 IU of vitamin D for whether the tibia is fractured or intact. Treatment is primarily
14 days. surgical, though medical augmentation with BMPs and/or
The surgical procedure is performed under tourniquet; bisphosphonates may also be useful. A wide variety of surgi-
the approach is via an anterior longitudinal incision. The cal procedures exists, including bypass grafting, intramedul-
diseased periosteum is incised longitudinally and excised lary fixation, free fibula transfer, Ilizarov external fixation,
circumferentially in its entirety. Posterolaterally to the periosteal grafting, and Masquelet’s procedure; some cases
tibia, the fibular hamartomatous periosteum is also excised. may ultimately end in amputation.
Treatment of CPT has continued to evolve over the past 17. Tudisco C, Bollini G, Dungl P, Fixen J, Grill F, Hefti F, et al.
Functional results at the end of skeletal growth in 30 patients
25 years in a mostly additive fashion based on the principles
affected by congenital pseudoarthrosis of the tibia. J Pediatr Orthop
of removal of pathologic tissues, replacement with viable tis- B. 2000;9:94–102.
sue, stabilization, and, most recently, addition of medical 18. Ohnishi I, Sato W, Matsuyama J, Yajima H, Haga N, Kamegaya M,
stimulation. While this combined method has produced the et al. Treatment of congenital pseudarthrosis of the Tibia. J Pediatr
Orthop. 2005;25(2):219–24. doi:10.1097/01.bpo.0000151054.
best chance of healing and avoidance of refracture, addi-
54732.0b.
tional study will be needed to distill this extensive complex 19. Ofluoglu O, Davidson RS, Dormans JP. Prophylactic bypass graft-
series of treatment measures to ascertain the minimal num- ing and long-term bracing in the management of anterolateral bow-
ber of steps to optimize treatment. ing of the tibia and neurofibromatosis-1. J Bone Joint Surg Am.
2008;90(10):2126–34. doi:10.2106/JBJS.G.00272.
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Congenital Posteromedial Bowing
of the Tibia 26
Benjamin Joseph, Hitesh Shah, and N.D. Siddesh
series of 33 patients gathered over a 36-year period; Hoffman

Introduction and Wenger [8] reported 13 patients seen over a 14-year
period; Johari et al. saw 31 children in an 18-year period [9]
Congenital posteromedial bowing of the tibia is a relatively and we reported 20 patients seen over a 19-year period [10].
uncommon condition. It is one among a group of congenital These four reports, all based on retrospective data from ter-
bowing deformities of the tibia that include anteromedial, tiary care centers, suggest that most of such centers would
anterolateral, and posteromedial bowing. In the past these probably encounter one or two new cases of congenital pos-
three deformities were arbitrarily grouped together and teromedial bowing per year. These hospital-based estimates,
referred to as congenital kyphoscoliotic tibia [1]. It is well however, are prone to several biases and hence are, at best,
recognized that the clinical features, natural history, and long- rough indicators of the magnitude of the problem. In 2008
term prognosis of posteromedial bowing of the tibia are dis- we put in place a registry for congenital posteromedial bow-
tinctly different from the more sinister anterolateral bowing ing of the tibia and have been prospectively following up all
associated with neurofibromatosis and congenital pseudar- new cases. In these last 5 years we documented 34 new cases.
throsis and anteromedial bowing associated with fibular defi- This suggests that the condition is much more common than
ciency [2–5]. Though a diagnosis may be made in the antenatal previously estimated from retrospective studies. The greater
period during routine ultrasound evaluation of pregnancy [6, frequency of cases noted over the last 5 years is likely to be
7] in the majority of instances the diagnosis is made at birth. a reflection of more reliable nature of data generated pro-
spectively rather than an increase in the incidence.
Incidence
Demographic Features
The exact incidence of congenital posteromedial bowing of
the tibia is unknown though hospital-based reports suggest Bilateral involvement is rare; we encountered one bilateral
that it is an uncommon condition. Pappas [5] reported a case among 54 cases. There is an unexplained predilection
for the left limb to be affected; left-sided involvement is
almost twice as frequent as the right [10]. Boys and girls
appear to be equally affected.
B. Joseph, MS Orth, MCh Orth (*)
Department of Paediatric orthopedics, Aster Medcity,
Kuttisahib Road, Cheranalloor, Kochi, Kerala 682027, India
e-mail: [email protected] Etiology
H. Shah, MS (orthopedics), DNB (orthopedics)
Department of orthopedics, Kasturba Medical College, Dawson attributed the deformity to malunion of an intrauter-
Kasturba Hospital, Manipal University, Madhavnagar, Manipal, ine fracture though he provided no real evidence to support
Karnataka 576104, India
this hypothesis [11]. De Maio et al. [7] had the unique oppor-
tunity of having performed an autopsy on a fetus with pos-
N.D. Siddesh, MS (orthopedics), FRCS (Glasg)
teromedial bowing aborted at 24 weeks of gestation. They
Department of orthopedics, Kasturba Medical College, Manipal
University, Madhav Nagar, Manipal, Karnataka 576104, India noted evidence of amniotic rupture and attributed this as the
e-mail: [email protected] possible underlying cause of the deformity in that fetus.

496 B. Joseph et al.
towards the medial side (Fig. 26.5a) [10]. The epiphysis may
Deformities then develop asymmetrically into a wedge-shaped epiphysis
(see Fig. 26.5b) resulting in a valgus tilt of the ankle mortise
The Tibia [10]. The growth delay of the fibula with a high lateral mal-
leolus referred to above also contributes to ankle valgus.
The tibia is bowed posteriorly and medially (Fig. 26.1a–d); in
the newborn the posterior bow is usually more pronounced
Box 26.1. The Deformity
than the medial bow though the medial bow may exceed the
• Posteromedial bowing at the junction of the middle
posterior bow in some instances [10]. The degree of posterior
and lower thirds of the tibia and fibula.
angulation in the newborn may be as high as 90° (see
• Posterior bow is usually more severe than medial
Fig. 26.1b). The site of the deformity is characteristically at the
bow.
junction of the middle and lower thirds of the leg. A skin dim-
• Shortening is proportionate to severity of bowing.
ple may be seen at the apex of the bow. The tibia is invariably
• Always associated with a calcaneo-valgus defor-
shorter than the unaffected side and the degree of shortening is
mity of the foot.
proportionate to the severity of bowing (Fig. 26.2a, b).
• Valgus deformity of the ankle may develop later
Though the degree of shortening frequently is in the order
due to growth abnormalities of the distal tibia and
of 15 or 20 % up to 40 % of shortening has been recorded
fibula.
[10]. Occasionally there may be an associated torsional
deformity of the tibia (see Fig. 26.2b).
The Fibula
The fibula is also bowed and the degree of deformity paral-

lels the tibial deformity. The distal fibula physis is often at a
higher level than normal suggesting that there is a relative
growth retardation of the fibula as compared to the affected
tibia (Fig. 26.3).
The Ankle and Foot
The tibial deformity is invariably associated with a calcaneo-

valgus deformity which may be very severe at birth with the Fig. 26.2 Examples of a short tibia associated with congenital postero-
medial bowing. The shortening is mild when the bowing is mild (a) and
dorsum of the foot lying almost in contact with the shin
greater when the bowing is more severe. Occasionally, a torsional
(Fig. 26.4). The distal tibial epiphysis may appear late and deformity may also be present (b). The patella and lateral malleolus
when it does appear it may be eccentrically positioned have been marked to demonstrate the torsional deformity
Fig. 26.1 Congenital posteromedial bowing of the tibia in a newborn. The clinical and radiographic appearances of the posterior bow (a, b) and
the medial bow (c, d) are clearly seen. A delay in the ossification of the proximal tibial epiphysis and the cuboid is evident
26 Congenital Posteromedial Bowing of the Tibia 497
The Natural History
Rate and Pattern of Spontaneous Remodeling
The posteromedial bowing deformities of the tibia and fibula

tend to improve spontaneously over time. The resolution of the
bony deformity occurs by two distinct mechanisms; one entails
resorption of bone from the convex surface and new bone
deposition on the concavity of the bow in accordance with
Wolff’s law [10]. De Maio et al. demonstrated that this pattern
of remodeling begins in utero [7]. At 24 weeks of gestation an
abundance of osteoclasts were present at the apex of the defor-
Fig. 26.3 The fibular physis is at a higher level than normal (at the mity while an osteoblastic response was seen in the concavity.
level of the ankle joint) As this process of remodeling progresses the angular defor-
mity decreases and the angulation between the proximal and
distal segments of the bone diminishes. The second mecha-
nism of spontaneous correction of the deformity involves
realignment of the proximal and distal growth plates. Physeal
reorientation occurs at a faster rate than remodeling at the site
of the deformity [10]. Remodeling of the deformity by both
these mechanisms occurs at a rapid rate during the first year of
life and thereafter the rate of remodeling decreases quite sub-
stantially dropping to half the initial rate (Fig. 26.6) [10].
Residual Deformities, Shortening,

and Functional Problems
Complete resolution of the diaphyseal deformity does not

occur in a proportion of children and residual deformity that
is clinically discernable may be seen in children older than 4
or 5 years of age (Fig. 26.7a, b) [10].
Shortening of the limb increases with growth [8] and the
Fig. 26.4 Calcaneo-valgus deformity seen in association with congen-
limb length discrepancy at skeletal maturity may frequently
ital posteromedial bowing of the tibia
be of a magnitude to warrant treatment. Pappas noted a
median shortening of 4.1 cm at skeletal maturity with a range
between 3.3 and 6.9 cm [5]. The foot length of the affected
limb is often less than the normal foot [5, 8, 12].
Fig. 26.5 Eccentric ossification

of the distal tibial epiphysis in a
toddler with congenital
posteromedial bowing of the tibia
(a) and a wedge-shaped distal
tibial epiphysis in an older child
with congenital posteromedial
bowing of the tibia (b). The
valgus tilt of the ankle becomes
more evident as the child grows
older (c)
Fig. 26.6 The rate of

spontaneous resolution of 60 Rapid
bowing is rapid in the first year resolution
of life, slows down thereafter,
and reaches a plateau by 4–5
years of age 50
Medial bow (Degrees)

40
Moderate
resolution
30
Slow resolution
20
Plateau
10
0
0 1 2 3 4 5 6
Age (years)
Fig. 26.7 Residual posterior

bowing of both tibiae in a child
aged 4 years with bilateral
posteromedial bowing of the tibia
(a) and residual bowing at 8
years in another child (b). The
AP radiograph shows that the
distal tibial articular surface is
not parallel to the plane of the
knee joint even though the
medial bow has remodeled to a
very large extent
Limitation of ankle motion may persist; limitation of both

plantar flexion and dorsiflexion has been reported [8, 10]. Evaluation
Weakness of plantar flexion (Fig. 26.8) has been observed
in some children even though the bony deformity may have Establishing the diagnosis is not difficult once the plane of
remodeled almost completely [10]. On occasion the weakness the deformity is clear. The evaluation should include a care-
of the gastrocsoleus may be severe enough to result in a cal- ful documentation of the clinical estimate of the severity of
caneal hitch and a visible gait abnormality requiring surgical the diaphyseal deformity in the coronal and sagittal planes.
intervention [10]; we have encountered two such children. The foot should be passively plantar flexed and inverted to
assess the rigidity of the calcaneo-valgus deformity.
Box 26.2. Spontaneous Resolution of Bowing Anteroposterior and lateral full-length plain radiographs of
• Occurs by local remodeling and by physeal both tibias and a scanogram should be taken to facilitate
realignment. accurate estimation of the angulation and shortening. The
• Occurs rapidly in the first year of life. degree of shortening of the tibia should be documented and
• Little remodeling occurs after 4–5 years of age. expressed both as a percent of the normal tibial length and in
• Complete resolution may not occur. centimeters. The extent of bowing should be estimated by
measuring the diaphyseal angle and the inter-physeal angles
The child should be reexamined at 6 months of age to eval-

uate the extent of correction of the calcaneo-valgus deformity
and the tibial bowing. If sufficient resolution of the foot defor-
mity has not occurred casting should be considered. The child
should be seen again at 1 year of age to plan the extent of shoe
raise that may be required to facilitate more normal walking.
The child should then be followed up annually for the next 4
years at which time a decision should be taken regarding
intervention for residual deformities and shortening.
The child should be reviewed finally at skeletal maturity
to document if limb lengths have been equalized and if there
are any residual deformities or functional limitations.
Treatment
Indications
1. Treatment is indicated for any deformity of the leg, ankle,

or foot that is cosmetically unacceptable provided ade-
Fig. 26.8 The power of plantar flexion is being tested in a boy with quate time has been given for spontaneous resolution of
congenital posteromedial bowing of the tibia. Weakness of plantar flex- the deformities.
ion on the effected side is clearly evident. This weakness that was pres- 2. Treatment should be recommended if by the age of 6
ent before he underwent corrective osteotomy for residual deformity
still persists years sufficient resolution of the deformity has not
occurred to make the articular surface of the distal tibia
parallel or almost parallel (<5°) to the knee joint on the
anteroposterior radiograph of the tibia.
3. Treatment should be recommended for any functional
deficit that may affect normal gait.
Aims of Treatment
Inter-physeal Diaphyseal
angle • Correct the calcaneo-valgus deformity of the foot in the
angle
infant.
• Correct residual coronal, sagittal, and transverse plane
(torsional) deformities of the tibia and fibula in the older
child.
• Restore the normal alignment of the ankle to restore a
horizontal tibial plafond.
• Equalize limb lengths.
• Improve the power of ankle plantar flexion if there is
severe weakness of the gastrocsoleus.
Fig. 26.9 The diaphyseal bow and the inter-physeal angles can be
measured on the anteroposterior and lateral radiographs Treatment Options
Correction of the Calcaneo-Valgus Foot

on the radiographs (Fig. 26.9). The parents should be coun- Deformity
selled and reassured that the deformity will reduce spontane- Passive stretching: The mild degree of calcaneo-valgus
ously but they need to be warned that complete resolution deformity responds to gentle passive stretching similar to the
may not occur. They should also be instructed in performing response seen in idiopathic congenital calcaneo-valgus
passive stretching exercises of the foot into plantar flexion deformities not associated with posteromedial bowing of the
and inversion and encouraged to do it at least twice a day. tibia.
than 5 cm. An acceptable option is to perform a contralateral

proximal tibial and fibular epiphysiodesis at the appropriate
time based on the anticipated correction estimated from a
growth chart. This option is particularly attractive when the
shortening is in the order of 2–3 cm and there are no residual
deformities to be addressed.
Limb lengthening: Gradual lengthening of the tibia is typi-

cally needed when the limb length discrepancy at skeletal
maturity is likely to exceed 5 cm. Lengthening may also be
considered for smaller degrees of discrepancy when defor-
Fig. 26.10 A medial screw epiphysiodesis has been performed to cor- mity correction is also required. In such situations both
rect ankle valgus. The wedge-shaped distal tibial epiphysis and the these problems can be addressed by a single procedure
abnormal proximal location of the distal fibular physis are seen [14]. However, the lengthening cannot usually be per-
formed through the osteotomy site used for correcting the
tibial deformity as the apex of the tibial diaphyseal defor-
Serial casting: The more severe degrees usually respond to mity tends to be too far distal; a second osteotomy in the
serial manipulation and cast application; very rarely anterior proximal tibia is typically required for more reliable new
soft tissue release may be required [13]. bone formation during limb lengthening. If a circular fix-
ator is used, gradual correction of the deformity at the dis-
Correction of Sagittal, Coronal, or Torsional tal osteotomy site can be undertaken while distraction for
Deformities of the Tibia and Fibula limb length equalization proceeds at the proximal osteot-
Acute correction: Acute correction by performing an osteotomy site. Limb lengthening through an osteotomy in the
omy at the site of the deformity is a simple and attractive proximal tibia can also be undertaken with a monolateral
option particularly when the residual deformity is in a single frame once acute correction of the biplanar deformity is
plane and when the extent of limb length discrepancy does achieved at the site of bowing in the same sitting
not warrant formal limb lengthening. Fixation of the osteot- (Fig. 26.11a, b).
omy may be with either internal or external fixation.
Correction of Muscle Imbalance Across
Gradual correction: Gradual correction with an external fix- the Ankle
ator may be the more appropriate option when there are Tendon transfer: Tendon transfers are seldom effective in
deformities in two or three planes and if concomitant limb restoring powerful push-off if the gastrocsoleus is com-
lengthening is required. pletely paralyzed [15] but may be effective in posteromedial
bowing of the tibia where the gastrocsoleus is weak with
Correction of the Valgus Ankle some residual muscle function. We prefer to perform a pero-
Physeal manipulation (guided growth): Valgus deformity of neal translocation in which the peroneus longus tendon is
the ankle due to abnormal distal tibial physeal and epiphy- re-routed behind the calcaneum under tension (without
seal growth is best addressed by modulating physeal growth dividing and re-attaching the tendon as in a tendon classic
such that the growth of the medial part of the physis is transfer) to augment the gastrocsoleus in this situation
restrained using either an extra-periosteal plate and screw (Fig. 26.12) [16].
device or a single transphyseal screw. The latter option is
simpler and the authors’ preferred option (Fig. 26.10).
Box 26.3. Treatment
Supra-malleolar osteotomy: If the distal tibial epiphysis is • Non-operative correction of calcaneo-valgus foot
normal in shape and the relative lengths of the tibia and fib- deformity in infancy.
ula are normal a valgus deformity of the distal tibia should be • Surgical correction of residual deformities of the
corrected by a supra-malleolar osteotomy. tibia and fibula after 6 years of age
• Equalization of limb lengths
Correction of Limb Length Inequality • Correction of valgus ankle
Contralateral epiphysiodesis: In many instances the pro- • Correction of weak plantar flexion
jected limb length discrepancy at skeletal maturity is less
Decision Making
The factors to be taken into consideration while planning

treatment are [17]:
• The degree of spontaneous resolution of the tibial defor-
mity in a child over 4 years of age
• The extent of anticipated limb length inequality at skele-
tal maturity
• The site of ankle deformity, if present
The outline of treatment is shown in Table 26.1.
Pitfalls in Diagnosis and Treatment
Pitfalls in the diagnosis and management of posteromedial

bowing can be prevented by careful observation of the site
and the plane of the deformities. An erroneous diagnosis of
congenital pseudarthrosis of the tibia can be avoided by not-
ing that the direction of tibial bowing is diametrically oppo-
site to anterolateral bowing of pseudarthrosis.
Fig. 26.11 Corrective osteotomy for correction of residual bowing Before embarking on treatment of ankle valgus it is imper-
combined with a more proximal osteotomy for limb lengthening has ative that the site of the deformity is clearly identified. An
been performed. Acute correction of the posterior (b) and medial (a) abnormal wedge-shaped distal tibial epiphysis with an inclined
bow was first performed through the distal osteotomy and the fixator
was applied taking care to ensure that the normal alignment of the tibia distal tibial articular surface should be treated by medial
was restored in both planes. The proximal osteotomy for the limb hemiepiphysiodesis while ankle valgus caused by distal tibial
lengthening was then performed angulation should be treated by a supra-malleolar osteotomy.
Fig. 26.12 Diagrammatic S SS’: Axis of subtalar joint S

representation of how the muscle AA’: Axis of ankle joint
balance is restored across the axis EDL
EDL
of the ankle joint by performing a EHL EHL
peroneal translocation operation
TA TA
A A‘ A A‘
PB PB
TP TP
Peroneus Longus
Peroneus Longus
Achilles Achilles
tendon tendon
S‘ S‘
Tendon of muscle with Grade III muscle power

Tendon of muscle with Grade V muscle power
Table 26.1 Outline of management of congenital posteromedial bowing of the tibiaa

Indication Treatment recommendation
Anticipated limb length discrepancy at skeletal maturity < 2 cm No intervention
Near-complete resolution of tibial deformities in all planes
No ankle deformity
Anticipated limb length discrepancy at skeletal maturity 2–4 cm Contralateral epiphysiodesis of the proximal tibia and fibula
Near-complete resolution of tibial deformities in all planes at appropriate time so as to equalize limb lengths by skeletal
No ankle deformity maturity
Anticipated limb length discrepancy at skeletal maturity > 4 cm Proximal tibial lengthening to equalize limb lengths close to
Near-complete resolution of tibial deformities in all planes skeletal maturity
No ankle deformity
Anticipated limb length discrepancy at skeletal maturity > 2 cm Tibial metaphyseal lengthening with diaphyseal osteotomy
Unacceptable residual tibial deformity to correct tibial deformity
No ankle deformity
Ankle valgus at supra-malleolar level (no wedging of distal tibial epiphysis) Supra-malleolar osteotomy
(in addition to treatment outlined in rows 1, 2, 3, or 4)
Ankle valgus at physeal level with wedging of the distal tibial epiphysis Medial screw epiphysiodesis of the distal tibia (in addition to
treatment outlined in rows 1, 2, 3, or 4)
a
Modified from [17]
9. Johari AN, Dhawale AA, Salaskar A, Aroojis AJ. Congenital

References postero-medial bowing of the tibia and fibula: is early surgery
worthwhile? J Pediatr Orthop B. 2010;19–6:479–86.
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tibia. J Bone Joint Surg Am. 1952;34:349–494. bowing of the tibia: a retrospective analysis of growth abnormali-
2. Heyman CH, Herndon CH. Congenital posterior angulation of the ties in the leg. J Pediatr Orthop B. 2009;18–3:120–8.
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4. Heyman CH, HerndonCH CH, Heiple KG. Congenital posterior 12. Grimes JB, Blair 3rd VP, Gilula LA. Roentgen rounds #81.
angulation of the tibia with talipes calcaneus: a long-term report of Posteromedial bowing of the tibia. Orthop Rev. 1986;15–4:249–55.
eleven patients. J Bone Joint Surg Am. 1959;41:476–88. 13. Yadav SS, Thomas S. Congenital posteromedial bowing of the
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6. Zollinger PE, Wessels MW, Wladimiroff JW, Diepstraten JA. Limb lengthening in congenital posteromedial bow of the tibia.
AFM. Prenatal ultrasonographic diagnosis of posteromedial bow- Strategies Trauma Limb Reconstr. 2012;7(3):147–53.
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15:150–3. Loder RT, Torode I, editors. Paediatric Orthopaedics—a system of
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Controversies in Blount’s Disease
27
John G. Birch
Introduction Classification
Much has been written about Blount’s disease since its pre- Separation of Blount’s disease into two distinct forms, infantile
sumed earliest description by Erlacher [1], including early and adolescent, is generally accepted. The infantile form typi-
“definitive” (and classic) works of Blount [2] (for whom cally onsets as an evolution or persistence of physiologic varus,
many have adopted his name to describe the condition) and with (usually) progressive clinical and radiographic deformity.
Langenskiöld [3] (who described the classic radiographic The Langenskiöld classification of infantile Blount’s is fre-
classification of the infantile form of the disease). Blount and quently cited (Fig. 27.1). He described six presumably progres-
Langenskiöld both described two forms of the disorder: sive radiographic stages, the mildest form characterized by
occurring in a younger population with generally greater mild medial epiphyseal and physeal irregularity, with progres-
epiphyseal and physeal distortion (infantile Blount’s disease) sively severe distortion, culminating in Stage VI. Stage VI is
and that occurring in older patients with generally less pro- characterized by complete medial physeal arrest.
nounced epiphyseal and physeal distortion (adolescent Adolescent Blount’s disease (Fig. 27.2a) is characterized
Blount’s disease). Despite having been described so long by later onset, with more subtle physeal and much less
ago, this disorder can be aptly described as an arena of con- epiphyseal distortion. Interestingly, there are neither radio-
troversy: controversy as to name, classification (by age and graphic classifications nor descriptions of the natural history
radiographic type), etiology, and treatment of all types. In of adolescent Blount’s disease during remaining growth:
this chapter, we review these controversies as well as what is steady progression of varus deformity once onset during the
known, and unknown, about Blount’s disease remainder of growth is assumed.
There is some controversy as to the existence of an inter-
mediate form of Blount’s disease (called “juvenile” by
Terminology Thompson) [4, 5]. This form is characterized as intermediate
in age of onset and radiographic severity of epiphyseal and
Usually progressive proximal tibial varus deformity in an physeal distortion. At best, this form is much less common
otherwise healthy child or adolescent was termed osteochon- that either infantile or adolescent Blount’s disease. Some
drosis deformans tibiae by Blount, and tibia vara by cases have been described as having an appearance of a
Langenskiöld. Tibia vara (infantile or adolescent) is com- “slipped proximal tibial epiphysis” with diffuse widening
monly used in the literature, while some authors prefer to and irregularity of the physis, and the impression of medial
refer to both forms as “Blount’s disease.” displacement of a relatively intact epiphysis to produce the
varus deformity [5] (Fig. 27.3).
Differential Diagnosis
The most difficult differential diagnosis for infantile Blount’s

J.G. Birch, MD, FRCS(C) (*)
disease is the distinction between persistent physiologic
Department of orthopedics, Texas Scottish Rite Hospital
for Children, 2222 Welborn Street, Dallas, TX 75219, USA varus and early infantile Blount’s disease. Some authors
e-mail: [email protected] report the efficacy of the metaphyseal-diaphyseal angle in

Fig. 27.1 Langenskiöld radiographic classification of infantile radiographic severity as advanced as Stage IV. Stage VI is characterized
Blount’s disease. Langenskiöld described six presumably progressive by complete medial proximal tibial physeal closure (bar). Reprinted
radiographic stages of deformity in infantile Blount’s disease. Stage I is from Langenskiöld A. Tibia vara. Acta Chir Scand 1952; 103:9, with
often difficult to distinguish from persistent physiologic varus. permission from John Wiley & Sons
Langenskiöld noted that spontaneous correction was possible despite
Fig. 27.2 Radiographic

appearance of adolescent
Blount’s disease. (a) The
characteristic radiographic
features of Adolescent Blount
Disease include varus deformity
of the proximal tibia and medial
proximal tibial physeal widening.
Distal femoral varus deformity
(accentuating the varus
deformity) and presumably
secondary distal tibial valgus
deformity may be present. (b)
Radiographic appearance after
growth modulation of lateral
distal femur, lateral proximal
tibia, and proximal fibular
epiphysiodesis. Note the residual
leg length inequality
27 Controversies in Blount’s Disease 505
Fig. 27.4 Anteroposterior radiograph of the left lower extremity of a

13-year-old male with poorly controlled vitamin D-resistent rickets
(VDRR). Note significant varus deformity of the distal femur, proximal
tibia, and distal tibial as well as widening and irregularity of all physes
Other disorders to consider are epiphyseal dysplasias,

Fig. 27.3 Nine-year-old boy presenting with progressive varus defor- dwarfing syndromes, thrombocytopenia-absent-radius (TAR)
mity of the left tibia. Note the “intermediate” nature of the patient by
presenting age and radiographic epiphyseal/metaphyseal distortion syndrome, and metabolic bone disease (true vitamin D defi-
(between “infantile” and “adolescent” Blount’s disease). Note the irreg- ciency, vitamin D resistant (hypophosphatemic) rickets, and
ularity of the entire proximal tibial physis. Some authors refer to such the mimicking metaphyseal dysostoses (Schmidt and Jansen
intermediate cases as “juvenile Blount’s disease” types) (Figs. 27.4 and 27.5).
providing this distinction [6–9]. The problem is compounded

by the opportunity for true infantile Blount’s disease to Natural History
resolve spontaneously (as reported originally by
Langenskiöld [3], and for presumably true persistent physi- Langenskiöld reported that spontaneous correction can occur
ologic varus to progress to infantile Blount’s disease. in infantile Blount’s disease, even occasionally in advanced
stages [3]. While this certainly seems to be true in milder
stages, our experience has been less fortunate in later stages.
Box 27.1. Differential Diagnosis of (Progressive) Varus
Thus, the short-term natural history for infantile Blount’s
Deformity in Children
disease may be to progress or resolve. The long-term natural
• Persistent physiologic varus
history is towards premature intra-articular pathology or
• True infantile Blount’s disease (resolving or
early degenerative joint disease, even when angular defor-
progressive)
mity has been addressed, presumably because of the associ-
• Renal-metabolic disorders (vitamin D deficiency,
ated epiphyseal distortion [10, 11].
renal osteodystrophy)
Interestingly, the short-term natural history of adolescent
• Vitamin D-resistant rickets (VDRR)
Blount’s disease is not known: no documentation of radio-
• Thromocytopenia absent radius (TAR) syndrome
graphic changes during growth has been reported. In general,
• Focal fibrocartilaginous dysplasia of the proximal tibia
patients appear to have a more benign long-term natural history
• Epiphyseal dysplasias (multiple; spondylo-epiphy-
with respect to degenerative arthritis [10, 12]. However, the
seal; metaphyseal dysostosis)
association of adolescent Blount’s disease with morbid obesity,
506 J.G. Birch
even in such cases from time to time, if the clinical picture

(e.g., family expectations, resistance to surgery, or similar sce-
narios) seems to warrant a trial of bracing.
Growth Modulation
Whether identifiable in the published literature or not, it is
likely that any experienced pediatric surgeon approaches
high tibial osteotomy with trepidation, based on some previ-
ous bitter or frightening experience with the major acute
complications of that surgery, specifically, compartment syn-
drome, deep infection, or peroneal nerve injury (never mind
skin irritation, challenging fixation/casting scenarios, and
recurrent deformity). In that context, the wise surgeon always
seeks a more benign treatment, and will generally recom-
mend a trial of the same, if there is any hope of effectiveness.
This is the current state of “growth modulation” in infantile
Blount’s disease [22–26]. The concept is tantalizing: implant
one of the commercially available 2-hole (or more) plates on
the proximal lateral tibia spanning the physis, for the purpose
of temporarily tethering growth in that area, thereby avoid-
ing the significant complication risks associated with high
Fig. 27.5 Anteroposterior radiograph of a 3-year-old girl with tibial and fibular osteotomy, hope to effect gradual correc-
thrombocytopenia-absent-radius (TAR) syndrome. Note the apparent tion of deformity with further growth. Peer-reviewed data
medial proximal tibial epiphyseal defect and severe resulting varus
deformity of the legs. The patient has no thumb or radius confirming the efficacy of such treatment is currently lim-
ited, but favorable, Scott et al. [24] reporting 89 % success
rate in 18 affected limbs. The likelihood of recurrence of
and the recent reports of the failure of deformity correction to deformity after correction and removal of a growth modula-
impact body mass index portend poorly for the general health tion device is not known. More esoteric questions such as
and longevity of patients with associated obesity [13–18]. lateral tether due to perichondral ring damage by disruption
of appositional epiphyseal/physeal growth by the implant in
the very young patient or by direct surgical injury; indica-
Treatment tions for concomitant epiphysiodesis of the relatively over-
grown fibula; or management of the often-associated internal
Controversies in the Treatment of Infantile tibial torsion and proximal tibial flexion deformities
Blount’s Disease (Fig. 27.6), remain to be clarified in the era of growth modu-
lation. It is clear that such devices, particularly but not exclu-
Does Bracing Work? sively canulated, titanium screws are at risk for fracture [24].
There are several studies that address the question of efficacy At a minimum, solid, stainless steel screw devices, with or
of bracing in the prevention of progression, or more impor- without more robust (i.e., “H” or similar 4-hole plates) are
tantly, the resolution of deformity in infantile Blount’s disease preferred for growth modulation in Blount’s disease. The
[19–21]. Unfortunately, these studies are retrospective, and surgeon and family must contract to regular longitudinal fol-
lack the ability to address fundamental questions such as low up to monitor growth and intervene as needed, and the
patient compliance with treatment protocols. Furthermore, family should be educated as to the possibility of implant
wear protocols (day, night, or day-and-night) also vary among breakage, and failure of this surgical modality, even in the
published studies. It is not surprising then that authors have presence of good radiographic indications and apparently
arrived at different conclusions, that is, “bracing is effective” technically adequate implant insertion. It is also not clear at
(in specific cases) or “bracing is ineffective”. We still typically what stage of successful correction in the still-growing child
prescribe long-leg, anti-varus braces in patients who are age a growth modulation plate should be removed, i.e., when the
three or less, and have radiographic Langenskiöld stage II or mechanical axis has been corrected, or after allowing the
less infantile Blount’s disease, based on our intra-institutional limb to overcorrect into some valgus. Scott et al. [24]
anecdotal and retrospective experience. We further are skepti- reported that three of eight patients with adequate follow up
cal about the efficacy of bracing in patients with bilateral after full correction and implant removal developed mild
infantile Blount’s disease, although we may prescribe them recurrent varus deformity, although none required treatment.
are several important considerations the surgeon must assess

before proceeding:
1. Is the physis still “open” medially, i.e., is the extent of
medial physeal involvement less than Langenskiöld stage
VI? If the patient has progressed to Langenskiöld stage
VI, recurrent deformity is inevitable, and an alternative or
adjunct procedure (e.g., completion of epiphysiodesis
effect and management of limb length inequality)
selected.
2. What is the current leg length discrepancy, and what’s the
plan to manage it?
3. What are the concomitant deformities, where are they,
and how severe are they? Typically, concomitant defor-
mities include distal femoral valgus (variable), proximal
tibial “procurvatum” (flexion deformity), and internal
tibial torsion. Typically, all of these should be addressed
by at least full correction (some argue for “overcorrec-
tion” in the skeletally mature patient) to unload the medial
proximal tibial epiphyseal load-bearing [30].
The surgeon has to decide on the geometry and type of
osteotomy: opening wedge, closing wedge, oblique osteoto-
mies have all be described [27–30]. In contradistinction to
adolescent Blount’s, there has been no argument for leaving
the fibula intact, presumably because the magnitude of defor-
mity, the desire to overcorrect the varus (coronal plane)
deformity, and the need to externally rotate the distal frag-
Fig. 27.6 Radiographic appearance of infantile Blount’s disease. (a) ment more or less mandate that the fibula be divided to allow
Radiographic appearance of left lower extremity. Note the epiphyseal full correction. Next to be decided is fixation: cast only, cast
distortion and varus deformity of the proximal tibia, and mild compen-
and (one or two pins), and external fixation have all been
satory valgus deformity of the distal femur and distal tibia. (b)
Radiographic appearance of the left knee, same patient. (c) Lateral used. Finally, should prophylactic anterior compartment fas-
radiograph of the knee. Note the procurvatum deformity of the proxi- ciotomy be performed? Some authors support performing a
mal tibia, and the rotational deformity of the limb limited anterior compartment fasciotomy in conjunction
with tibial osteotomy, while others make no recommenda-
tion [27–30]. At a minimum, careful and continuous
Those results and the adage of overcorrecting varus defor- evaluation of the patient for the presence of compartment
mity when high tibial osteotomy is performed in similar syndrome (untoward pain despite adequate release of con-
patients suggest that the surgeon should allow slight overcor- strictive splints or casts, or inability to dorsiflex the foot) is
rection to occur before removing the growth modulation essential. In my personal experience, partial prophylactic
plate in skeletally immature patients. anterior compartment fasciotomy neither absolutely protects
against the development of compartment syndrome, nor is it
Risks of High Tibial Osteotomy and Acute an entirely innocuous procedure: patients may complain of
Deformity Correction muscle herniation, discomfort, weakness, or suffer cutane-
High tibial (below the tibial tubercle) osteotomy of the tibia ous nerve injury. The surgeon must carefully weigh his or her
with concomitant osteotomy of the fibula to “over-correct” strategy in the context of these risks.
the varus deformity is the presumptive “gold standard” man-
agement of infantile Blount’s disease [27–30]. Typically, the Effectiveness of Physeal Arrest Resection
distal fragment is externally rotated as well (Fig. 27.7a, b). Studies have demonstrated that at least in some cases of
This operation has serious risks, the most important of which infantile Blount’s disease (approximately 50 % in our experi-
are compartment syndrome and direct or indirect injury to ence), medial proximal tibial physeal growth may be restored
the peroneal nerve from manipulation, traction, or entrap- by a variation of Langenskiöld’s physeal arrest resection sur-
ment within fascial planes during manipulation of the frag- gery [31, 32], usually combined with high (below the physis)
ments (Fig. 27.8). Soft tissue irritation, deep infection, and osteotomy. This procedure is neither universally accepted
recurrent deformity are also not inconsequential risks. There nor successful. Patients with Blount’s disease typically have
508 J.G. Birch
Fig. 27.7 Three-year-old girl with progressive infantile Blount’s disease restoration of the tibial mechanical axis. The distal fragment is manipu-
of the left leg, Langenskiöld stage III, who failed conservative treatment lated to correct both varus and internal rotational deformities associated
with an anti-varus brace. (a) Preoperative clinical appearance. (b) with infantile Blount’s disease. (d) Radiographic results 2 years postop-
Preoperative radiographic appearance. (c) Postoperative radiographic eratively, age 5 years. Patient has symmetric valgus deformity, with
appearance. Note the lateral translation of the distal fragment to effect improvement of epiphyseal distortion of the left proximal tibia
criterion for consideration of physeal bar resection as poten-

tially indicated. Our experience is that even patients who
experience resumption of growth will subsequently develop
late deceleration/cessation of that growth. Embedding metal-
lic markers or some other method of exact determination of
growth persisting is important to allow prompt recognition
of secondary cessation of growth (Fig. 27.9a, b). Completion
of the epiphysiodesis at that time can save the patient from
the development of recurrent deformity requiring an addi-
tional osteotomy, and appropriate management of any asso-
ciated leg length inequality.
Utility of Physeal Arrest Resection Surgery

in the Absence of a Bony Physeal Arrest
Andrade and Johnston [34] have recommended “physeal bar
Fig. 27.8 Peroneal nerve compression detected and released in the resection” surgery in patients with advanced infantile
immediate postoperative period. The patient developed peroneal nerve Blount’s disease, but at Langenskiöld stage less than VI, i.e.,
palsy shortly after surgery. Her compartments were soft. At exploration, without demonstrable frank physeal bony bar formation on
the nerve was found to be compressed under a fascial band as it passed advanced imaging. They report good (>80 % successful in
into the deep anterior compartment. Arrow indicates contused peroneal
nerve after release of the fascial band. Same patient as Fig. 27.7 preventing recurrence of varus deformity, in patients less
than 7 years of age) results, but there is not a comparable
group treated by high tibial osteotomy alone to confirm the
an advanced bone age [33]. The wise surgeon will assess the relative efficacy of this procedure.
patient’s bone age and the adage of “average” proximal tibial
physeal growth of 6 mm per year in the healthy proximal What to Do with the “Failed” Case?
tibial physis to determine if adequate growth remains to war- Usually, the surgeon is faced with many things to consider,
rant an effort at this procedure. A minimum of 4 years of when the patient has undergone previous high tibial osteot-
growth remaining would seem to be a reasonable threshold omy with recurrent deformity. These include: the extent of
Fig. 27.9 This 6-year, 9-month

old girl underwent third-time
repeat hight tibial osteotomy,
combined with partial physeal
bar excision for recurrent
infantile Blount’s disease of the
left tibia. (a) Four months
postoperatively, the patient has
persistent iatrogenic valgus
deformity at the osteotomy site.
Note the metallic markers in the
proximal medial tibial epiphysis
and metaphysis. (b) Sixteen
months postoperatively (1 year
after radiograph in part a), the
patient has persistent valgus
deformity. The markers have
separated by approximately
20 mm
lower extremity scarring, recurrent (potentially complex) Is There a Role of “Hemi-Plateau

deformity, including variable amount of varus, procurvatum, Elevation”?
and internal tibial torsion, leg length inequality in unilateral Radiographs in the advanced stage of infantile Blount’s
cases, and potential continued asymmetric growth in the leave the observer with the distinct impression of a “sag-
skeletally immature patient (both within the tibia due to the ging” or “depressed” medial tibial plateau. This perception
medial arrest, and between the affected and unaffected legs). (or reality) has led a number of authors to describe “hemi-
There can be no “cookbook” strategy in such cases plateau elevation” [36–38] (in effect, an intra-articular frac-
(Fig. 27.10). The surgeon must individualize the correction ture with restoration of the transverse condylar relationships
plan with careful consideration of all these parameters in the [unless the surgeon is fortunate enough to effect cartilagi-
development of a treatment strategy suitable and acceptable nous deformation of the articular surface without fracture]).
to the family and their circumstances. External fixation, with It should be noted that some other authors dispute at least
either acute or gradual correction, provides the surgeon with the universal presence of a true depression of the medial
an excellent tool to manage all of these deformities during plateau, and confirmed the presence of a cartilaginous
one treatment stage, if acceptable to the patient and family. medial tibial plateau by knee arthrogram [39], or more
The surgeon must always keep in mind the fact that patients recently, MRI [35, 40]. The procedure of hemi-plateau ele-
with infantile Blount’s disease may have premature or “atrau- vation has been described in isolation or in conjunction with
matic” meniscal tears or other degenerative changes, even in second osteotomy of the tibia to correct coexisting varus
the presence of presumably adequate angular and rotational deformity. The surgeon should recognize the most likely
deformity correction [10, 35]. This risk is presumably the event of intra-articular fracture, and the inevitability of no
result from the epiphyseal distortion that is unique to infantile further (favorable) physeal growth, if performing this sur-
Blount’s disease and which distinguishes it from adolescent gery; thus, completion of the proximal tibial and fibular epi-
Blount’s disease. Thus, if the patient has significant intra- physiodesis at the time of surgery would seem appropriate.
articular complaints or abnormalities on physical examina- We have not, in our institutional experience, used this proce-
tion, appropriate pursuit of the potential for these structural dure to manage even the most severe cases of infantile
abnormalities (MRI or arthroscopy) should be carried out. Blount’s disease.
510 J.G. Birch
Fig. 27.10 Patient with failed previous treatment of infantile Blount’s the previous valgus-producing osteotomy. (b) Clinical appearance. (c)
disease. (a) Radiographic appearance. The patient has had several prior Clinical appearance during staged treatment. The patient was managed
surgical procedures, including high tibial osteotomies and attempted by staged surgical procedures, including (partial) implant removal, and
lateral proximal tibial physeal arrest with retained implants, recurrent two-level osteotomies of the tibiae with gradual correction of the varus
varus deformity, and post-surgical proximal metaphyseal valgus defor- and valgus deformities using a circular external fixator. (d) Final post-
mity. Note the medial translation of the patient’s mechanical axis operative radiographic appearance. (e) Final clinical appearance
caused by a failure to lateralize the distal fragment in conjunction with
Box 27.2. Controversies in Infantile Blount’s Disease Box 27.3. Controversies in Adolescent Blount’s Disease
• Etiology, including obesity • Etiology, including obesity
• Differentiation between persistent physiologic • Role of growth modulation in surgical armamentarium
varus and true infantile Blount’s disease • Indications for correction of associated deformities
• Efficacy of lower extremity bracing (distal femoral varus, distal tibial valgus)
• Efficacy, timing, and time-to-removal of growth • Gradual versus acute correction by high tibial oste-
modulation plates for surgical correction otomy, with or without external fixation
No specific useful radiographic classification exists for

Adolescent Blount’s Disease adolescent Blount’s disease, either for morphological char-
acterization or prognostic purposes. The “classic” deformity
While adolescent Blount’s disease was described by both consists of proximal tibial varus deformity of the affected
Langenskiöld and Blount in their original series, side by side extremity with a medial proximal tibial physeal widening or
with infantile Blount’s disease, it is clear that adolescent “zone of injury” on radiographs (see Fig. 27.2). Biopsy
Blount’s disease is a completely distinct disorder. Not only specimens from this area of radiographic physeal lucency
do infantile and adolescent Blount’s diseases affect an show disorganized microscopic physeal architecture, and/or
entirely different age group, the radiographic features, nature replacement of normal physis by fibrous tissue. It is assumed
and location of concomitant ipsilateral limb deformities, that some patient risk factor (familial, persistent mild physi-
incidence of intra-articular abnormalities, and published ologic varus, subclinical metabolic abnormality) with or
long term premature degenerative arthritis prevalence are all without obesity results increased compressive forces across
distinctly different as well [10–12]. Furthermore, while obe- the physis with disruption of normal function and decelera-
sity seems to be a risk factor for both conditions, obesity tion of growth [4, 41, 42]. There are no short-term natural
seems to be more specifically related to adolescent Blount’s history reports to confirm the presumption of progressive
disease [13–16, 18]. worsening varus deformity or radiographic abnormality in
untreated patients during the remainder of growth. Setting correction will result in increased physical activity and
aside general health issues related to obesity, with or without subsequent weight reduction (Fig. 27.11). This calls into
persistent varus deformity of the leg, published reports sug- question what our responsibility as surgeons should be: while
gest a more benign natural history with respect to degenera- we console ourselves that deformity correction will open the
tive arthritis of the knee in patients with adolescent Blount’s door to a healthier life-style and thus represents a noble pur-
disease compared to infantile [10–12]. suit on behalf of the patient, warranting tolerance of myriad
increased challenges and complications associated with per-
forming angular deformity correction in patients with morbid
Treatment Considerations in Adolescent obesity (anesthesia risks, sleep apnea, wound complications,
Blount’s Disease deep infection, fixation challenges, and greater difficulty
effecting complete deformity correction). Furthermore, there
Role of Obesity and Impact of Treatment is no clear evidence that correcting angular deformity associ-
on Obesity ated with Blount’s disease increases the longevity of the knee
in affected extremities. Thus, as patient health advocates, we
It is well recognized that obesity in the general population is must seriously question the merit of surgical treatment for
increasing and represents a significant health problem. Sadly, adolescent Blount’s disease until an effective weight man-
that increased incidence is extending into the adolescent and agement program has been embraced by the patient.
now pediatric population [14–16, 43–45] with significant In the overall picture, probably the most important associ-
implications for long-term health consequences. It has been ated abnormality in patients with adolescent Blount’s disease
long noted that patients with adolescent Blount’s disease are is morbid obesity, because not only is it a threat to the
frequently (but not universally) obese or morbidly obese, and patient’s overall health, its presence results in specific poten-
obesity, rightly or wrongly, is considered a risk factor for the tial for sleep apnea, type II diabetes, and increased risks of
development of adolescent Blount’s disease. Interestingly, delayed wound healing, deep infection, and myriad soft tis-
there are no reliable population surveys that confirm an sue challenges in the perioperative period. In addition,
increased incidence of adolescent Blount’s disease in asso- patients with obesity will mask significant varus deformity
ciation with the increased prevalence of adolescent obesity in (indeed, the “fat-thigh syndrome” as described by Davids
the general population. Soberingly, Sabharwal et al. [17] [46] is a potential cause of adolescent Blount’s: the need to
have noted that correction of angular deformity (both of stand with the legs separated to accommodate fat thighs
infantile and adolescent Blount’s disease) is NOT associated results in the skeletally immature a secondary adaption of
with subsequent reduction in BMI in their study population, medicalization of the lower leg to bring the feet closer to the
thus calling into question the myth or circular argument that midline) (Fig. 27.12). One of the consequences is that “full”
leg deformity begets pain and exercise intolerance, with the correction of the varus deformity will typically make the
implicit expectation of surgeon and patient that deformity patient appear to have valgus deformity (Fig. 27.13), which
Fig. 27.11 Recurrent infantile

Blount’s disease in an obese
child. (a) At age 8, this patient
had progressive, recurrent
infantile Blount’s disease
associated with obesity. (b) At
age 15, the patient’s deformities
have recurred after high tibial
osteotomy, but through a
self-directed program of diet and
exercise, the patient lost weight
despite the leg deformities, and at
this time, weighs less than he did
at age 8
512 J.G. Birch
is usually cosmetically displeasing to the family/patient.

This puts the surgeon in a vexing situation of either inadver-
tently or deliberately undercorrecting varus deformity, or
justifying the production of deformity in the patient’s per-
ception. There is no easy answer here, but we usually prefer
to correct the deformity anatomically (as best we can),
explaining the outcome and its rationale in advance and in
the (perhaps unlikely) hopes that the patient will lose weight
in the future. The surgeon should educate the patient and the
family regarding the presence of associated deformities, and
be prepared to address them, should the need arise.
Associated Deformities
In contradistinction to infantile Blount’s disease, ipsilateral

deformities of the distal femur and distal tibia are frequent
(see Figs. 27.2a and 27.13) [47–50]. Interestingly, the most
Fig. 27.12 Patient with adolescent Blount’s disease and morbid obe-
common associated deformity of the distal femur is an
sity. The “fat thighs” require the patient to stand with the legs relatively
abducted. Some conjecture that this posture in an adolescent may be the accentuating varus, in contradistinction to some cases of
cause of adolescent Blount’s disease, at least in some patients infantile Blount’s disease and many traumatic physeal
Fig. 27.13 Apparent valgus

“deformity” after correction of
adolescent Blount’s disease varus
deformity in the left leg of an
obese patient. (a) Preoperative
radiographic appearance. (b)
Postoperative radiographic
appearance. Note the incomplete
correction of the varus deformity.
(c) Postoperative clinical
appearance. Note that despite
incomplete correction of the
varus deformity radiographically,
the patient appears to have valgus
deformity clinically
Finally, similarly to infantile Blount’s disease, patients

will usually be noted to have an internal tibial torsional
deformity on clinical examination, and on careful radio-
graphic assessment, “procurvatum” (flexion deformity) of
the proximal tibia [50]. The severity of these deformities
must be taken into consideration in determining the best sur-
gical treatment strategy for individual patients.
Growth Modulation
Open hemi-epiphysiodesis of the lateral proximal tibia, with

or without concomitant epiphysiodesis of the proximal fibula
and/or lateral femur was the first described method of
“growth modulation” of this deformity [51]. It is interesting
to note that in at least some cases, this procedure is effective
despite the persistence of presumed “causative roles” of
deformity and obesity in the disruption/deceleration of nor-
mal medial proximal tibial physeal growth. Considering this
procedure with acceptance of an uncertain outcome is almost
universally motivated by the challenges and risks created by
osteotomy, particularly in the obese patient. Open hemi-
epiphysiodesis has been replaced by plate (or staple) growth
modulation methods [25, 52, 53] (see Fig. 27.2b). Enthusiasm
for the original “8” plate (titanium cannulated screws and
2-hole plate) has waned in favor of stronger non-cannulated
Fig. 27.14 Apparently healthy adolescent with left-sided varus deformity stainless steel screws, and/or more robust “H” or similar
typical of adolescent Blount’s disease, developed apparently idiopathic plates (i.e., two screws on either side of the physis) due to
contralateral valgus deformity (“windswept” deformity). There was no
reports of screw fracture with the original device [22, 23], an
identifiable endocrine or skeletal dysplasia abnormality in this patient
event which seems to be nearly unique to the metaphyseal
screws in Blount’s patients (particularly adolescent type).
growth disturbances which often develop at least a partial Both the surgeon and patient must accept that even apparently
compensating reverse deformity at the adjacent physes. This technically adequate implant placement and retention do not
suggests a more global etiology (rather than simple decelera- guarantee effective correction of the angular deformity. For
tion of growth of the medial proximal tibial physes or unrec- this reason, and for the happy possibility of correction prior
ognized endocrinopathy as the underlying cause or at least to skeletal maturity, both the patient and surgeon must com-
accelerant of adolescent Blount’s disease. Along those lines, mit to regular longitudinal follow up to prevent overcorrec-
interestingly, several patients we have treated have devel- tion, and manage failure to correct appropriately.
oped a contralateral valgus deformity (i.e., “windswept”
appearance) during the course of treatment for presumptive
adolescent Blount’s disease (Fig. 27.14), in the absence of an Acute Correction with Internal Fixation
identifiable metabolic disorder or endocrinopathy. This too,
would suggest a systemic component to the etiology of ado- High tibial osteotomy (either above or below the tibial tuber-
lescent tibia vara, at least in some patients. cle, at the surgeon’s preference, because the patient is nearly
Distal tibial valgus (which would seem a more intui- skeletally mature) can be opening wedge, closing wedge, or
tively logical consequence of proximal varus deformity) is a combination at surgeon’s preference. Due to the average
often noted in patients with adolescent Blount’s disease. patient’s age, size, weight and body habitus, rarely is pin
“Comprehensive correction” strategies [47] describe surgi- fixation and cast immobilization practical or effective. The
cal correction of this deformity. However, it has been our surgeon is therefore left with the decision of internal fixation
experience that patients rarely complain of ankle pain or or external fixation, the latter of course then subjecting all to
deformity, and only rarely has primary or secondary correc- the inconvenience, pin care, and increased soft tissue irrita-
tion of this deformity been necessary in our patient tion of such patients. Internal fixation is typically of some
population. plate device. Some authors have noted that fibular osteotomy
514 J.G. Birch
is not necessary if modest opening-wedge correction strat-

egy is used [54]. Does Juvenile Blount’s Disease Exist?
The patient is at risk for wound dehiscence, deep wound
infection, delayed union, non-union, implant failure, com- Some authors [4, 5] note a third, intermediate type of Blount’s
partment syndrome, inadequate correction, and the inability disease, intermediate in time of onset (between infancy and
to adjust correction postoperatively (without revision sur- adolescence) and radiographic deformity (having less epiph-
gery); some of these complications are inherent to tibial oste- yseal distortion typical of infantile Blount’s, but more exten-
otomy and the patient’s body habitus, and some are risks sive physeal changes than seen in classical adolescent
associated with acute correction and internal fixation strat- Blount’s). We have a few cases which fit this description, and
egy: the surgeon and patient should be cognizant of these whom do not neatly fit either “traditional” category, and thus
risks and deem them acceptable in context. believe this is a separate entity. Treatment decisions, how-
ever, remain the same as for either condition, except that
conservative treatment (observation or bracing) would seem
Correction with External Fixation to be unhelpful modalities. There are no reports or personal
(Acute or Gradual) (with 6-Strut experience with growth modulation strategies in such
Fixators/Others) patients to our knowledge.
Gradual correction of adolecent tibia vara [54–63] offers

several theoretical and practical advantages: there is presum- Summary
ably a lower risk of compartment syndrome and deep infec-
tion, greater deformities can be addressed more precisely, Infantile and adolescent Blount’s diseases must be consid-
lengthening can be achieved, it is easier by fragment transla- ered separate and distinct entities, occurring in a dissimilar
tion to effect a truly anatomic restoration of limb alignment, patient population, at different ages, with entirely different
and the complex deformity (with procurvatum, internal tibial radiographic features, concomitant deformities, and, likely
torsion, with or without shortening) can be addressed, either natural history. What they share are a lack of our understand-
acutely, gradually, or sequentially. For smaller deformities ing as to their etiology, and (presumably) progressive, com-
with minimal lengthening required, the fibula may remain plex varus deformity of the tibia, which challenges the
intact and unfixed (at least proximally). Six-strut (hexapod surgeon to create an effective treatment plan. Gradual correc-
or Taylor Spatial Frame™) are particularly valuable for this tion of these deformities by growth modulation or by oste-
deformity, although traditional circular fixators or monolat- otomy and external fixation, with or without lengthening, are
eral fixators are preferred by some authors. important and valuable surgical correction techniques that
The flexibility of external fixation devices and strategy should be in the surgeon’s armamentarium. Many aspects of
however, have to be weighed against the generally more pro- infantile and adolescent Blount’s disease warrant attention
longed postoperative recovery and management of the device by further research, including identification of their etiolo-
and the patient in the device, particularly with the increased gies, favorably influencing childhood obestity, unequivo-
soft tissue irritation in this specific patient population [63]. cally determining the effectiveness of bracing in the infantile
form, developing an effective growth modulation method of
correcting the associated internal tibial torsional deformity,
Adolescent Blount’s Disease Summary and determining an appropriate threshold for correcting con-
comitant deformities of the distal femur and distal tibia.
Patients with adolescent Blount’s represent a significant
challenge to the clinician because of the complex nature of
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sary? J Child Orthop. 2008;2(3):199–204. 60. Gilbody J, Thomas G, Ho K. Acute versus gradual correction of
55. Clarke SE, McCarthy JJ, Davidson RS. Treatment of Blount dis- idiopathic tibia vara in children: a systematic review. J Pediatr
ease: a comparison between the multiaxial correction system and Orthop. 2009;29(2):110–4.
other external fixators. J Pediatr Orthop. 2009;29(2):103–9. 61. Price CT, Scott DS, Greenberg DA. Dynamic axial external fixation
56. Coogan PG, Fox JA, Fitch RD. Treatment of adolescent Blount dis- in the surgical treatment of tibia vara. J Pediatr Orthop.
ease with the circular external fixation device and distraction osteo- 1995;15(2):236–43.
genesis. J Pediatr Orthop. 1996;16(4):450–4. 62. Rozbruch SR, Blyakher A, Haas SB, Hotchkiss R. Correction of
57. Feldman DS, Madan SS, Koval KJ, van Bosse HJ, Bazzi J, Lehman large bilateral tibia vara with the Ilizarov method. J Knee Surg.
WB. Correction of tibia vara with six-axis deformity analysis and 2003;16(1):34–7.
the Taylor Spatial Frame. J Pediatr Orthop. 2003;23(3):387–91. 63. Wilson NA, Scherl SA, Cramer KE. Complications of high tibial
58. Feldman DS, Madan SS, Ruchelsman DE, Sala DA, Lehman osteotomy with external fixation in adolescent Blount’s disease.
WB. Accuracy of correction of tibia vara: acute versus gradual cor- Orthopedics. 2007;30(10):848–52.
rection. J Pediatr Orthop. 2006;26(6):794–8.
Part V
Sequelae and Complications
Methods to Enhance Bone Formation
in Distraction Osteogenesis 28
Hae-Ryong Song, Dong Hoon Lee, Seung-Ju Kim,
and Ashok Kumar Ramanathan
including donor site morbidity and blood loss. To overcome

Introduction these drawbacks, tissue engineering strategies have demon-
strated a potential role in developing bone graft substitutes.
Regeneration of bone defects resulting from conditions Different mechanical and biophysical stimuli could provide
including trauma and congenital deformity correction by dis- effective augmentation of bone regenerate maturation. These
traction osteogenesis (DO) is one of the most interesting include biologic stimulation such as bone morphogenetic
issues in the field of regenerative medicine [1]. DO is an proteins (BMP), platelet-rich plasma (PRP), stem cells, para-
increasingly popular technique used to stimulate new bone thyroid hormone, and growth hormone [5–7]. In addition,
formation to treat orthopedic disorders resulting from bone physical stimulation such as ultrasound, mechanical, and
defects and deficits [2]. However, the long treatment period electromagnetic stimulation has been used [8–11]. Even
with various complications [3] and the potential for nonunion though a bone defect during DO possesses the intrinsic
under some circumstances remain major limitations of cur- capacity to heal spontaneously following surgery, beyond a
rent treatment procedures. Regeneration of bone in the pres- certain critical size defect bone may not heal adequately by
ence of rigid fixation and maintenance of the osteogenic itself and further intervention is often required.
tissue (marrow, endosteum, nutrient artery, and periosteum)
require many factors for stimulation of bone regeneration [4].
Therefore, the development of procedures to accelerate the Box 28.1 Quantitative Methods to Monitor the
formation and maturation of regenerate is clearly desirable. Regenerate Bone
To regenerate the bone defects, autologous and allogenic • Quantitative ultrasound
bone grafting are the most widely used methods. However, • Dual-energy X-ray absorptiometry
these methods are associated with a number of drawbacks, • Quantitative computed tomography
• Pixel value ratio method
H.-R. Song, MD, PhD (*)
Department of Orthopedic Surgery, Korea University Medical
Center, Guro Hospital, #80, Guro-Dong, Guro-Gu, Seoul 152703,
Republic of Korea Monitoring of Regenerate Bone
D.H. Lee, MD, PhD Various methods have been proposed for the monitoring of
Department of Orthopedic Surgery, Severance Children’s Hospital, the regenerate bone [12] quantitatively and qualitatively.
134, Sinchon-Dong, Seodaemun-gu, Seoul 120752,
Republic of Korea Indirect quantitative methods, including ultrasound, dual-
e-mail: [email protected] energy X-ray absorptiometry (DEXA), and quantitative com-
S.-J. Kim, MD, PhD puted tomography, have already been found to be sensitive
Department of Orthopedic Surgery, KEPCO Medical Center, [13–15]. However, owing to its high cost, inability to detect
308, Uicheon-ro, Dobong-Gu, Seoul 132703, Republic of Korea soft tissue changes, and poor measurements in the presence of
e-mail: [email protected] artifacts, the routine use of such modalities in clinical practice
A.K. Ramanathan, MBBS, MS(Ortho) is not popular. An image analysis method is also proposed for
Department of Orthopedic Surgery, Madurai Medical College, the monitoring of the regeneration of the bone. Radiographic
1/c, S.Kodikulam North Main Street, K.Pudur, Madurai,
Tamil Nadu 625007, India investigation is one of the most important methods because it
e-mail: [email protected] is the simplest; it provides continuous information and it can

520 H.-R. Song et al.
be easily interpreted. A recent approach that is being used to compression and distraction at an amplitude of 1 mm/day for
determine callus stiffness is to evaluate the pixel value ratio 3 weeks, and observed improved bone healing in terms of
(PVR) using a picture archiving and communication system cortical thickness, cortical:cancellous bone ratio, and mineral
(PACS). The PVR is the ratio of the proximal segment to that apposition. In contrast, Greenwald et al. [33], in an animal
of the regenerate, and it correlates well with the bone mineral model of mandibular distraction, distracted to a length of
density (BMD) [16, 17]. Shim et al. [18] analyzed serial pixel 2.5 mm (at a rate of 0.25 mm twice daily) for 5 days, then
values of different cortices in both femoral and tibial length- compressed 1.0 mm for a 2-day period, and redistracted to a
ening and demonstrated that pixel values for distraction callus length of 5 mm. They demonstrated no significant differences
conformed to a sigmoidal curve. Song et al. [19] reported that between experimental (“pumping”) and control group in
BMD measurement can thus function as an effective adjunct radiological and histological parameter. The timing of oscil-
to measure callus stiffness, along with PVR, using digital lating motion is also a conflicting issue. Kassis et al. [35]
radiographs, especially in situations where callus maturation showed that the effect of axial micromotion is effective only
and stiffness are doubtful. after the end of elongation rather than during active distrac-
Many studies [20–22] have been published in an attempt tion. Further investigations are necessary to establish the
to find a relation between the callus pattern and the prognosis effect and the optimal protocol for “pumping” including the
of the limb lengthening procedure because such a system or timing, amplitude of sequential movement, and frequency.
classification can help the surgeon to quantitatively assess Rhythm of distraction is another conflicting issue for
the progress of the distraction callus and to predict the chance enhancing bone regenerate. Ilizarov in an animal model
of callus fracture and can thus impact the clinical outcome of demonstrated better callus in motorized distraction group
the procedure. Devmurari et al. [23] reported that the Ru Li (1/60 mm, 60 times/day) compared with the standard
classification [24] is an effective method for the evaluation of rhythm (1/4 mm, 4 times/day) [36, 37]. Mizuta proved that
the chance of callus fracture and a lucent pathway was seen an increase in the distraction frequency (eight steps vs. four
in all fracture cases with concave, lateral, and other shapes steps) in open-wedge osteotomies of the proximal tibia
(Fig. 28.1). Early removal of the fixator may lead to fracture with hemicallotasis provides better bone formation, result-
of the regenerate, whereas prolonged use leads to joint stiff- ing in a shorter external fixation period [38]. Meanwhile,
ness, pin-site infection, and discomfort to the patient, as well there have been reports that the rhythm of distraction does
as delayed complications like subsidence and angulation not significantly influence bone healing [39, 40].
[25, 26]. Research is in progress to define callus maturation Lengthening procedure is not confined to the bone and soft
and corticalization and hence to identify the ideal time for tissues are also important. Makarov et al. demonstrated in a
removal of the fixator. goat model that a more fractionated rhythm of distraction
was associated with enhanced preservation of muscle fibers
and greater regenerative activity of the muscle [41]. Further
Physical Stimulation investigation is necessary to clarify the benefit of the more
fractionated distraction, and the optimal rhythm.
Mechanical Stimulation
Controlled mechanical stimulation is considered to be an Low-Intensity Pulsed Ultrasound

important stimulus for the regenerate in distraction osteo-
genesis. There are preclinical and clinical evidences that Low-intensity pulsed ultrasound (LIPUS) is a form of
physiologic weight bearing stimulates new bone formation mechanical stimulation that is delivered as high-frequency
and consolidation of new bone in distraction osteogenesis acoustic pressure waves. The physical process through which
[27–30]. Controlled compressive but not shear force has LIPUS stimulates bone healing remains unclear. It is specu-
been shown to affect the biologic nature of the healing lated that ultrasound enhances fracture healing by inducing
response by early expression of BMP 2/4 and the messengers low-level mechanical forces at the fracture site, reproducing
for collagen 1 and osteocalcin [31] and increase the size and the effect of functional loading [42]. The mechanisms
strength of the regenerate [30, 32]. through which the mechanical signal is translated into a bio-
“Pumping” of the regenerate (also referred to as the accor- chemical signal have not been fully understood but ultra-
dion maneuver)—the procedure of alternative compression sound seems to influence certain cellular reactions involved
and distraction—is a well-known method to stimulate new in each phase of the healing process such as inflammatory
bone formation in the clinical situation of poor regenerate reaction, angiogenesis, chondrogenesis, intramembranous
[33, 34]. But, from the literature review, we found that it is ossification, endochondral ossification, and bone remod-
still a conflicting issue. Mofid et al. [34], in an animal model eling [42, 43]. LIPUS is one of the best studied treatment
of mandibular distraction, performed daily alternative modalities in distraction osteogenesis, but the results from
28 Methods to Enhance Bone Formation in Distraction Osteogenesis 521
Fig. 28.1 Callus patterns
preclinical experiments are still conflicting regarding its [44, 45, 52, 53]. Further well-designed studies are required
efficacy [44, 45]. There are well-designed clinical studies to clarify the role of LIPUS in humans undergoing DO.
for the acute fracture healing, suggesting that LIPUS can
be beneficial in the treatment of certain non-operatively
treated fracture by shortening the time to radiological and Pulsed Electromagnetic Field
clinical union [46–48]. Most successful reports with
LIPUS are in the treatment of conservatively managed Most of the available studies regarding pulsed electromagnetic
acute fractures. There are only scanty evidences for surgi- field (PEMF) are related to its effect on the treatment of acute
cally treated fracture and most of them reported disap- fractures [54–56] and suggest that PEMF may be beneficial in
pointing results [49–51]. Emami et al. concluded that the treatment of certain non-operatively treated fractures.
low-intensity ultrasound treatment did not shorten healing However, there are only a few reports about its effect on dis-
time in acute tibial fractures treated with a reamed and traction osteogenesis [11, 56–58]. Though these studies are
statically locked intramedullary nail [49]. reporting supportive effect on enhancing callus healing, fur-
The effect of LIPUS on distraction osteogenesis is not ther high-level studies in human subjects are necessary before
conclusive especially regarding the timing of application we can recommend PEMF as a valid treatment option.
Jamshidi vacuum aspiration is minimally invasive and has

Box 28.2 Biologic Stimulants low donor-site morbidity. Lastly, we are not aware of any
• Local application reports of malignant transformation of autologous BMCs in
– BMP the literature [77]. Though a lot of animal studies using bone
– BMAC/PRP marrow transplant have shown that bone marrow has enriched
– Stem cells osteoblast progenitor cells and it is a valuable bone graft in
– Experimental—TP508, ED71, FGF-2 bone defect, there have been only limited investigations on its
• Systemic application application in distraction osteogenesis [78, 79].
– Parathyroid hormone The PRP is abundant in various growth factors released
– Growth hormone from platelets, which have been known to increase vascular
– Experimental—hyperbaric oxygen, androgens ingrowth and mitogenic effects on bone-forming cells [80–83].
Kawasumi et al. [82] observed that a high platelet concentra-
tion combined with osteoblastic cells in PRP accelerated new
bone formation during distraction osteogenesis in a rat model.
Biologic Stimulation In contrast to these findings, Arpornmaeklong et al. [84] noted
an inhibitory effect of PRP on osteogenic differentiation of
Local Application-Bone Morphogenetic marrow-derived pre-osteoblasts.
Protein There have been few clinical studies using PRP in human
subjects [85–87]. Latalski et al. [85] reported a shorter heal-
The BMPs are known to have a strong osteoinductive poten- ing index in the PRP group in a retrospective human DO
tial [59–61]. There are several preclinical studies on the effect study with a small sample size. The role of PRP in bone
of BMPs on distraction osteogenesis [5, 62–65]. Mizumoto regeneration is still a controversial issue and its mechanism
et al. [65] used a single injection of rhBMP-7 into osteotomy is not yet fully understood [84, 85, 88–98]. BMCs and PRP
gap on the day of surgery in a rat DO model and observed a have been used simultaneously showing favorable clinical
larger amount of new bone throughout the distraction phase. results. Kitoh et al. [78, 99] studied transplantation of
Hamdy et al. showed that application of OP-1 early during the culture-expanded bone marrow cells combined with PRP
distraction phase in rabbit tibiae accelerated new bone forma- that accelerated new bone formation during the distraction
tion by densitometry and micro CT analysis [62, 64]. Also, osteogenesis, especially in the femur.
Song et al. [5] demonstrated enhanced bone formation by Lee et al. [86] showed that the combined injection of bone
injection of BMP-2 at the end of distraction in rat femora. marrow aspirate concentrate (BMAC) and PRP during surgery
Several clinical studies have been reported using BMP-2 or enhanced bone regeneration in human tibial distraction osteo-
BMP-7/OP-1 for open tibial fractures and spinal fusion [66, genesis (Fig. 28.2). The difference in the abovementioned
67], but not for distraction osteogenesis so far. There are con- studies conducted by Kitoh et al. and Lee et al. lies in how to
flicting issues about the risk of cancer by using rhBMP-2 process BMC-culture expansion or concentration. Jäger et al.
[68–73]. TGF-beta ligand is known to have the ability to alter [77] described the strength of the concentration compared
cell signaling that can lead to tumor promotion, and certain with culture-expanded method. An immediate autologous
BMPs are expressed during the growth of some types of can- transplantation of bone marrow concentrate can prevent com-
cer [68, 69, 71, 73–76]. Further investigations for optimal plications related to the reduced quality of the transplanted
dose, optimal timing, and potential risk of various BMPs are cells such as pre-aging due to telomere shortening, reduced
required before they can be recommended for clinical use. viability, or dedifferentiation/reprogramming that is associ-
ated with in vitro cultivation. The risk of infection is reduced
by decreasing the ex vivo time period; and injection can be
Bone Marrow Cells/Platelet-Rich Plasma performed by single-stage surgery. Although there is only lim-
ited clinical evidence, BMAC and/or PRP seems to be a prom-
Osteogenic differentiation from bone marrow-derived mesen- ising treatment option. Though all these studies show a
chymal stem cells (MSC) has several advantages when com- favorable prophylactic role of BMAC/PRP in enhancing the
pared to the differentiation of other mesenchyme tissues [77]. regenerate in DO, their role in established delayed/nonunion
First, bone marrow cells (BMCs) have abundant mononuclear after DO is uncertain and warrant further studies.
cells. Second, the differentiation of osteoblasts from BMCs is Well-designed preclinical and clinical studies are neces-
well described and standardized. Third, due to the autogenous sary, especially to evaluate whether BMAC or PRP contrib-
properties the complications such as infection, immunogenic utes more to bone formation—or if the effect is synergistic,
reactions, and disease transmissions can be minimized. the optimal timing of injections and the optimal concentra-
Fourth, the harvesting process of bone marrow cells using a tions and cell counts.
Fig. 28.2 Serial anteroposterior plain radiographs (postoperative an injection group, showing postoperative 1- to 5-month states with
1–5 months, monthly) of the tibia in a patient undergoing lengthen- an increment of 1 month. Images in group B (non-injection group)
ing over nail. More callus regeneration was seen in the injection were from a patient in a control group with the same postoperative
group (group A). Images in group A were obtained from a patient in months above
Stem Cells factors including platelet-derived growth factor, insulin-

like growth factor, and transforming growth factors. PRP
There have been many debates about using biotechnology on contains all these growth factors [105]. PRP can also be a
fracture healing but there is little scientific evidence about the suitable carrier for cell transplantation because it coagu-
positive effect of tissue engineering on osteogenesis. Tissue lates immediately by an addition of thrombin and calcium.
engineering combines osteogenic bone marrow mesenchymal Combination of stem cells and PRP accelerates bone
stem cells, synthetic scaffolds, and growth factors in order to healing and bone remodeling process by stimulating angio-
form hybrid constructs. Stem cells have been used for other genesis [106].
indications including the treatment of long bone defects [100], In a study conducted by Kitoh et al. [99] in 46 patients of
fracture healing [101], and avascular necrosis [102] apart from skeletal dysplasia and limb length discrepancy, the group
enhancing the regenerate in distraction osteogenesis. that received transplantation of osteogenic differentiated
stem cells (approx. 2.3 × 107 cells) and PRP around 3 weeks
Stem Cells after the lengthening surgery had a shorter healing index of
Stem cells can be of two types: approx. 31 days/cm compared with the control group which
1. Bone marrow-derived mesenchymal stem cells (MSC) had an average healing index of 55 days/cm and also had
2. Osteogenic differentiated progenitor cells reduced associated complications.
Bone marrow-derived mesenchymal stem cells (BMC)
can be directed towards the osteogenic lineage if cul- Scaffolds
tured with osteogenic supplements like dexamethasone, Stem cells may need a scaffold for osteoconduction. The
β-glycerophosphate, and ascorbic acid phosphate [103]. In 3-D structure of a scaffold determines the extent of cell
a study done by Peters et al. [104] in rats using histomor- migration and differentiation, bone ingrowth, vasculariza-
phometric analysis, it has been shown that locally applied tion, and mass transfer between the cells and the environ-
osteogenic differentiated progenitor cells are more effective ment. All of these processes benefit from increased scaffold
than mesenchymal stem cells in enhancing bone healing. porosity, interconnected pore networks, large surface area-
to-volume ratio and increased surface roughness [107].
Growth Factors Bioactive scaffolds aid in bone bonding. It is important for
Growth factors may be added to improve the results of scaffolds to interact with surrounding tissues to induce spe-
stem cell therapy. There are several osteoinductive growth cific cellular responses [108].
Approximately 3–5 ml of bone marrow is collected

Box 28.3 Properties of an Ideal Scaffold from the anterosuperior iliac spine and added to a con-
• Increased and interconnected porosity tainer filled with 30 ml of 10 % FBS-α MEM and 350 units
• Large surface area-to-volume ratio of heparin (Fig. 28.3). The mixture is then taken to the lab
• Same mechanical strength as bone and centrifuged at 4 °C for 10 min, after which the super-
• Bioactive natant was discarded and 20 ml of culture medium was
• Biocompatible added to the remaining pellets. The mixture is then filtered,
• Biodegradable 10 ml of the medium is added per T-75 culture flask, and
• Optimal release profile the culture is initiated. The incubator is maintained at
37 °C with 5 % CO2. The next day, 50 μg L-ascorbic
acid/10 ml and dexamethasone 10−7 M are added to facili-
One method to achieve this goal is to construct the scaf- tate cell differentiation into osteoblasts. The cell culture
fold with biomaterial that can stimulate platelets to release condition is evaluated using a light microscope, and the
growth factors. For instance, collagen is known to be as culture medium is changed on the fifth day of culture, after
effective as thrombin in activating platelets [109]. The acti- which the culture medium is changed every 3 days with the
vated platelets would then release growth factors, which will subsequent addition of L-ascorbic acid. On the 14th day of
enhance tissue regeneration, proliferation, and differentia- culture, nitro blue tetrazolium chloride 5-bromo-4-chloro-
tion. Therefore, a scaffold made with collagen type 1 will 3-indolyl phosphate (NBTBCIP) staining was performed
likely be effective in bone formation and bonding. to confirm activation of the alkaline phosphatase. Twenty-
The mechanical strength of a scaffold should approximate four days after beginning the culture, Alizarin red staining
that of bone. Weaker scaffolds cannot support the skeleton, is performed to detect newly produced calcium, and thus
whereas stiffer scaffolds lead to stress shielding. The carrier confirming that most of the cultured cells are osteoblasts.
should be biocompatible to minimize interference with bone 1 vial (0.4 ml) contains autologous cultured osteoblasts
induction. It must be biodegradable to minimize the effects on over 12 million. Every patient may need 1–6 vials (12–72
the biomechanical properties of the regenerate, and yet it must million cells).
persist in vivo long enough to maintain bioactive elements at
the site of implantation and optimize their release [110]. Injection Technique (Fig. 28.4)
Scaffolds available for use with stem cells include PRP In the non-sterile area, transfer whole amount of the cell
gel carrier, collagen, hydroxyapatite (HA) ceramic scaffold, suspension in two vials into the mixing vial using an 18-G
resorbable polylactide membrane, and polycaprolactone needle. In the sterile area, transfer only 0.1–0.2 ml of throm-
(PCL). Collagen scaffold seems to be a good delivery vehicle bin into the mixing vial and discard the rest of thrombin.
for other graft materials [110]. Maracci et al. [100] have Mix stem cell and thrombin in a mixing vial and transfer
demonstrated that extensive long bone defects in human sub- 1 ml into red (thrombin) syringe. Mix 1 ml of collagen and
jects treated with stem cell-seeded HA scaffolds have healed 1 ml of fibrinogen with two 3-ml syringes using a three-way
and integrated well with the host bone. Even after a 7-year connector (Fig. 28.5). Transfer 1 ml of mixed collagen and
follow-up the durability of the regenerated bone was good fibrinogen into blue (fibrinogen) syringe. Install the red
and there were no long-term complications noted. syringe and blue syringe into Y-piece and implant stem cells
with injector and spinal needle by checking the defect site
Other Uses of Stem Cells through C-arm (Fig. 28.6). After injection, this becomes a
A case of avascular necrosis of femoral head being treated gel within 3–5 min.
effectively by stem cells has been reported by Kim et al. Although the initial results are encouraging, further stud-
[102]. Lee et al. [111] have reported successful reconstruc- ies are necessary to assess the safety and efficacy of stem
tion of 15-cm segmental defects by bone marrow stem cells cells in distraction osteogenesis.
and autogenous bone graft in central hemangioma of man-
dible. Trials are also being carried out by using stem cells in
osteoporotic fractures [112]. “Osteogenic matrix coating,” a Other Experimental Local Stimuli
method to prevent loosening of joint prostheses by coating
them with osteogenic cells or their precursors, has been pro- Osteogenic peptides are cheaper to produce as they are syn-
posed by Ohgushi and Caplan [113]. thetic. They are less likely to lose their bioactivity during
storage and delivery because of their short, linear structure.
Osteogenic Progenitor Stem Cell
Culture Technique TP508
Our technique for procuring and processing the bone marrow One of these osteogenic peptides is TP508, which includes a
aspirate and culture of osteogenic progenitor stem cells is 23-amino acid peptide that is the non-proteolytic receptor-
explained below [101]. binding domain of human thrombin. The effects of TP508
Fig. 28.3 Collection of bone

marrow
Fig. 28.4 Flowchart depicting

the preparation of stem cell
mixture
Fig. 28.5 Using a three-way

connector, stem cell and
thrombin are mixed in a red
syringe. Similarly, collagen and
fibrinogen are mixed in a blue
syringe. Then both of these are
attached to the Y-piece
Fig. 28.6 Injection of the stem

cell preparation under C-arm
control
include changes in the inflammatory response, improve- the center of distracted callus facilitated the consolidation
ments in cell recruitment, and angiogenesis [114]. The doses during bone lengthening in rabbit tibiae even under strenuous
of TP508 used in the experimental studies vary from 0.1 μg conditions of rapid and long distraction. In a study conducted
to 300 μg, and results differ depending on the dose. The ani- by Jiang et al. [122], osteodistraction was applied in craniofa-
mals given the higher dose had a larger area of newly formed cial bone of rabbits to observe the effects of MSCs with or
bone [115–117]. without bFGF gene transfected on bone regeneration. They
noted that excellent bone formation and highest BMD and
ED-71 BMC were achieved in the bFGF gene transfected group.
Yamamoto [118] reported that administration of 2-β-(3-
hydroxypropoxy)-1α, 25-dihydroxyvitamin D3 (ED-71), an
analog of synthetic vitamin D3, increased the bone mineral Optimal Timing of Biologic Stimulants
content (BMC) at the lengthened callus. According to
Yamane et al. [119] ED-71 promoted both bone matrix for- There is no clear evidence to guide us in terms of the timing
mation and mineralization of the callus at 1 week after com- of injection to achieve better bone regeneration. Angiogenesis
pletion of lengthening, during which BMC increased has been shown to be of vital importance and intricately
markedly and may be effective for shortening the duration of involved in the inflammatory response, soft callus formation,
therapy in callus distraction. and transition from cartilaginous callus to bone [123, 124].
Proangiogenic factors such as vascular endothelial growth
FGF-2 factor (VEGF) are especially important during early phase of
Fibroblast growth factor-2 (FGF-2 or basic FGF) is recog- distraction, when their expression is significantly higher than
nized as a potent mitogen for a variety of mesenchymal cells. in later periods [125]. So, administration of growth factors
In skeletal tissues, FGF-2 is produced by cells of osteoblastic during the early phases may stimulate neoangiogenesis and/
lineage, accumulated in bone matrix, and acts as an autocrine/ or enhance the formation of regenerate bone. Several preclini-
paracrine factor for bone cells [120]. FGF-2 shows variable cal studies showed enhanced new bone formation by apply-
regulations of proliferation and differentiation of cells of ing BMPs on the day of the surgery [65, 126, 127] and
osteoblastic lineage and modulates bone formation. Aronson immunohistochemical analysis has shown high expression of
[121] has reported that the age-related deficits of endosteal growth factor receptors during the distraction phase [82, 125,
bone formation were reversed by administering exogenous 128–131]. In a randomized controlled trial, Lee et al. [86]
rhFGF-2 in an animal study. In another study Okazaki et al. clinically demonstrated that injection of BMAC and PRP on
[120] have reported that a single local injection of FGF-2 at the day of surgery enhanced new bone formation (Fig. 28.7).
Fig. 28.7 BMAC and PRP are prepared in the operation room at the eral blood are centrifuged to make BMAC (b) and PRP (c). Six millili-
end of index surgery: 60 ml of bone marrow is aspirated at the iliac crest ters of the aspirate sampled from the mid-layer are prepared for the
(a) and 60 ml of peripheral blood is obtained. Bone marrow and periph- injection (d)
Hamdy and Rauch et al. [62, 132] observed high levels of higher expression of osteocalcin in osteotomy sites when
expression of BMP-2, 4 and OP-1 during the distraction compared with the individual treatment or control groups
phase, with a rapid decline thereafter. They hypothesized [141]. Skripitz et al. [142] conjectured that since PTH
that the best time for exogenous injection of BMPs would be seemed to have a greater effect on new bone formation than
at the end of the distraction phase, when the endogenous on normal bone remodeling, it might become useful for
expression of BMPs is decreasing. But, they could not improving the incorporation of orthopedic implants and
observe enhanced bone healing when OP-1 is injected at the stimulating fracture repair. Neer et al. [143] reported that the
end of distraction, but found twofold increase in bone vol- clinical benefits of PTH reflect its ability to stimulate bone
ume when injected during early phase of distraction [62, 64]. formation and thereby increase bone mass and strength.
There are also several reports of enhanced healing when Given the enhancement of bone formation by the local stim-
applied at the end of distraction [85, 133, 134]. The optimal ulator such as BMP and the systemic application of PTH,
timing of the injection of the biological stimulants is an combination therapies may be beneficial for bone tissue
important but still unresolved issue. Hence further well- engineering.
designed investigations are required. The mechanism of this anabolic effect, however, has not
been well established. It is widely believed that the anabolic
effect of PTH may be due to increased osteoblast differentia-
Biological Stimulation: Systemic Application tion. Other report indicated that prevention of osteoblast
apoptosis is a crucial mechanism for the anabolic effects of
Parathyroid Hormone PTH on bone [144].
Parathyroid hormone (PTH) is one of the most promising

therapeutic agents for osteoporosis. Human and animal stud- Growth Hormone
ies have demonstrated that daily systemic injection of para-
thyroid hormone increases BMD [135–138]. PTH stimulates Systemic administration of recombinant homologous growth
bone formation and resorption and can increase or decrease hormone (GH) has been reported to greatly accelerate ossifi-
bone mass, depending on the mode of administration. cation of bone regenerate in distraction osteogenesis [145].
Continuous infusions and daily subcutaneous injections of GH administration increases bone turnover by enhancing
PTH stimulate bone formation similarly but have different both bone formation and resorption [146]. Correspondingly,
effects on bone resorption and bone mass [139, 140]. It has an increase in the mechanical strength of the whole bone
been reported that the combination of systemic and local occurs, whereas the mechanical quality of the osseous tissue
parathyroid hormone led to higher BMD, BMC, and bone is equivalent in GH-injected animals and controls [7].
area, a trend for greater radiographic-detected bone area and Various studies demonstrate that systemic administration of
GH is able to enhance bone regeneration and mechanical BMD of 44 % in the tibia and 61 % in the femur distal to the
strength of healing critical size bone defects in animal mod- regenerate in children undergoing limb lengthening. Maffulli
els [147–149]. et al. [162] noted that the BMC adjacent to the regenerate
decreased to less than 40 % of its original value in 6 of 11
patients. There was a reversal of osteopenia, increased regen-
Other Experimental Systemic Stimuli erate volume, and increased mechanical strength of the bone
when bisphosphonates were administered. However, healing
Hyperbaric oxygen therapy has been used to treat a variety of index remained very prolonged [163].
diseases and has been described as helping patients who have Im et al. [164] have shown that both risedronate and alen-
delayed healing or bone defects [150]. This technology con- dronate could increase osteoblast and osteoblast progenitor
sists of intermittently administering 100 % oxygen at pres- numbers in primary human trabecular cultures with enhanced
sures greater than one atmosphere absolute entirely enclosed expression of BMP-2, type I collagen, and osteocalcin. These
in a pressure chamber [150]. However, one systematic review effects are not limited to osteoblastogenesis but also through
[151] performed recently failed to locate any relevant clini- the promotion of osteoblast survival as per their efficacy as
cal evidence to support or refute the effectiveness of hyper- anti-apoptotic agents [165]. Furthermore, bisphosphonates
baric oxygen therapy for the management of delayed union were reported to enhance bone marrow mesenchymal stem
or established nonunion of bony fractures. Good-quality cell osteogenic differentiation [166] and facilitate human
clinical trials are needed to define its role in DO. adipose-derived stem cell osteogenesis for bone regeneration
Androgens such as testosterone are also known to have pro- [167]. Not only bone formation but also bone resorption are
liferative effects on osteoblasts and increase fracture healing highly activated in the regenerated bone, implying high bone
by systemic and local stimulation of bone formation [152]. In turnover. Sufficient nitrogen-containing bisphosphonate
clinical application, androgens may be a possibility to increase (N-BP) causes a notable modulation in morphological prop-
bone formation, especially in elderly patients. Furthermore, it erties of the regenerated bone through inhibition of highly
may be possible to shorten postoperative rehabilitation activated bone resorption and eventually increased mechani-
because of the effects of androgens on muscles [153]. cal properties [168]. The main effects of N-BP are at the
lumbar spine and proximal femur, where they stop bone loss,
reduce fracture risk, and increase BMD [169]. In addition,
Anticatabolic Therapy: Systemic Application N-BP are prescribed for the treatment of bone diseases such
as osteoporosis, multiple myeloma, cancer metastases, and
Bisphosphonate Paget’s disease.
However, the conventional systemic way of administra-
The potential anabolic effect of bisphosphonates has been tion of bisphosphonates may have some undesirable side
reported [154, 155]. Recent advances in the pharmacological effects such as gastrointestinal ulceration and jaw osteone-
treatment of osteoporosis with bisphosphonates [156–158] crosis [169, 170]. Recent studies have indicated that local
have encouraged surgeons to explore their use in limb length- administration of a low dose of bisphosphonates was able to
ening. In general, bisphosphonates have been considered enhance bone regeneration in vivo [171–173].
antiresorptive agents with limited effect on osteoblasts [159].
The anabolic effect of bisphosphonates has been questioned
as they are not internalized by osteoblasts, and therefore, they Other Experimental Anticatabolic Therapies
may not exert a direct effect on osteoblast activity. However,
there is evidence to support that there is an effect of bisphos- The influence of calcitonin on various stages of bone forma-
phonates on the osteoblast side not directly on mature osteo- tion has been investigated [174]. Weiss et al. [175] reported
blasts but more likely on osteoblast precursors in the bone that when calcitonin is administered during the initial phases
marrow. First, only nanomolar concentrations of bisphospho- of bone formation, it increases bone formation due to a stim-
nates are needed to stimulate the production of osteoclast ulation of proliferation of cartilage and bone precursor cells.
inhibitory factor, which is mostly secreted by osteoblast pre- In humans, serum calcitonin rises during pregnancy, growth,
cursors [160]. Second, bisphosphonates have shown to stimu- and lactation [176]. It is during these periods of calcium
late fibroblastic colony formation by murine and human bone stress that a tonic antiresorptive hormone will best exert its
marrow both in in vitro and ex vivo cultures [161]. effect to limit skeletal loss. This may be the primary role of
The use of external fixators or any form of fixation may be calcitonin in skeletal conservation [177]. There is consider-
associated with stress shielding of the spanned bone interval able support for the thesis that, in addition to its inhibitory
in this case, the regenerate and the surrounding bone result- effects on bone resorption, calcitonin enhances osteoblastic
ing in osteopenia. Eyres et al. [15] noted a mean decrease in bone formation both in vitro and in vivo [178]. The putative
anabolic osteoblastic effect of calcitonin may be of clinical 12. Windhagen H, Kolbeck S, Bail H, Schmeling A, Raschke
M. Quantitative assessment of in vivo bone regeneration consoli-
importance in sustaining the bone formation rate despite
dation in distraction osteogenesis. J Orthop Res. 2000;18(6):
inhibition of bone resorption. 912–9.
13. Romanowski CA, Underwood AC, Sprigg A. Reduction of radia-
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computed tomography scanogram techniques. Br J Radiol.
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unsolved issue in distraction osteogenesis. Avoiding the detri- X-ray absorptiometry and radiography compared. J Bone Joint
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Residual Deformities of the Hip
29
Shawn C. Standard and Daniel K. Ruggles
(AVN) of the proximal femoral epiphysis, osteomyelitis of

Introduction the proximal femur, and abnormal bone development that
occurs in the various skeletal dysplasias.
There are many causes of hip deformity in the growing child. Coxa magna is defined as the enlargement and deforma-
Although the etiologies are numerous, the resulting deformi- tion of the femoral head. This deformity can cause loss of
ties can be categorized anatomically by several basic descrip- congruency between the femoral head and acetabulum result-
tive terms. Angular deformities of the hip in the coronal ing in femoral-acetabular impingement (FAI) (Fig. 29.2a, b).
plane result in coxa valga or coxa vara. Similarly, angular Severe coxa magna is associated with chronic inflamma-
deformities in the sagittal plane are described as flexion or tion, loss of motion, labral pathology, chondromalacia, and
extension deformities. Axial plane torsional deformities are eventual degeneration of the hip joint [3–6]. Coxa magna
either external with retroversion of the hip or internal with is caused by a combination of the collapse of the proximal
anteversion of the hip. These six basic deformity terms; femoral epiphysis and altered growth of the articular car-
varus, valgus, flexion, extension, internal or external torsion tilage. The immature femoral head’s articular cartilage is
can be applied to other joints and long bones. However, alter- a true growth cartilage, which allows the femoral head to
ation of the hip joint’s unique configuration creates deformi- grow in a circumferential fashion [7]. The alteration of this
ties specific to this joint. These deformities are coxa breva, growth due to abnormal joint pressures from joint contrac-
coxa magna, and acetabular deformities, such as dysplasia, tures along with concurrent collapse of the epiphysis can
retroversion, and protrusio (Box 29.1). result in a coxa magna deformity. This deformity is caused
Coxa breva is defined as shortening and widening of the by avascular necrosis of the proximal femoral epiphysis. The
femoral neck, which alters the relationship of the tip of the most common cause of proximal femoral epiphyseal AVN is
greater trochanter to the center of the femoral head (Fig. 29.1). Legg–Calvé–Perthes disease. Other causes of pediatric hip
This creates a mechanical disadvantage of the hip joint by AVN include infection, sickle cell disease, significant ste-
decreasing the lever arm of the hip and decreasing the dis- roid use, trauma of the proximal femur, and iatrogenic loss
tance between the origin and insertion points of the gluteus of proximal femoral blood supply [8].
medius muscle [1, 2]. This deformity is caused by a growth Acetabular dysplasia is defined as an abnormal shape of
disturbance of the proximal femoral physis. The most com- the acetabulum with a decrease in depth, increased in cir-
mon causes of the abnormal growth are avascular necrosis cumference, and an increased slope in relation to the hori-
zontal pelvic line [9]. This deformity results in an
oblong-shaped, or “capacious” acetabulum, that allows lat-
eral and proximal migration of the femoral head with con-
S.C. Standard, MD
International Center for Limb Lengthening, Rubin Institute
current reduction of joint coverage (Fig. 29.3). In this
for Advanced Orthopedics, Sinai Hospital of Baltimore, abnormal position, the contact surface between the articu-
Schoeneman Building, 2nd Floor, 2401W. Belvedere Ave, lar cartilage of the femoral head and acetabulum is
Baltimore, MD 21215, USA decreased. Consequently, there are increased and concen-
trated contact forces that ultimately increases wear rate of
D.K. Ruggles, DO (*) the articular cartilage.
Department of orthopedic Surgery, Nationwide Children’s
Hospital, 700 Children’s Drive, Ste. A2630, Columbus,
Concurrently, the surgeon needs to recognize that a nor-
OH 43205-2664, USA mal shaped femoral head must be present for an acetabulum
e-mail: [email protected] to be defined as truly dysplastic. In other words, an oblong

536 S.C. Standard and D.K. Ruggles
Residual Deformities of the Hip Joint

Secondary to Developmental Dysplasia
The early postnatal examination and screening for congenital

dislocation of the hip has substantially decreased the inci-
dence of patients presenting with late dislocations of the hip
joint during infancy and early childhood [12, 13]. However,
the stable hip with mild to moderate hip dysplasia that is silent
during the early developmental years remains a challenge to
detect with clinical exam alone. The primary treatment for hip
dysplasia depends on the age of the child at diagnosis. The
various treatment options range from simple observation or
Fig. 29.1 Typical appearance of a coxa breva deformity demonstrated bracing to complex periacetabular osteotomies. The diagnosis
in this patient with a history of Perthes disease of the right hip. Reprinted
with permission from the Rubin Institute for Advanced Orthopedics, of hip dysplasia for the older infant, toddler, and young child,
Sinai Hospital of Baltimore is consistent with the radiographic Acetabular Index (AI)
measurement >30° [14, 15]. Hip dysplasia for the older child,
preadolescent, or adolescent is confirmed by the radiographic
acetabulum with a congruent enlarged femoral head does not Center Edge Angle of <20° [16] (Box 29.2). Other radio-
constitute acetabular dysplasia (Fig. 29.4). This situation is graphic signs of acetabular dysplasia are an abnormal sourcil,
better described as coxa magna with a congruent oblong decreased femoral head coverage, and lateral/proximal migra-
acetabulum, which can produce a functional, mobile hip tion of the femoral head, which collectively help to confirm
joint in certain situations. The abnormal acetabulum with a and stratify the severity of the hip dysplasia [16].
concurrent abnormal femoral head must be carefully evalu-
ated for congruency, impingement, and the area of force
transfer of the articular surfaces. Box 29.2. Radiographic Parameters of Acetabular
The etiology of acetabular dysplasia is most commonly Dysplasia
developmental in nature. However, other conditions resulting • Acetabular index >30°
in chronic joint instability, such as Down’s syndrome, or • Center Edge Angle <20°
severe joint contractures, such as cerebral palsy, can result in • Flattened/upsloped sourcil
hip subluxation with concurrent acetabular dysplasia [10, 11].
This chapter will concentrate on the residual hip deformi-
ties resulting from various etiologies including; developmen- Treatment of Residual Hip Dysplasia
tal dysplasia of the hip (DDH), slipped capital femoral for Patients <6 Months of Age
epiphysis, and Legg–Calvé–Perthes disease. Innovative treat-
ment strategies are presented with detailed surgical tech- The treatment for residual hip dysplasia without clinical
niques of six innovative procedures: triple pelvic osteotomy, instability for infants <6 months of age consists of applica-
modified Dunn procedure (with surgical hip dislocation), tion of a Pavlik harness or abduction bracing [17]. The har-
modified Southwick intertrochanteric osteotomy, Morscher ness is worn initially 16–18 h per day and then weaned to a
femoral neck lengthening osteotomy, femoral head reduction nighttime brace within the first month of treatment. The
osteotomy, and hip distraction with a hinged external fixator. night time bracing continues until the radiographic AI mea-
surement is <30° and the sourcil is normal in appearance.
After the hip has normalized based on such radiographic
Box 29.1 Residual Deformities of the Hip parameters, the bracing is discontinued and the patient is fol-
Defining key points: lowed annually until the age of 5 years.
• True acetabular dysplasia includes INCONG-
RUENCY of the hip joint
• The significance of coxa breva is determined by the Treatment of Residual Hip Dysplasia
center of femoral head to tip of greater trochanter for Patients >6 Months to 2 Years
distance
• Coxa magna is caused by avascular necrosis of the The treatment for residual hip dysplasia for patients
immature femoral head leading to abnormal and >6 months to 2 years of age consists of hip abduction bracing
asymmetric growth. (Box 29.3). These older infants and toddlers are often too
large and strong for the Pavlik harness, but a nighttime
29 Residual Deformities of the Hip 537
Fig. 29.2 A 12-year-old patient

with severe coxa magna resulting
from Perthes disease of the right
hip as demonstrated on the AP
(a) and lateral (b) views.
Baltimore
compliance with the bracing often improves. Despite limited

published data, this simple orthotic treatment is common
among pediatric orthopedists and can yield a high success
rate with resolution of the hip dysplasia in the vast majority
of patients if the brace is worn consistently. A recent study
has shown that part-time abduction bracing significantly
improves residual acetabular dysplasia at 6 months of age
versus observation alone [18]. After the hip has normalized
radiographically, the abduction brace is discontinued and the
patient is followed annually at least until the age of 5 years.
Box 29.3. Nonoperative Treatment of Acetabular

Dysplasia <2 Years of Age
Fig. 29.3 A 14-year-old female with Down’s syndrome and bilateral
acetabular dysplasia noted by the enlarged, oblong, and shallow acetab- • Pavlik harness for patients <6 months of age
ulum and concurrent femoral head migration. Reprinted with permis- • Simple hip abduction orthosis for patients
sion from the Rubin Institute for Advanced Orthopedics, Sinai Hospital >6 months of age
of Baltimore
• Night time bracing until radiographic parameters
and appearance normalizes
Treatment of Residual Hip Dysplasia

for Patients >2 Years Until Closure
of the Triradiate Growth Plate
Once the child is older than 2 years of age, the nighttime

bracing becomes very difficult due to patient cooperation
and compliance. However, in the older toddler, if the night-
time hip abduction brace could be tolerated, then this would
be the initial treatment strategy as stated above for the
younger child. For the majority of older toddlers and young
Fig. 29.4 An example of an enlarged and oblong acetabulum second- children, the treatment for clinically significant hip dysplasia
ary to remodeling effects of a large femoral head after Perthes disease. consists of a single cut pelvic osteotomy that hinges on the
This acetabulum should not be termed dysplastic due to the absence of triradiate growth plate or the pubic symphysis (Box 29.4).
a normal femoral head. In this situation, the right acetabulum is insuf-
ficient due to the decrease in force transfer noted by the reduced sourcil The direction and severity of acetabular deficiency influ-
and patient’s symptoms. Reprinted with permission from the Rubin ences the exact type of pelvic osteotomy that is performed.
Institute for Advanced Orthopedics, Sinai Hospital of Baltimore The type and direction of coverage of the commonly used
pelvic osteotomy is as follows: the Pemberton pelvic oste-
abduction brace is very effective. The main difficulty with otomy primarily provides anterior coverage, the Dega pelvic
this treatment in the older toddler is the compliance with the osteotomy covers anterolaterally, and the San Diegoplasty
orthosis. However, in the authors’ experience, once the covers laterally [10]. These three “volume-reducing” osteot-
potential surgical option is discussed with the parents, the omies are very similar but with distinct differences. All three
Another type of pelvic osteotomy that can be used in this

age group is the Salter osteotomy that consists of a complete
osteotomy from just above the anterior inferior iliac spine to
the sciatic notch, including both inner and outer tables of the
ilium [23]. The Salter is a redirectional osteotomy that hinges
on the flexible symphysis pubis and requires internal fixation
due to the amount of inherent instability. However, the Salter
osteotomy affords greater movement of the acetabulum and
thereby a greater ability to correct a more severe dysplasia.
The surgeon must be aware that a dysplastic acetabulum with
poor posterior coverage is a relative contraindication for a
Fig. 29.5 The classic Dega osteotomy curves from the anterior inferior Salter osteotomy, as while it provides excellent anterolateral
iliac spine to the triradiate cartilage posteriorly hinging on both the inner coverage, it can further uncover the femoral head posteriorly
iliac table and the triradiate cartilage. This is demonstrated in both the sche- (Fig. 29.7a–f).
matic drawing (a) and bone model (b). Reprinted with permission from the
Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Postoperative care for innominate pelvic osteotomies
requires non-weight bearing for approximately 6 weeks.
The patients between the ages of 2 and 5 years are placed
are incomplete osteotomies, hinging on the triradiate carti- into a 1-½ hip spica cast for the initial 6 weeks, whereas the
lage and do not usually require internal fixation for stability. older patients (6 years and older) are often placed into a hip
The Dega osteotomy is the classic pelvic osteotomy that abduction pillow. After the initial 6-week period, the cast/
hinges on the triradiate growth cartilage [19, 20]. The Dega pillow is discontinued and physiotherapy is started. The
osteotomy begins at the anterior inferior iliac spine and patient gradually returns to full weight bearing. Full activity
curves posteriorly around the acetabulum to end at the trira- is allowed after reestablishing full hip strength and range of
diate cartilage. Medially, the Dega pelvic osteotomy is motion. The patient is then followed annually until skeletal
directed in a curvilinear fashion over the acetabulum towards maturity.
the triradiate cartilage, leaving the inner table intact. Wedges
of bone graft (autograft harvested from the iliac crest or the
proximal femur when a shortening is being performed, ver- Box 29.4. Acetabuloplasties in the Skeletally Immature
sus allograft bone), can strategically be placed in the osteot- Choosing your osteotomy:
omy site to provide a combination of increased anterior and/ • Planning your osteotomy is determined by acetabu-
or lateral coverage (Fig. 29.5a, b). lar deficiencies specific to underlying etiologies
Two modifications of the Dega-type osteotomy are the (i.e., DDH = anterolateral, congenital femoral defi-
San Diegoplasty osteotomy and the Pemberton osteotomy ciency = lateral, neuromuscular = posterolateral).
(Fig. 29.6a–e). The San Diegoplasty osteotomy does not vio- • The Salter osteotomy can provide significant defor-
late the inner table; however, a notch of bone is removed mity correction, but requires internal fixation for
with a Kerrison rongeur at the anterior inferior iliac spine stability.
and the sciatic notch. This allows more coverage directed • The Dega, San Diegoplasty, and Pemberton osteot-
laterally without sacrificing posterior coverage, and was omies do not require fixation routinely.
originally described for hip dysplasia associated with neuro- • Bone graft wedges can strategically be placed in a
muscular disease, which typically entails posterior acetabu- Dega osteotomy to provide more anterior, posterior,
lar deficiency [21] (see Fig. 29.6a–c). or lateral coverage.
The Pemberton osteotomy, first described in 1965, also
hinges on the triradiate growth cartilage, but the direction of
this osteotomy is from an anterior to posterior direction
instead of a lateral to medial direction, as with the Dega and Treatment of Residual Hip Dysplasia
San Diegoplasty [22]. The Pemberton osteotomy begins at for Patients After Closure of the Triradiate
the anterior inferior iliac spine and curves over the acetabu- Growth Plate
lum and ends at the triradiate growth cartilage posterior to
the joint identical to the Dega osteotomy. However, the As the patient becomes older, the treatment for hip dysplasia
Pemberton osteotomy divides both the inner and outer tables becomes more complicated and invasive. However, unlike
of the ilium as it heads over the acetabulum to the triradiate the asymptomatic younger child who is usually diagnosed
cartilage. This creates a pure anterior opening wedge oste- incidentally and treated prophylactically, the older patient
otomy with a greater increase of anterior coverage for the typically presents with mild to moderate pain associated
acetabulum (see Fig. 29.6d, e). with activity. Therefore, the treatment goals in this age group
Fig. 29.6 (a–c) The bone model depicts the San Diegoplasty pelvic tilage behind the acetabulum. Unlike the Dega osteotomy, the
osteotomy that begins at the anterior inferior iliac spine (AIIS) and ends Pemberton osteotomy divides both the inner and outer tables of the
at the sciatic notch, hinging on the inner table of the ilium. (d, e) The ilium. Reprinted with permission from the Rubin Institute for Advanced
bone model depicts the Pemberton osteotomy that is directed from ante- Orthopedics, Sinai Hospital of Baltimore
rior to posterior, beginning at the AIIS and ending at the triradiate car-
Fig. 29.7 (a) A 5-year-old male with R DDH. (b) Status-post open restoration of normal acetabular index, center-edge angle, and Shinton’s
reduction, capsulorraphy, femoral shortening derotational osteotomy, line. (d–f) Bone models representing the Salter osteotomy. Reprinted
and Salter innominate osteotomy. (c) Healed osteotomy with complete with permission from Scott Nelson, MD
are the alleviation of pain, improved joint function, and pre- described as two-incision procedure, further modifications
vention of early degenerative joint disease of the hip. of the triple osteotomy utilize a one-incision technique [26].
In the authors’ practice, the preferred method of treatment Because this is a complex osteotomy, a brief description of the
for symptomatic residual hip dysplasia in the young patient technique will follow, highlighting certain technical tips.
after the closure of the triradiate cartilage is the periacetabular The Ganz periacetabular osteotomy is another very popu-
triple osteotomy (Box 29.5). The triple is a redirectional oste- lar type of pelvic osteotomy [27]. This particular osteotomy
otomy including ischial, pubis, and supra-acetabular innomi- maintains the integrity of the posterior column by perform-
nate bone cuts. An early technique described by Steel has ing a bone cut connecting the iliac osteotomy to the partial
continued to evolve [24]. Tonnis modified the procedure by ischial osteotomy. This osteotomy travels through a very nar-
performing the ischial cut in closer proximity to the acetabu- row column of bone in the young patient. Therefore, the
lum, facilitating acetabular fragment mobility and greater author prefers the periacetabular triple technique for adoles-
correction of severe acetabular dysplasia [25]. Originally cent and smaller adult patients
superior pubic ramus osteotomy in the correct position.

Periacetabular Triple Osteotomy Typically, the osteotomy site lies between the femoral nerve
of the Pelvis: Operative Technique and artery. However, with smaller patients, the osteotomy
position can be medial to the femoral vein. The interval
Positioning and Exposure between the femoral nerve and artery is exposed down to the
periosteum of the superior pubic ramus. Great care must be
The patient is placed supine on the operating table with no used when retracting the neurovascular bundle. If the interval
bumps placed under the pelvis. The entire lower extremity, to for the exposure of the superior pubic ramus is performed
include the groin, gluteal region, and subcostal area are lateral to the femoral nerve, the tension placed on the neuro-
prepared and isolated into the sterile field. A single incision vascular structures will be substantially increased in order to
is performed in the flexion crease of the hip joint, approxi- reach the proper osteotomy position.
mately 1 cm below the anterior superior iliac spine. This
incision starts just medial to the palpable neurovascular bun-
dle and extends 6–8 cm lateral to the anterior superior iliac Osteotomies
spine (Fig. 29.8).
The entire operation is performed through this single After the exposure of the correct interval for the pubic ramus
anterior incision. The interval between the sartorius and ten- osteotomy is completed, the overlying periosteum is divided.
sor fascia lata muscles is defined in the typical fashion pro- Multiple drill holes are created in the superior pubic ramus
tecting the lateral femoral cutaneous nerve. The rectus using a smooth 1.8- to 2.0-mm wire. The osteotomy is com-
femoris tendon is identified and released, tagging the inser- pleted with an osteotome. The interval for the ischial osteot-
tional portion of tendon. Next, the iliac crest is exposed. omy is then created. This interval lies between the medial
Both the inner and the outer tables are subperiosteally dis- aspect of the hip capsule and the iliopsoas muscle. The inter-
sected to the sciatic notch. Surgical gauze and/or bone wax val is carefully created with gentle blunt dissection using a
may be used to control bleeding from the iliac wing. large curved Mayo scissor. The interval is opened and wid-
Dissection of the medial structures is subsequently per- ened by gentle spreading of the blunt tipped scissor. A small
formed. The fascia overlying the sartorius and iliopsoas mus- Cobb elevator is placed into the interval to palpate the superior
cles is divided. The iliopsoas tendon is identified (typically portion of the ischium, just below the acetabulum (Fig. 29.10).
on the posterior aspect of the muscle) and fractionally A Cobb elevator is advanced on both the medial and lateral
lengthened within the muscle belly. Gentle retraction of the sides of the ischium to push the soft tissues away and prepare
iliopsoas muscle belly brings the femoral nerve into the a path for the Ganz osteotome (see Fig. 29.11d, e).
operative field. The femoral nerve should be identified prior The Ganz osteotome is inserted into the prepared interval
to the division of the iliopsoas tendon. The femoral nerve, and positioned just under the acetabulum. It is critical to have
artery, and vein are carefully identified and exposed with adequate fluoroscopic visualization for this osteotomy. The
individual small vessel loops placed around each structure. osteotome is aimed approximately 45–55° proximally in
Fluoroscopic visualization of the superior pubic ramus is order to direct the osteotomy above the ischial spine
performed to confirm the osteotomy position just medial to (Fig. 29.11a–e).
the pubic eminence (Fig. 29.9). It is always surprising how This osteotomy is visualized with an oblique fluoroscopic
medial the dissection must be carried out in order to place the view, similar to an obturator Judet view (C-arm is rotated
Fig. 29.8 (a, b) The single incision technique is depicted from an ante- neurovascular bundle. Reprinted with permission from the Rubin
rior and lateral view. The iliac crest is marked with the dashed line Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
ending at the ASIS. The longitudinal marked structures are the palpable
toward the operative hip between 30° and 50° from the AP osteotome handle is levered distally in order to aim the oste-
position). Again, fluoroscopic visualization of the acetabu- otomy above the ischial spine. Before the final osteotomy of
lum, ischium, and ischial spine is essential in this oblique the ilium, two reference half pins are inserted into the acetab-
view in order to complete the periacetabular ischial osteot- ular fragment. These two reference half pins enhance the
omy successfully. Another critical technical tip is the protec- accuracy of the bony correction and help prevent secondary
tion of the femoral nerve while completing the ischial deformities (Fig. 29.12).
osteotomy. During this osteotomy, the hip must be flexed The first half pin is place from the anterior to posterior
15–20° to prevent stretch of the femoral nerve as the Ganz direction, just lateral to the superior pubic ramus osteotomy.
This anterior reference half pin marks the acetabular version.
The second half pin is inserted from lateral to medial in the
supra-acetabular region, parallel to the roof or sourcil of the
acetabulum. This lateral reference half pin marks the dys-
plastic acetabular roof. After the reference half pins are
placed, the final osteotomy of the ilium is performed. A Gigli
saw is carefully passed through the sciatic notch. The final
osteotomy is completed with the Gigli saw from the sciatic
notch to the anterior aspect of the ilium, just above the ante-
rior inferior iliac spine.
Deformity Correction and Fixation
The acetabular fragment should become freely mobile after

the third cone cut. If the fragment does not easily move, then
the pubic ramus osteotomy and ischial osteotomy are revis-
ited with repeat passage of the osteotome. After mobilizing
the acetabular fragment, the reference half pins are used to
reorient the acetabulum and temporary fixation is performed
(Fig. 29.13a). The anterior half pin should still be pointed in
a straight anterior to posterior direction if the version of the
Fig. 29.9 A 4-year-old female undergoing a triple osteotomy for right acetabulum was planned to remain the same. If anteversion
hip dysplasia. Intraoperative fluoroscopic image demonstrating the is needed, then the anterior half pin should be internally
position for the superior pubic ramus cut. This position is usually rotated. Similarly, if the anterior half pin is externally
directly behind or medial to the neurovascular bundle. Reprinted with
permission from the Rubin Institute for Advanced Orthopedics, Sinai rotated, retroversion has been imparted to the new acetabular
Hospital of Baltimore position. The temporary fixation consists of three separate
Fig. 29.10 A 16-year-old female

undergoing a triple pelvic
osteotomy for retroversion of the
right hip. (a) Intraoperative
fluoroscopic image of a Cobb
instrument palpating the superior
portion of the ischium just
beneath the acetabulum. (b)
Image demonstrating the
placement of the Ganz osteotomy
at same location as identified by
the Cobb instrument. Reprinted
Baltimore
Fig. 29.11 (a) Fluoroscopic

view denoting the position of the
Ganz osteotome in the AP view
preparing to perform the lateral
cortical cut of the ischium. (b)
Fluoroscopic view in the oblique
position to confirm placement of
the Ganz osteotome under the
acetabulum and directed above
the ischial spine. (c) Same
fluoroscopic view as Fig. 29.11b
with the ischial spine highlighted.
(d, e) Clinical pictures of the
specialized Ganz osteotomes.
Baltimore
Fig. 29.12 Fluoroscopic view

demonstrating the placement of
the two reference half pins prior
to the completion of the iliac
osteotomy. The AP half pin
marks the acetabular version
whereas the lateral to medial half
pin marks the slope of the
acetabular roof. Reprinted with
permission from the Rubin
Baltimore
Fig. 29.13 (a) After the

acetabular fragment is reduced
into the corrected position,
temporary fixation is achieved
with smooth wires. (b) The
smooth wires are sequentially
overdrilled with a 3.2-mm
cannulated drill and 4.5-mm solid
screws are inserted. This is the
same patient as Fig. 29.16.
Baltimore
Fig. 29.14 (a) A 14-year-old

female with Down’s syndrome
and concurrent severe coxa valga
and acetabular dysplasia. (b) Left
hip corrected with proximal varus
osteotomy of the femur with
periacetabulr triple osteotomy.
Baltimore
Fig. 29.15 (a) An 18-year-old male with history of Perthes disease (c) Subsequent triple pelvic osteotomy in same patient to address
of the right hip who had previously undergone aproximal femoral val- residual hip pain due to decrease contact area between newly shaped
gus osteotomy with residual impingement pain and gluteus medius femoral head and oblong acetabulum. Reprinted with permission from
weakness. (b) Same patient after Ganz femoral head reduction surgery the Rubin Institute for Advanced Orthopedics, Sinai Hospital of
and relative femoral neck lengthening with greater trochanter transfer. Baltimore
Fig. 29.16 A 13-year-old female basketball player presents with new- removal with excellent left hip configuration and no compromise of the
onset left hip pain with radiographs depicting underlying acetabular pelvic outlet for future childbearing. This is the same patient shown in
dysplasia (a), radiographs after triple osteotomy with improvement of Figs. 29.11, 29.12, and 29.13. Reprinted with permission from the
femoral head position and coverage and normalization of the center Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
edge angle (b). (c) Radiographic appearance of pelvis after hardware
1.8-mm Ilizarov or similar sized smooth k-wires. After ade- The following figures are case examples of the triple pel-
quate correction and repositioning of the acetabulum is com- vic osteotomy (Figs. 29.14, 29.15, and 29.16).
pleted and confirmed under fluoroscopy, the wires are Closure proceeds with repair of the iliacus fascia to the
sequentially overdrilled with a cannulated 3.2-mm drill bit gluteus medius fascia to cover the exposed iliac crest, repair
and solid 4.5-mm screws are inserted for final stabilization of the rectus femoris tendon, and a layered closure of the
(Fig. 29.13b). subcutaneous tissues and skin over a drain.
Postoperative Care on the severity of the SCFE and the prior treatment. Some
patients will present with a long standing SCFE with severe
The patient is placed into a hip abduction pillow prior to leav- deformity that has never previously been diagnosed.
ing the operative suite and converted to a single leg hip The typical treatment of in situ screw fixation for minimal
abduction brace before discharge from the hospital. Toe- or mild SCFE has proven effective with long-term success
touch weight bearing is allowed with crutches or a walker for [30]. However, the same strategy for moderate or severe
6 weeks. After radiographic confirmation of healing, the magnitude of SCFE often results in unacceptable residual
patient is advanced to full weight bearing and physiotherapy. deformities and is associated with pain, gait abnormality and
joint dysfunction [31, 32]. There is no absolute criterion for
the amount of deformity that requires immediate or eventual
Box 29.5. Modified Triple Osteotomy
reconstruction. However surgeons are increasingly manag-
Technical tips: ing even milder deformity associated with FAI in order to
• Can be performed with a single anterior ilioinguinal prevent poor long term outcomes [33, 34]. The decision to
incision. perform a corrective osteotomy should be based on the
• Identify, isolate with a vessel loop, and protect the patient’s function and symptoms, as well as the magnitude of
femoral nerve, artery and vein prior to iliopsoas deformity. If conservative observation is the treatment strat-
tenotomy as well as during the superior ramus and egy, then the physician should follow the patient well into
ischial bone cut. young adulthood to monitor the presence of early degenera-
• Adequate medial exposure must be achieved for the tive joint disease or impingement that would require a more
superior rami bone cut. aggressive treatment plan.
• The superior rami osteotomy site usually lies The recommended treatment strategy for residual hip
directly behind the neurovascular bundle. deformities secondary to SCFE consists of either a modified
• A multiple-drill hole technique, Ganz osteotome, Southwick intertrochanteric osteotomy or a modified Dunn
and Gigli saw are utilized for the pubic rami, ischial, procedure for femoral head reorientation. The osteotomy
and ileal bone cuts, respectively. elected may depend on whether the proximal femoral growth
• Proper oblique fluoroscopic views are critical to plate is open or closed. If the physis remains open, then the
performing the ischial osteotomy. author’s treatment of choice is the modified Dunn procedure
• The Ganz osteotomy must be directed above the via a surgical hip dislocation described by Ganz [35]. Once
ischial spine for the correct ischial osteotomy. the physis has closed, the proximal femoral periarticular reti-
• It is critical to flex the hip 15–20° while levering the naculum becomes very thin resulting in a higher risk of avas-
Ganz osteotome distally to achieve the proper oste- cular necrosis for the modified Dunn procedure. In the face
otomy direction; this position protects the femoral of a significant proximal femoral deformity with a closed
nerve from a tension injury. capital femoral physis, a modified Southwick intertrochan-
• Two half-pins are used as anatomical reference teric osteotomy is recommended.
points and for controlled mobilization of the Performing a subcapital osteotomy versus an intertro-
fragment. chanteric osteotomy for a chronic SCFE deformity remains
• Three to four large fragment screws (4.5 mm) are controversial. Several studies of the modified Dunn proce-
adequate for fixation. dure have reported favorable results with significantly low
rates of avascular necrosis [36–38]. However, there exists
inconsistent reports on the incidence of avascular necrosis
associated with subcapital osteotomies [39–41]. The deci-
Residual Deformities of the Hip Joint sion may be surgeon dependent, and related to experience
Secondary to Slipped Capital Femoral with surgical hip dislocation and the Modified-Dunn proce-
Epiphysis dure. Surgeons may elect to stage treatment of a skeletally
immature, chronic stable SCFE by first performing in situ
The residual deformities that result from a previous slipped screw fixation, and then following with a planned intertro-
capital femoral epiphysis (SCFE) are typically varus, exten- chanteric osteotomy after physeal closure. An open or
sion, external femoral rotation, leg length discrepancy, and arthroscopic osteoplasty, or “bumpectomy,” of the femoral
anterolateral femoral acetabular impingement (FAI) [28, 29]. neck deformity can also be performed in combination with
The clinical relevance of each of these deformities depends this approach to avoid femoral-acetabular impingement.
Surgical Hip Dislocation

The Modified Dunn Procedure for Residual
Deformities of the Proximal Femur The next step in the procedure is the exposure of the hip
Secondary to SCFE: Operative Technique capsule and the Z capsulotomy. A small Cobb elevator is
used to lift the gluteus minimus muscle off of the capsule and
The modified Dunn procedure has been described multiple provide an interval for a Taylor retractor to be inserted onto
times in the current literature. H. Huber et al. provided an the outer table of the ilium. The surgeon will note a promi-
excellent description of this procedure in 2009 [39]. nence in the hip capsule caused by the superior ridge of the
femoral neck that was “left” behind as the femoral head pro-
gressively slipped in a posterior and medial direction. The
Positioning and Exposure Z-capsulotomy is performed with the longitudinal limb in
the midline of the capsule. The anterior limb of the capsu-
The modified Dunn procedure is performed with the patient lotomy is performed at the base of the femoral neck while the
in the lateral decubitus position. The incision is centered on posterior limb is extended along the posterior rim of the ace-
the greater trochanter with approximately 5–8 cm distal and tabulum (Fig. 29.19).
5–8 cm proximal (Fig. 29.17). The critical step at this point in the procedure is the cre-
The interval between the tensor fascia lata and gluteus ation of the retinacular soft tissue flap that will contain the
maximus muscles is used to gain access to the level of the blood vessels to the femoral head. Careful removal of bone
vastus lateralis and gluteus medius muscles. The posterior from the base of the greater trochanter will create this flap
border of the vastus lateralis muscle is identified at the level that consists of the proximal femoral periosteum, pericapsu-
of the vastus ridge. The posterior border of the gluteus lar retinaculum, and short external rotator muscles. The cre-
medius muscle is identified along with the piriformis tendon ation of this flap is accomplished by removing 5–8 mm of
and the sciatic nerve. A greater trochanteric osteotomy is bone from the posterior aspect of the greater trochanteric base
performed with an oscillating saw that connects the posterior by the “inside out technique”(Fig. 29.20). It is critical to mini-
border of the vastus lateralis muscle to the posterior border mize soft tissue tension and stretch while excising the bone.
of the gluteus medius muscle. The greater trochanteric frag- Similarly, the proximal aspect of the greater trochanter
ment should be approximately 1–1.5 cm thick for adequate base is removed to create a surface that is flush with the supe-
fixation at the end of the procedure. The digastric muscle flap rior portion of the femoral neck. This will result in the “rela-
to include the vastus lateralis muscle, the greater trochanteric tive” femoral neck lengthening after the greater trochanteric
fragment, and gluteus medius muscle is flipped medially fragment is advanced distally during the closure.
allowing exposure of the gluteus minimus muscle and under- If the femoral head has not been previously secured with
lying hip joint capsule (Fig. 29.18a–c). prior screw fixation, then temporary fixation with a stout
single threaded K-wire is performed (Fig. 29.21). The hip is
gently dislocated with release of the ligamentum teres from
the fovea capitis. After the femoral head is delivered out of
the joint, the acetabulum and labrum are inspected for any
detrimental changes. Next, the periosteum and retinaculum
of the femoral neck is carefully peeled from anterior to the
mid-line both medially and laterally. The lateral aspect of
this dissection is critical and the continuity of the soft tissue
flap to the femoral head must be maintained.
Following dislocation, the femoral head fixation is removed

Fig. 29.17 The incision is centered on the greater trochanter for a total and a curved osteotome is inserted into the physis. Gentle
length of 15–20 cm. The clinical photograph depicts the outline of levering of the curved osteotome is performed to allow the
the iliac crest, the ASIS, the greater trochanter, and the longitudinal
femoral head to gradually detach from the femoral neck.
incision centered on the greater trochanter. Reprinted with permission
from the Rubin Institute for Advanced Orthopedics, Sinai Hospital of While the femoral head is carefully being levered off the
Baltimore neck, the retinacular soft tissue flap should be visualized to
Fig. 29.18 (a, b) The greater trochanteric osteotomy begins at the pos- ment should be approximately 1.0–1.5 cm in thickness. Reprinted with
terior border of the vastus lateralis muscle and extends to the posterior permission from the Rubin Institute for Advanced Orthopedics, Sinai
border of the gluteus medius muscle. (c) The greater trochanteric frag- Hospital of Baltimore
prevent any tearing or stretching. If the final posterior por-

tion of the physis is difficult to detach, the osteotome is care-
fully directed slightly distally, removing a small portion of
the posterolateral femoral neck with the femoral head in
order to protect the blood supply. The femoral head along
with the soft tissue vascular flap is allowed to fall back into
the acetabulum and the femoral neck is lifted into the opera-
tive field for inspection (Fig. 29.22).
The femoral neck is trimmed of excess bone and short-
ened to eliminate any tension of the vascular leash with fem-
oral head reduction. The shortening does not have to be
excessive, approximately 1 cm, as long as no tension of the
blood supply is evident when the femoral head is reduced.
Fig. 29.19 The Z-capsulotomy is created with a longitudinal incision
in the capsule in line with the femoral neck. The anterior limb of the The femoral head is carefully retrieved from the acetabulum
capsulotomy is performed anterior at the base of the femoral neck. The and reduced to the “new” femoral neck. The vascular reti-
posterior limb of the capsulotomy is directed along the posterior border nacular leash is inspected to insure no tension is present after
of the acetabulum. Reprinted with permission from the Rubin Institute which temporary K-wire fixation is performed. The hip joint
for Advanced Orthopedics, Sinai Hospital of Baltimore
is reduced and its position is confirmed under fluoroscopy.
The fixation is finalized via 4.5-mm screws, 3.5-mm screws,
or threaded K-wires. The size of the fixation depends on the
size of the femoral head and balancing stable fixation while
minimizing the amount of area of the femoral head violated
to prevent devascularization. The capsule is repaired with
care not to over tighten the posterior capsule, which could
stretch the blood supply to the femoral head. The greater tro-
chanter is advanced distally and repaired with two 4.5 mm
solid screws or two 5.5-mm cannulated screws. The goal of
the greater trochanteric transfer is to advance the proximal
tip of the greater trochanter below the newly established cen-
ter of the femoral head (Fig. 29.23). The remainder of the
closure is performed in the typical fashion over a drain.
Fig. 29.20 The posterior aspect of the base of the greater trochanter is Postoperative Care
excised to create the soft tissue vascular sleeve to the femoral head. The
excision of 5–8 mm of bone produces soft tissue redundancy that allows
The patient is placed into hip abduction pillow prior to
greater mobility of the femoral head during the realignment procedure.
Reprinted with permission from the Rubin Institute for Advanced leaving the operative room and converted to a single leg
Orthopedics, Sinai Hospital of Baltimore hip abduction brace before discharge from the hospital.
Fig. 29.21 (a, b) A 14-year-old male who presented with an acute tion of the hip without compromising the vascular supply to the femoral
slipped capital femoral epiphysis. (c, d) Intraoperative fluoroscopic head. Reprinted with permission from the Rubin Institute for Advanced
views depicting temporary head fixation to allow for surgical disloca- Orthopedics, Sinai Hospital of Baltimore
postoperative mark, the patient begins gradual advance to

full weight bearing, hip joint range of motion, and hip mus-
cle strengthening.
Box 29.6. Modified Dunn Procedure

Technical tips:
• Adequate thickness of the greater trochanter
osteotomy must be maintained for later fixation
(1–1.5 cm width).
• The most critical aspect of the exposure is creating
and maintaining the posterior retinacular soft tissue
sleeve.
• The femoral head must be temporarily stabilized
before the hip is dislocated to insure the vascular
leash is not torn.
• Removing 5–8 mm of bone from the posterior mar-
gin and the base of the greater trochanter as well as
shortening the neck up to 1 cm will aid in relieving
tension on the vascular flap.
• Fixation of the femoral head can be done with
Fig. 29.22 The femoral head is allowed to fall back into the acetabu- 4.5 mm screws, 3.5 mm screws, or threaded
lum after gently levering the femoral head off of the neck through the
pathologic physis. Reprinted with permission from the Rubin Institute Steinman pins.
for Advanced Orthopedics, Sinai Hospital of Baltimore • The hip capsule should be repaired with minimal
tension to avoid stretch of the vascular supply to the
femoral head.
The patient remains toe touch weight bearing for 12 weeks
• The greater trochanter should be advanced until the
with gentle and limited passive range of motion of the hip.
proximal tip is at or below the center of the femoral
The initial 6-week postoperative range of motion is per-
head.
formed with a CPM (continuous passive motion) device. The
• The greater trochanter is repaired with two 4.5-mm
second 6-week period therapy protocol consists of gentle,
or 5.5-mm screws with washers.
limited passive hip range of motion and CPM. At the 12-week
Fig. 29.23 (a, b) Demonstrating the same patient as in Fig. 29.21 with frog pelvis radiographs at 8-month postoperative follow-up. Reprinted
intraoperative fluoroscopic views of the final femoral head alignment with permission from the Rubin Institute for Advanced Orthopedics,
after reduction of the slipped capital femoral epiphysis. (c, d) AP and Sinai Hospital of Baltimore
prepared and draped. A lateral longitudinal incision is per-

The Modified Southwick Osteotomy formed starting at the base of the greater trochanter and
for Residual Deformities of the Proximal extended distally for approximately 10–15 cm. A typical lat-
Femur Secondary to SCFE: Operative eral approach to the proximal femur is performed. This is
Technique accomplished by splitting the tensor fascia lata and retracting
the vastus lateralis muscle anteriorly. The lower extremity can
The Southwick osteotomy has been described in very compli- be extended, adducted, and internally rotated to visualize the
cated terms in the past, such as a biplanar or a triplanar oste- normal profile of the femoral head within the acetabulum. This
otomy [42]. In actuality, the osteotomy can be a simple position can only be obtained by allowing the leg to hang off
transverse osteotomy stabilized with either an internal blade the side of the operating table. This is accomplished by raising
plate fixation or a simple Ilizarov external fixation. The femo- the operative table and allowing the leg to extend off the side
ral neck deformity can be described as being in a single of the table on a sterile padded Mayo stand. The position of the
oblique plane in the anterolateral direction. This oblique plane leg denotes the deformity that must be corrected.
deformity is seen as a varus deformity in the coronal view and
an extension deformity in the sagittal view. A true second
plane of deformity in a residual SCFE exists in the axial plane Deformity Correction and Fixation
with retroversion of the hip, or external femoral torsion.
However, with proper execution, the Southwick osteotomy A threaded guide pin is inserted into the center of the femoral
can correct both the oblique plane deformity and the external head under fluoroscopic guidance in both the coronal and
femoral torsion deformity. The one legitimate criticism of the sagittal planes. The guide pin is first measured to determine
Southwick osteotomy is the inability to resolve the anterolat- the length of the blade, and then the appropriate-sized chisel
eral prominence at the femoral head-neck junction that causes is placed over the guide pin. The position of the guide pin
the femoral acetabular impingement (Fig. 29.24). determines the correction of the varus deformity, whereas,
The simple modification to the original Southwick proce- the rotation of the chisel will determine the deformity correc-
dure is an addition of an anterior approach to the hip with a tion in the sagittal plane. In order to correct the extension
capsulotomy and excision of the impinging prominence deformity caused by the posteriorly slipped epiphysis, the
(Fig. 29.25) (Box 29.7). The Southwick osteotomy can be chisel is rotated the number of degrees towards the ceiling
performed with either internal or external fixation. The equal to the amount of sagittal plane deformity. After the
authors’ internal fixation implant of choice is a 130° cannu- correct chisel track has been created, a transverse osteotomy
lated locking blade plate. The external fixation of choice can in the intertrochanteric zone is performed with an oscillating
be either a ring fixation device or lower profile external fix- saw. The chisel is withdrawn and the appropriate sized can-
ator as described by Sabharwal [43]. nulated 130° blade plate is inserted over the original guide
pin. An ideal 130° blade plate design has no medial offset.
The distal femoral fragment is first rotated internally to cor-
Positioning and Exposure rect the external femoral torsion and then reduced and
secured to the plate with cortical screws, correcting the varus
The operative technique for the Southwick osteotomy using a and extension deformities. The osteotomy is distal to the
130° blade plate begins with the patient placed on the operat- center, or apex, of the deformity, which lies at the level of the
ing table in the supine position. The entire lower extremity previous physis, creating an obligatory translation of the dis-
to include the groin, gluteal region, and iliac crest is sterilely tal fragment best visualized in the sagittal plane.
Fig. 29.24 (a, b) A 13-year-old female AP and frog pelvis radiographs otomy with residual anterior deformity resulting in femoral acetabular
demonstrating residual deformity after slipped capital femoral epiphy- impingement (FAI). Reprinted with permission from the Rubin Institute
sis of the left hip treated with in situ screw fixation. (c, d) AP and frog for Advanced Orthopedics, Sinai Hospital of Baltimore
pelvis radiographs after proximal femoral Southwick realignment oste-
Fig. 29.25 (a) An example of anterolateral impingement as seen from after bony impingement resection. (c, d) A similar bony resection
a surgical dislocation approach; this impingement is similar to that seen accomplished through an anterior approach without the need for com-
after realigning the residual deformity from a SCFE with an intertro- plete dislocation of the hip joint. Reprinted with permission from the
chanteric osteotomy. (b) The same femoral head as shown in Fig. 29.25a Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Fig. 29.26 (a, b) AP and frog pelvis radiographs of 18-year-old male osteotomy with concurrent open anterior femoral acetabular impinge-
with residual proximal femoral retroversion and external torsion from a ment resection. Postoperatively, the patient attained hip flexion to 110°
previous SCFE treated at an outside facility at age 13. Preoperatively, with no clinical impingement and a neutral foot progression angle dur-
the patient had hip flexion to 80° with significant impingement and con- ing gait. Reprinted with permission from the Rubin Institute for
current external foot progression angle during gait. (c, d) AP pelvis and Advanced Orthopedics, Sinai Hospital of Baltimore
lateral hip radiographs demonstrating the results after a Southwick
Femoral Neck Osteoplasty over the interval between the sartorius muscle and the ten-
sor fascia lata muscle. A typical anterior approach is per-
After completing the intertrochanteric osteotomy, all com- formed by elevating the sartorius and tensor fascia lata
ponents of the SCFE have been corrected except the antero- muscles off of the iliac crest and releasing the rectus femo-
lateral impingement. An anterior approach is performed ris tendon. The anterior hip capsule is identified and a T or
through a “bikini” type incision made in the flexion crease Z capsulotomy is performed. The anterolateral bony promi-
of the hip. The incision is approximately 2 cm below the nence is identified and removed with osteotomes and/or
anterior superior iliac spine and measures 6–8 cm centered ronguers completing the femoral neck osteoplasty
(Fig. 29.26). Anteriorly, the rectus femoris tendon is

repaired along with the insertions of the sartorius and tensor
fascia lata muscles. Laterally, the vastus lateralis muscle is
repaired to the vastus ridge and the fascia lata sutured longi-
tudinally. The superficial tissues and skin are closed in a
layered fashion over a drain.
Postoperative Care
Postoperatively, the patient is placed in a hip abduction pil-

low and converted to a single leg hip abduction brace before Fig. 29.27 (a, b) A 13-year-old female as previously depicted in
Fig. 29.24 demonstrating the preoperative and postoperative radio-
being discharged from the hospital. The patient remains with
graphs of a proximal femoral Southwick osteotomy using an Ilizarov
toe touch weight bearing for approximately 6 weeks and then external fixation device. Reprinted with permission from the Rubin
advanced to full weight bearing after radiographic confirma- Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
tion of healing at the proximal femoral osteotomy site.
Alternative Technique: Percutaneous Box 29.7. Modified Southwick Osteotomy

Osteotomy and External Fixation Technical tips:
• A 130°, zero-offset, cannulated blade plate is an
The Southwick osteotomy stabilized with an external fixa- ideal implant for the procedure.
tion device is similar in concept, but is performed in a percu- • Directing the blade plate chisel in an appropriate
taneous fashion. The initial proximal half pin is placed oblique plane is critical for correcting varus and
parallel to a proximal reference wire inserted from the tip of flexion deformities.
the greater trochanter to the center of the femoral head. This • The lower extremity can be extended, adducted,
half pin is placed directly lateral to medial at the base of the and internally rotated to achieve a correct AP posi-
greater trochanter. A femoral arch is attached to the initial tion of the proximal femur—this is achieved by
half pin and rotated the number of degrees towards the ceil- allowing the lower extremity to come off and
ing equal to the amount of sagittal plane deformity. This is slightly under the OR table on a sterile padded
the similar technique to the chisel position described above. Mayo stand; this position allows for accurate inser-
After the proximal femoral arch is placed in the perfect position of the chisel for the blade plate
tion mimicking the deformity, a second half pin is inserted • To minimize the risk of impingement, the surgeon
from an anterolateral to posteromedial direction stabilizing should consider performing an anterior approach
the proximal construct. A distal femoral arch is mounted via with anterior capsulotomy to remove the anterolat-
two transverse half pins placed perpendicular to the ana- eral “bump” at the neck-epiphyseal junction.
tomic axis of the proximal third of the femur. A third half pin • A percutaneous intertrochanteric osteotomy with
is added to both the proximal and distal arches completing application of an Ilizarov external frame is a proven,
and strengthening the final construct. A multiple drill hole alternative technique.
percutaneous osteotomy is performed through a lateral 1-cm
incision in the intertrochanteric region. The two femoral
arches are grasped like handles and the fragments are manip-
ulated correcting the deformity. The sequence of correction Residual Deformities of the Hip Joint
is rotation, translation, and then angulation. The two arches Secondary to Legg–Calvé–Perthes Disease
should be parallel or nearly parallel once the deformity is
corrected. The two arches are now connected via three to Idiopathic osteonecrosis of the epiphysis in the immature hip,
four threaded rods with conical washers (Fig. 29.27). The better known as Legg–Calvé–Perthes disease or the abbrevi-
anterior impingement excision is performed as previously ated Perthes disease, almost always leaves a mark or “foot
described. Postoperatively, the patient with external fixation print” on the involved hip even after resolution of the pro-
is allowed 50 % weight bearing immediately with concur- cess. The evolution of the deformity and final head morphol-
rent physiotherapy for range of motion of the hip. ogy depends on the age of the patient and degree of vascular
Radiographs are performed every 4 weeks to determine insult [44, 45]. The vast majority of younger patients (onset
complete consolidation of the osteotomy and eventual exter- of symptoms <6 years) with Perthes disease progress through
nal fixation removal. the natural healing process with good clinical outcome [46].
of femoral head deformity, but has a higher incidence of a

clinically significant leg length discrepancy (>2.0 cm) [48].
These young patients tend to be <4 years of age at the onset
of symptoms. The older patient (onset >7 years) has a worse
prognosis for natural resolution, but often does not develop a
clinically significant leg length discrepancy. In the authors’
opinion, this is due to the fact that the femoral neck has
established the majority of its natural length by this age. The
more common cause of leg length discrepancy in Perthes dis-
ease is iatrogenic in nature. The proximal femur varus oste-
otomy is a common treatment for Perthes disease. However,
an overly aggressive varus osteotomy can result in a signifi-
cant coxa vara, limb shortening, and a decreased articulo-
Fig. 29.28 A 16-year-old male with a history of left hip Perthes dis- trochanteric distance, which is associated with a mechanically
ease with residual coxa magna and coxa breva. Reprinted with permis-
sion from the Rubin Institute for Advanced Orthopedics, Sinai Hospital weak hip abductor mechanism causing a long lasting limp
of Baltimore [49–52]. Furthermore, the “containment” afforded by the
varus osteotomy does not unload the hip or guarantee main-
tenance of this containment in the postoperative period.
Therefore, the author does not recommend the varus osteot-
omy as the treatment of choice for Perthes disease. Instead,
for the Perthes disease that has failed conservative manage-
ment in the younger age group or in late onset Perthes dis-
ease, the author recommends articulated hip distraction via
open soft tissue releases and external fixation placement.
Details of this technique are described later in the chapter.
Coxa Breva
As previously stated, coxa breva is defined as shortening and

Fig. 29.29 A 12-year-old male with severe right hip residual defor- widening of the femoral neck, which alters the relationship
mity after conservative treatment for late onset Perthes disease. of the tip of the greater trochanter to the center of the femoral
Reprinted with permission from the Rubin Institute for Advanced
head [2]. This creates a mechanical disadvantage of the hip
Orthopedics, Sinai Hospital of Baltimore
joint by decreasing the lever arm of the hip and decreasing
the distance between the origin and insertion points of the
However, even in the case of a successful outcome with gluteus medius muscle. The radiographic presence of coxa
Perthes disease, residual changes or deformities of the hip breva is not a definitive indication for surgical correction. In
can still be present. A recent multicenter, long-term study reality, coxa breva is ubiquitous in the post-Perthes patient,
indicates Perthes disease patients treated nonoperatively but very few actually require surgical reconstruction. The
leads to pain, femoral-acetabular impingement, poor subjec- indication for surgical reconstruction is based on the patient’s
tive function, and a higher incidence of osteoarthritis than function, gait, and leg length discrepancy. Coxa breva in the
previous reports [47]. presence of a persistent Trendelenburg gait, hip fatigue, and
The most common residual deformities are coxa breva hip pain with prolonged activity requires surgical reconstruc-
and coxa magna (Fig. 29.28). tion. Surgical treatments described for coxa breva include
In the older child or in the face of an unsuccessful previ- greater trochanteric epiphysiodesis, and greater trochanter
ous treatment, the deformities can be more severe with sub- transfer (i.e., relative neck lengthening), but ideal treatment
stantial effects on patient comfort and function. These types remains ill-defined [1, 53].
of residual deformities also include coxa breva and coxa The author’s preferred method of correction of coxa breva
magna, but severe femoral head deformity, residual proximal is a femoral neck lengthening osteotomy with concurrent
and lateral displacement of the femoral head, and concurrent greater trochanteric transfer, originally described by Edwin
joint contractures are also present (Fig. 29.29). Morscher in 1980 [54] (Box 29.8). The Morscher osteotomy
Leg length discrepancy is another residual effect of improves the lever arm length of the hip joint by lengthening
Perthes disease. Paradoxically, the younger patient with the femoral neck, corrects a leg length discrepancy up to
Perthes disease has a better prognosis for natural resolution 1.5–2.0 cm, and reestablishes the center of femoral head to
tip of greater trochanteric relationship through distal transfer and extending distally for approximately 10 cm. A lateral
of the greater trochanter. The greater trochanteric transfer not approach to the proximal femur is performed by splitting the
only increases the distance between the origin and insertion tensor fascia lata and lifting the vastus lateralis muscle ante-
of the gluteus medius muscle, but it also adds lateral distance riorly. Proximally, the gluteus maximus muscle fibers should
to the mechanical lever arm of the hip joint, which imparts be dissected away from the tensor fascia allowing the gluteus
greater mechanical advantage to the gluteus medius muscle. maximus to fall posteriorly. This allows for better visualiza-
For the Morscher osteotomy to be successful, the hip joint tion of the gluteus medius muscle and easier dissection of the
should be congruent with full range of motion with no evi- greater trochanteric fragment-gluteus medius muscle com-
dence of femoral acetabular impingement. The following is a plex later in the procedure.
brief description of the author’s preferred technique for the
Morscher osteotomy.
The Morscher Osteotomy for Coxa Breva The internal fixation of choice is a 130° cannulated blade
Secondary to Perthes Disease: Operative plate. After exposure of the lateral aspect of the proximal
Technique femur, a central threaded guide pin is inserted up the femoral
neck and into the femoral head. This guide wire represents
Positioning and Exposure the eventual path of the blade plate and the new femoral neck
orientation. Three additional guide wires are inserted parallel
The operative technique for the Morscher osteotomy begins to the initial guide wire. The second guide wire is inserted at
with the patient placed on the operating table in the supine the level of the superior base of the femoral head, which rep-
position. The entire lower extremity to include the groin, gluteal resents the new level of the superior border of the femoral
region, and iliac crest is sterilely prepared and draped (Fig. 29.30). neck. The third wire is inserted approximately 1 cm above
A lateral longitudinal incision is performed starting the second wire. This represents the new base of the greater
approximately 5 cm above the tip of the greater trochanter trochanter (Fig. 29.31).

pictures depicting the typical
patient position and draping for
the Morscher osteotomy.
Baltimore
Fig. 29.31 (a) AP pelvis radiograph demonstrating severe coxa breva the superior border of the new femoral neck, the third wire marks the
of the left hip. (b, c) The same patient undergoing left hip Morscher trochanteric osteotomy site, the fourth wire marks the inferior border of
osteotomy with intraoperative fluoroscopic views demonstrating the the new femoral neck. Reprinted with permission from the Rubin
placement of the guide wires. The first guide wire is inserted at an angle Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
of 130° and marks the path for the blade plate, the second wire marks
Fig. 29.32 A fluoroscopic image demonstrating the completion of the Fig. 29.33 Fluoroscopic image demonstrating the final osteotomy cut
first two osteotomy cuts allowing mobilization of the greater trochanter, that creates the inferior border of the new femoral neck and the inser-
removal of interval bone segment, and the creation of the new superior tion of the 130-degree blade plate. Reprinted with permission from the
border of the femoral neck. Reprinted with permission from the Rubin Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
The interval bone segment between the second and third

wire will be removed and used as bone graft. The final guide
wire is inserted at the level of the inferior border of the femo-
ral neck. This final guide wire represents the sliding osteot-
omy surface that will result in femoral neck lengthening as
well as lower extremity lengthening. Again, it is important
that the guide wires are inserted in a parallel or near parallel
fashion. Next, the appropriate sized blade plate chisel is
driven over the central guide wire. The chisel should be held
in neutral rotation in the sagittal plane to prevent secondary
flexion or extension of the osteotomy that would result in
minimal contact between the proximal and distal fragments.
Once the chisel is fully seated, the appropriate blade length
is calculated by adding an additional 1–1.5 cm of length to
the chisel length. An oscillating saw is used to cut the bone
along the two proximal and the distal most guide wires. The
first cut is the most proximal osteotomy is used to mobilize
the greater trochanteric fragment along with the gluteus
medius muscle. The second cut creates the new superior bor-
der of the femoral neck allows for mobilization and removal Fig. 29.34 Fluoroscopic image showing the reduction of the distal
femoral segment reduced to the blade plate that extended out of the
of the interval bone segment (Fig. 29.32). proximal segment by 1.0–1.5 cm, which results in a lengthened femoral
This bone should be placed into a moist sponge for later neck. Reprinted with permission from the Rubin Institute for Advanced
use. The final osteotomy creates the distal border of the fem- Orthopedics, Sinai Hospital of Baltimore
oral neck separates the proximal femoral segment from the
distal femoral shaft. After the osteotomies are completed, the The distal fragment is then reduced to the plate with bone
chisel is removed and the selected blade plate inserted over holding forceps. This maneuver causes the distal fragment to
the initial threaded guide wire. The blade should be driven to slide down the oblique osteotomy, accomplishing femoral
a level that leaves approximately 1–1.5 cm of blade exposed neck lengthening, as well as lower extremity lengthening
laterally (Fig. 29.33). (Fig. 29.34).
Fig. 29.35 (a, b) Fluoroscopic

images demonstrating the
advancement of the greater
trochanter with temporary wire
fixation and final fixation with a
cannulated screw and tension
wire. Reprinted with permission
The previously removed bone fragment is now notched shifted slightly anterior or posterior to allow enough space
and placed over the exposed end of the blade plate at the for the eventual trochanteric cannulated screw to pass with-
130° bend. out difficulty (Figs. 29.36 and 29.37).
The final step of the procedure is the mobilization and
advancement of the greater trochanter both laterally and dis-
tally. The appropriate level of greater trochanteric transfer is Box 29.8. Morscher Osteotomy for Coxa Breva
accomplished when the tip of the greater trochanter is at or Technical tips:
below the level of the center of the femoral head. This posi- • The initial guide wire is inserted at an angle of 130°.
tion is obtained by carefully mobilizing the gluteus medius This is the angulation of the new femoral neck.
muscle and abducting the hip 10–15° before fixation. The • A 130-degree, zero-offset, cannulated blade plate is
greater trochanteric fragment is stabilized with a cannulated an ideal implant for the procedure.
5.5-mm screw and washer, tension band wiring, or a combi- • The length of the blade plate is determined by mea-
nation of both techniques (Fig. 29.35). suring the initial guide wire inserted up the femoral
The operative incision is closed as previously described neck and adding 10–15 mm to this measurement.
in a layered fashion over a drain. • When the blade plate is inserted, the plate is left
away from the lateral cortex by 10–15 mm; this will
allow the distal fragment to shift laterally, lengthen-
Postoperative Care ing the femoral neck.
• It is critical not to translate the distal segment more
The patient is placed in a hip abduction pillow immediately than 50 % of the original diameter of the subtro-
and converted to a single leg hip abduction brace before dis- chanteric width of the femur. If greater than a 50 %
charge from the hospital. The patient is maintained at toe translation is performed, inadequate bone contact
touch weight bearing status and full time brace wear for the will be present and an increased risk for implant
initial 6 weeks. After 6 weeks, the brace is used at night for failure will occur.
an additional 6 weeks and gradual advancement of weight • The trochanteric fragment must be carefully mobi-
bearing, hip range of motion, and hip strengthening begins lized without compromising the innervation of the
under the supervision of a physiotherapist. gluteus medius muscle.
Two key tips for the Morscher osteotomy are the amount • The interval bone segment removed from the supe-
of lateral displacement of the distal fragment and the initial rior femoral neck osteotomy should be saved for
position of the chisel in the sagittal plane. First, the distal femoral neck bone grafting around the proximal
femoral fragment should not be translated laterally more bend of the 130-degree blade plate.
than 50 % of the original diameter of the proximal femur. If • The greater trochanter should be advanced until the
the bone-to-bone contact is less than 50 % of the original proximal tip is at or below the center of the femoral
diameter, there is a significant increase in implant failure. head.
Second, the chisel position in the sagittal plane should be
Fig. 29.36 Step-by-step schematic summary of the Morscher osteotomy. Reprinted with permission from the Rubin Institute for Advanced
Fig. 29.37 (a, b) A 12-year-old female with severe left hip coxa breva frog pelvis of a 13-year-old male with residual right hip deformity from
from previous AVN. This is the same patient depicted in Figs. 29.31, Perthes disease. (h, i) Immediate postoperative AP and lateral radio-
29.32, 29.33, 29.34, and 29.35. (c, d) One year post-reconstruction of graphs of the right hip after Morscher osteotomy. (j, k) Five-year fol-
the left hip using the Morscher osteotomy technique. (e) Three years low-up of the same patient after internal implant removal. Reprinted
post-reconstruction AP pelvis radiograph after internal implant with permission from the Rubin Institute for Advanced Orthopedics,
removal. (f, g) Another example of coxa breva demonstrated by AP and Sinai Hospital of Baltimore
to be related to the amount of epiphyseal involvement and

Coxa Magna the age of the patient. However, there is significant variation
to the pattern of collapse between individual patients and an
As previously stated, coxa magna is a common residual exact method for predicting the amount of eventual collapse
deformity of Perthes disease. Coxa magna is defined as has not been developed. The second variable in the develop-
the enlargement and deformation of the femoral head. ment of coxa magna is the abnormal articular cartilage
This deformity can result in the loss of congruency between growth. This abnormal growth is caused by asymmetric
the femoral head and acetabulum resulting in femoral-ace- pressure applied to the growing femoral head. The cause of
tabular impingement (FAI). Severe coxa magna causes pain, the abnormal femoral head growth and deformation is
loss of motion, labral and cartilage degeneration, and even- directly related to the loss of hip joint motion and the pres-
tual osteoarthritis [3–6]. Coxa magna is caused by a combi- ence of a significant hip joint contracture. In the stiff hip that
nation of the collapse of the proximal femoral epiphysis and has lost significant range of motion, the joint pressures are
altered growth of the articular cartilage (Fig. 29.38). asymmetric over the femoral head. The zone of contact
The coxa magna deformity begins as the femoral head between the femoral head and the superior articular surface
settles after the initial subchondral collapse of the epiphysis. of the acetabulum and acetabular rim represents areas of
This results in a decreased height and an increased width of increased pressure, whereas the portions of the femoral head
the femoral head, similar to applying downward pressure on that are anterior and lateral to the acetabular rim and medial
a water balloon. The amount of continued collapse appears within the acetabular fossa are areas of decreased pressure.
Fig. 29.38 (a, b) Radiographic

example of a coxa magna
deformity resulting in loss of
congruency of the hip joint and
femoral acetabular impingement.
Baltimore
Fig. 29.39 (a, b) AP and frog

pelvis radiographs demonstrating
a congruent coxa magna and
oblong acetabulum in a patient
after late-onset Perthes disease.
Baltimore
The areas of increased pressure obviously expedite any col- author’s experience. Even mild changes to the overall diameter
lapse as the underlying epiphysis loses structural integrity. or shape of the femoral head are not tolerated in this age
Furthermore, this pressure also compresses and slows the group. Although these patients have initial good results, the
articular cartilage growth. Conversely, the areas of decreased mild to moderate incongruence of the hip joint will eventu-
pressure allow the articular cartilage to grow into the ally lead to FAI and the need for hip reconstruction to avoid
“unweighted” zones, thereby exacerbating the femoral head early degenerative changes.
deformation and coxa magna formation. The treatment of choice for significant coxa magna defor-
Another crucial variable in the evaluation of the coxa mity of the femoral head is the femoral head reduction oste-
magna deformity is the adaptive remodeling changes of the otomy, also known as the “head splitting” or “head reshaping”
acetabulum. In young patient (<6 years) with Perthes disease, osteotomy, initially described by Ganz [55]. This relatively
full collapse of the epiphysis can occur with coxa magna for- new technique utilizes Ganz’s well-described surgical hip
mation, but the femoral head remains spherical and the hip dislocation approach, and has shown promising early results
joint congruent. It is the author’s experience and opinion that [36, 56–58].The following is a brief description of the opera-
this outcome is directly related to the maintenance of ade- tive technique for the femoral head reduction osteotomy
quate hip range of motion during the entire healing process of (Box 29.9).
the femoral head. The only true method to maintain “contain-
ment” of the femoral head is to maintain adequate hip range
of motion. Adequate hip range of motion is defined by the The Femoral Head Reduction Osteotomy
author as hip motion that is >50 % of the contralateral normal for Coxa Magna Secondary to Perthes
hip. Even in the face of an enlarged and oblong femoral head, Disease: Operative Technique
adequate range of motion of the hip joint allows the acetabu-
lum to remodel into a larger and oblong congruent joint. This Positioning and Exposure
situation can provide a pain-free functional hip joint for
several decades with excellent quality of life (Fig. 29.39). The patient positioning and operative approach is identical
However, in older patients (>10 years for females and to the surgical hip dislocation as described above for the
>12 years for males), even with successful maintenance of modified Dunn procedure. After the capsulotomy and gen-
hip range of motion and a relatively spherical femoral head, tle dislocation of the hip, the acetabulum and labrum are
the amount of acetabular remodeling is minimal in the inspected for any pathologic changes. With the femoral head
Fig. 29.40 (a, b) AP and frog pelvis radiographs depicting the same the true three-dimensional shape of the femoral head does not consis-
patient in Fig. 29.28, a 16 year old male with coxa magna, coxa breva, tently correlate with various imaging studies; therefore, MRI and CT
and concurrent FAI. (c, d) Clinical appearance of the femoral head of the scans are rarely indicated or performed. Reprinted with permission from
same patient after surgical dislocation. It is the author’s experience that the Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
a b c
Fig. 29.41 (a–c) Schematic representation of the femoral head reduc- planning lines that resolves a three-dimensional puzzle into two sepa-
tion/reshaping osteotomy depicting the creation of a spherical femoral rate pieces that can be placed together to form a spherical object.
head by excising an interval deformed section of bone. (d–f) Clinical Reprinted with permission from the Rubin Institute for Advanced
pictures of another case demonstrating the femoral head osteotomy Orthopedics, Sinai Hospital of Baltimore
dislocated, the posterolateral soft tissue leash is inspected for Deformity Correction and Fixation
any undue tension and the exact three-dimensional head
shape is visualized for planning the osteotomy (Fig. 29.40). The femoral head cut is rarely in a pure anterior to posterior
Any cartilage softening and depressed areas are noted as direction. Usually the cut is from an anterolateral to a pos-
the femoral head is inspected. These areas need to be teromedial direction. The greater the amount of sagittal
included in the segmental resection of the mid-portion of the plane deformity, the more lateral the starting point for the
femoral head. Femoral head sizing templates, surgical osteotomy needs to be placed. The planned resection of bone
experience, and three dimensional awareness are used to can be in a shape of a wafer or a wedge depending on the
determine the exact position of the femoral head cuts deformity to be corrected. The greater the sagittal plane
(Fig. 29.41). deformity present, the more likely a wedge shape is needed
to completely correct the oblique plane deformity. Two of central free fragment from the medial head fragment that is
K-wires are inserted to represent the two longitudinal cuts still attached to the intact medial femoral neck, and the lateral
(Fig. 29.42). mobile femoral head segment attached via the soft tissue vas-
The most medial cut is performed first with great care to cular leash. The middle segment is carefully removed and the
leave greater than a third of the original diameter of the fem- lateral fragment mobilized (Fig. 29.44).
oral neck intact. Rotating the saw blade in a counterclock- The lateral fragment is reduced to the stable medial head
wise direction allows a greater segment of the medial neck to fragment and held with pointed bone reduction forceps.
remain intact for stability of the medial head fragment. The The lateral femoral head fragment usually requires proxi-
second osteotomy is now performed. This bone cut is the mal advancement along with the medial translation. The
closest to the posterolateral segment containing the blood vascular leash is inspected for tension or tenting over the
supply; so great care is needed to avoid the retinacular ves- residual base of neck. If a significant medial translation or
sels entering in the lateral femoral head segment. The final closing wedge is needed, the posterolateral base of femoral
transverse bone cut is performed at the base of the femoral neck must be reduced in size to prevent impingement and
neck, connecting the first two vertical bone cut (Fig. 29.43). tension on the vascular leash. After the femoral head is
Again, this transverse cut must be performed with great reduced and vascular supply inspected, three transverse
care to avoid the intact medial neck and the posterolateral headless screws are inserted to secure the femoral head
head-neck junction where the blood supply is entering the osteotomy (Fig. 29.45a, b).
lateral femoral head segment. This final cut creates separation The gap between the base of the lateral femoral head frag-
ment and the femoral neck is filled with a bone graft obtained
from the removed femoral head fragment. The femoral head
is gently reduced into the acetabulum and joint motion is
assessed. Any residual impinging bone of the anterior femo-
ral head or neck is excised. The capsule is repaired with
absorbable heavy suture and suture anchors at the base of the
femoral neck as needed. It is crucial not to over tighten the
capsular repair that can place undue tension on the posterior
capsule and thereby on the vascular leash.
The hip is placed in mild abduction and the greater tro-
chanter is advanced distally until the tip of the greater tro-
chanter is equal to or below the center of the newly shaped
femoral head. The greater trochanter is secured via two 5.5-
mm cannulated screws. It is crucial to direct the screws away
from the medial femoral neck base to prevent propagation of
Fig. 29.42 Two guide wires are placed at the planned longitudinal
a fracture form the screw hole to the medial femoral neck
osteotomy sites. Reprinted with permission from the Rubin Institute for which will result in destabilization the medial head fragment
Advanced Orthopedics, Sinai Hospital of Baltimore (Fig. 29.45c, d).
Fig. 29.43 Clinical pictures

denoting the two longitudinal
cuts (a) and the transverse cut (b)
that create the mobile lateral
femoral head fragment attached
via the vascular leash. Reprinted
Baltimore
The incision is closed in a routine layered fashion with the

tensor fascia lata, subcutaneous tissues, and skin closed over
a drain. The patient is gently placed into a supine position
with an abduction pillow and a final radiograph is performed
(Fig. 29.46).
Postoperative Care
Postoperatively, the patient is placed in a single-leg abduc-

tion brace full time except when using a continuous passive
motion (CPM) machine for hip flexion and extension. The
CPM machine is used 2–4 h during the day and all night for
the first 6 weeks. Alternatively, the patient may use the CPM
for a greater time during the day and utilize the hip abduction
brace at night. The protocol can be adjusted per the patient’s
preference. Physical therapy consists of simple passive range
of motion of the hip not to exceed the following parameters:
75° of hip flexion, 30–40° of hip abduction, full hip exten-
sion, zero hip adduction, and 30° of internal rotation and 30°
Fig. 29.44 Clinical picture of the excised middle segment. The bone at of external rotation. Active sitting and CPM motion can be
the base of the cut can be utilized as bone graft in the femoral neck. maximized, increasing the hip flexion to the patient’s toler-
Reprinted with permission from the Rubin Institute for Advanced ance. The CPM and daytime bracing are discontinued
6 weeks postoperatively. Toe touch weight bearing is man-
dated for 3 months to ensure complete healing of the femoral
head (Figs. 29.47 and 29.48).

pictures of the final configuration
of the femoral head after fixation
of the medial and lateral head
fragments with three transverse
headless screws. (c, d)
Fluoroscopic images of the final
femoral head fixation, hip
reduction, and greater trochanter
fixation. (e) Typical headless
screw used to fix the femoral
head osteotomy. Reprinted with
Baltimore
Fig. 29.46 (a, b) Preoperative and postoperative AP pelvis radio- tive frog pelvis radiographs. (e, f) Preoperative and postoperative clini-
graphs after surgical dislocation of the hip with femoral head reduction cal pictures. Reprinted with permission from the Rubin Institute for
and transfer of the greater trochanter. (c, d) Preoperative and postopera- Advanced Orthopedics, Sinai Hospital of Baltimore
Fig. 29.47 (a, b) Radiographic example of a 12-year-old female with reduction and reshaping, and greater trochanter transfer. Reprinted with
severe impingement secondary to residual deformity of the femoral permission from the Rubin Institute for Advanced Orthopedics, Sinai
head after late-onset Perthes disease of the left hip. (c, d) Same patient Hospital of Baltimore
after left hip reconstruction with surgical dislocation, femoral head
Fig. 29.48 (a, b) A 12 year-old male with residual right coxa magna femoral head reduction osteotomy and greater trochanter transfer.
resulting in an incongruent hip and FAI secondary to Perthes disease. Reprinted with permission from the Rubin Institute for Advanced
(c, d) Radiographs of same patient after right hip reconstruction via a Orthopedics, Sinai Hospital of Baltimore
Box 29.9. Femoral Head Reduction Osteotomy Residual Femoral Head Subluxation
Technical tips:
• Adequate thickness of the greater trochanter oste- Residual femoral head subluxation or migration can result
otomy must be maintained for later fixation from untreated or unsuccessful treatment of Perthes disease.
(1–1.5 cm width). The residual femoral head subluxation is truly a migration or
• The most critical aspect of the exposure is creating “settling” of the joint as the femoral head deforms from both
and maintaining the posterior retinacular soft tissue internal collapse and abnormal femoral head cartilage
sleeve to the lateral femoral head segment. growth. This migrated joint position is exacerbated by con-
• Removing 5–8 mm of bone from the posterior mar- current soft tissue contractures, typically hip flexion and hip
gin and the base of the greater trochanter will aid in adduction contractures. If this abnormal joint position is
relieving tension on the vascular flap. prolonged, then permanent femoral head deformity will
• The determination of the medial and lateral head result. The femoral head that demonstrates significant resid-
fragments is performed with femoral head sizing ual deformity after the reconstitution or healing phase can
templates and intra-operative three-dimensional only be corrected with the femoral head reduction osteot-
analysis. omy described above. However, if the femoral head is still
• The medial longitudinal femoral head osteotomy within the active phase of Perthes (fragmentation or early
should leave at least 1/3 of the femoral neck intact. reconstitution) and the hip joint is contracted with residual
• The lateral longitudinal femoral head osteotomy femoral head migration, then a specialized treatment exists
should be placed to remove all interval damaged/ that can salvage this situation. This treatment consists of a
deformed femoral head; the goal is to reduce the hip distraction protocol that consists of arthrodiastasis of the
femoral head to a size that is completely contained hip joint using a hinged external fixation device and selec-
within the acetabulum. tive soft tissue releases. Although there is limited prospec-
• The transverse femoral neck osteotomy that creates tive data on hip arthrodiastasis, the concept of using
the free interval and lateral femoral head fragments articulating hip distraction as an ideal means to reduce
should not violate the medial femoral neck. deforming joint pressures, and also maintain range-of-
• If the residual femoral neck is less than 1/3 the orig- motion, has been described [59, 60]. Adjunctive procedures
inal diameter, then a retrograde 4.5-mm screw include a multiple small diameter core decompression and
should be inserted from the proximal femoral autogenous bone stem cell graft injection. The author has
metaphysis into the medial femoral head fragment. achieved a high rate of success for the most severe late onset
• The femoral head osteotomy is stabilized with three Perthes patients after failed previous treatment, and thus uti-
to four 3.0 mm/4.3 mm headless screws. lizes this protocol for primary treatment of Perthes patients
• The mobile, lateral femoral head fragment is in high-risk groups and those that have failed early conser-
advanced proximally and medially to the stable vative treatment methods (Box 29.10).
medial femoral head fragment; the excised bone
should be used as bone graft under the lateral femo-
ral head fragment. Hip Distraction Treatment for Residual
• The hip capsule should be repaired with minimal Femoral Head Subluxation Secondary
tension to avoid stretch of the vascular supply to the to Perthes Disease: Operative Technique
femoral head.
• The greater trochanter should be advanced until the Patient Positioning
proximal tip is at or below the center of the femoral
head. The patient is placed supine on the operating room table after
• The greater trochanter is repaired with two 4.5-mm an epidural catheter is inserted. There are no bumps placed
or 5.5-mm screws with washers. under the patient’s hip or sacrum. The involved extremity is
• It is critical that the fixation screws for the greater sterilely prepared to include the entire lower extremity, glu-
trochanter are directed distal away from the residual teal region, and iliac crest as proximal as the subcostal mar-
medial intact femoral neck to prevent postoperative gin. A sterile Mayo stand with a pillow is prepared to allow
femoral neck fracture. for positioning the leg in adduction during pertinent parts of
the procedure.
Fig. 29.49 Fluoroscopic views

of a small-diameter core
decompression. Reprinted with
Baltimore
Fig. 29.50 Intraoperative

fluoroscopic image
demonstrating the insertion of the
fenestrated cannula into the
femoral epiphysis with injection
of bone stem graft. The bone
stem cell graft is visualized by
adding a small amount of dye,
which appears as the darkened
epiphysis. Reprinted with
Baltimore
Core Decompression The bone stem cell graft is then injected into the femoral
epiphysis via the fenestrated cannula. Prior to injection, the
The first step of the procedure consists of small diameter bone stem cell graft is diluted with 1 cc of radiopaque dye to
core decompression of the femoral head in order to stimulate allow visualization of the graft distribution under fluoros-
a healing response within the epiphysis. A 1.8-mm wire is copy (Fig. 29.50).
inserted percutaneously from the lateral thigh and driven up If dye is seen diluting into the joint, the cannula is read-
the femoral neck under fluoroscopic guidance into the femo- justed as necessary and the injection commences.
ral epiphysis (Fig. 29.49).
This is repeated 5–6 times in order to cover all affected
zones of the epiphysis. Soft Tissue Releases
The leg is placed into a “frog leg” position and an adductor

Stem Cell Injection tenotomy is performed through a small stab incision. The
adductor longus muscle and gracilis muscle are released dur-
The second surgical step begins with harvesting bone mar- ing this percutaneous tenotomy. The adductor longus muscle
row aspirate from the iliac crest. A 3-mm trephine needle is released with the hip and knee flexed, whereas the gracilis
is used to aspirate approximately 60 cc of bone marrow, muscle is released with the hip flexed but the knee extended.
followed by centrifuge preparation to separate the bone stem In a heavier patient, the physician may alternatively make a
cells. Next, a 1.5-mm wire is driven into the anterolateral 2- to 3-cm mini-open approach to perform adductor releases
portion of the femoral epiphysis. Via a small stab incision, under direct visualization.
the 1.5-mm wire is overdrilled with a 3.2-mm cannulated Following adductor releases, the leg is placed back into a
drill. A fenestrated cannula is inserted into the bony channel neutral supine position and a 3-cm anterior oblique incision
and manually pushed, or carefully tapped with a mallet, into is performed in the flexion crease of the groin approximately
the epiphysis. 2 cm below the anterior superior iliac spine. The incision
Fig. 29.51 Intraoperative

fluoroscopic views of the right
hip after intra-articular injection
of dye. The actual femoral head
deformity can be visualized and
documented. Reprinted with
Baltimore
starts medially at the point of the palpable femoral artery and to the pelvis by two supra-acetabular half pins. The distal pin
extends laterally. The interval between the sartorius muscle clamp is utilized for placement of two half pins into the fem-
and tensor fascia lata muscle is palpated and the fascia over- oral shaft. Avoid placing the distal half pin anteriorly near the
lying the sartorius muscle is opened. This fascia is divided cortex, as this places the patient at risk for femoral shaft frac-
from lateral to medial until the septum separating the sarto- ture. After securing the device to the half pins, a second arch
rius muscle and iliopsoas muscle is identified. The fascia is secured to the distal half pins. The hinge pin may now be
overlying the iliopsoas muscle is divided and the muscle is removed and fluidity of the articulation checked by passive
carefully retracted laterally. By gently pulling the iliopsoas hip flexion and extension to ensure a properly applied frame.
muscle laterally, the femoral nerve comes into the operative The final femoral fixation point is placed at the level of the
field and is easily identified. The fascia overlying the femoral lesser trochanter built off the posterolateral distal arch. The
nerve is divided and the medial and lateral borders of the final pelvic fixation point is inserted from an oblique anterior
nerve are dissected. The overlying fascia proximal and distal to posterior direction under fluoroscopic control down the
along the nerve is released allowing the nerve to mobilize. posterior column of the pelvis (Fig. 29.52).
The femoral nerve is retracted medially and the iliopsoas After completing external fixator application, the hip is
muscle is retracted laterally and everted exposing the ilio- acutely distracted 8–10 mm. A modular, removable anterior
psoas tendon. The tendon is lifted into the operative field hip extension bar is attached to the anterior portion of the
with a right angle clamp and sharply released under direct proximal and distal arches.
visualization. The incisions are closed in a typical layered
fashion and an arthrogram of the hip is performed. The hip is
examined under fluoroscopy to determine the exact shape of Postoperative Care
the femoral head, position of the labrum, and containment of
the femoral head with 20° of hip abduction (Fig. 29.51). The postoperative protocol consists of continued gradual dis-
traction at the rate of .5 mm per day for 7–10 days if needed.
The goal of distraction is to over reduce Shenton’s line by
Hip Distractor Application 5–10 mm. In smaller patients, the acute intra-operative dis-
traction is all that is needed. Physical therapy is employed to
The hinged external fixation device (Orthofix articulated hip maintain hip flexion and extension and mobilization with
distractor) is placed by inserting half pins into the pelvis and 50 % partial weight bearing. The anterior extension bar is used
into the middle third of the femoral shaft. The leg is posi- for 2–3 h during the day and all night for the duration of the
tioned on the prepared mayo stand with the hip in full exten- external fixation. The external fixation device is maintained
sion, neutral rotation, and 20° of abduction. A 3/32 Steinman for 4 months. Frame removal is performed under general anes-
pin is inserted perpendicular to the anatomic axis of the fem- thesia with a repeat arthrogram and Botox injection into the
oral shaft 8–10 mm below the center of the femoral head, and adductor longus and gracilis muscles. The dosage of Botox is
serves as the temporary hinge guide pin In the sagittal view, typically 10 units per kilogram of weight, with a max dose of
the position of the guide pin is confirmed to be in the center 200 units. Post-frame removal protocol consists of abduction
of the femoral head. The hinged external fixation device is hip bracing with a Scottish Rite brace and hip mobilization
placed over the hinge guide pin. The proximal arch is secured with physiotherapy and home exercises (Fig. 29.53).
Fig. 29.52 (a) An AP pelvis radiograph demonstrating a typical articu- distraction gap of 8–10 mm. (c) Clinical picture of the articulated hip
lated hip external fixation device application for arthrodiastasis. (b) distracting external fixation (Orthofix). Reprinted with permission from
Another example of articulated hip distraction demonstrating an adequate the Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Fig. 29.53 (a, b) An 11-year-old male with severe residual deformity in joint distraction prior to removal of external fixation; the radiograph
late fragmentation stage of Perthes disease; the patient had failed a previ- demonstrates significant reossification of the femoral head. (f, g) One
ous shelf arthroplasty at an outside institution. (c) Intraoperative arthro- year postoperative AP and frog pelvis radiographs demonstrating excel-
gram demonstrating lateral and proximal migration of the femoral head lent healing and sphericity of the femoral head with concurrent excellent
and true “hinge abduction.” (d) Radiograph 6 weeks following core congruency of the hip joint. (h, i) Three-year follow-up with excellent
decompression, open iliopsoas muscle release, percutaneous hip adduc- right hip healing and congruency; the patient continues to be pain free
tor tenotomy, and articulated hip distraction with full reduction of the hip and participates in all activities. Reprinted with permission from the
joint and early reossification. (e) AP pelvis radiograph after 4 months of Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore
Achieving consistent excellent results in treating avascu-

Box 29.10. Hip Distraction with a Hinged External lar necrosis of the immature femoral head continues to elude
Frame the pediatric orthopedic community. Surgical intervention
Technical tips: during the early fragmentation stage of Perthes may be cru-
• Arthrodiastasis is best performed simultaneously cial to preserving femoral head morphology and improving
with adjunctive procedures: soft tissue contracture long-term outcome. Currently, an appropriately applied hip
releases, core decompression, autograft bone stem distraction protocol may be the only treatment method that
cell injection. simultaneously provides containment as well as relief from
• Six multiplanar 6-mm half-pins are used to stabilize deforming joint pressures, while allowing maintenance of
the fixator: two supra-acetabular pins, one oblique range-of-motion. Further investigation is needed into hip
posterior column pin, two femoral shaft pins, and arthrodiastasis and adjunctive treatments such as core
one posterolateral pin at the lesser trochanter. decompression and stem cell injection to optimize the treat-
• Acutely distract the hip 8–10 mm, followed for ment protocol described in this chapter.
gradual distraction as needed to achieve a distrac- Avascular necrosis of the femoral head leads to proximal
tion gap of 5–10 mm. femur deformities, namely coxa breva and coxa magna.
• A removable, modular extension bar will help pre- Coxa breva negatively alters proximal femur biomechanics,
vent hip flexion contracture; the hip extension bar is and is associated with hip abductor weakness, limb length
inserted for 1–2 h during the day and all night discrepancy, and limping. In contrast to a relative neck
• Postoperative physiotherapy both prior to and after lengthening procedure, the Morscher osteotomy is a true
fame removal is paramount to optimizing neck lengthening osteotomy and directly addresses the fem-
outcome. oral neck deficiency. Intracapsular femoral head reshaping
• After the external fixation is removed, the adductor osteotomies are technically demanding, but provide the only
muscles are injected with Botox, a Scottish Rite hip reconstructive option for a symptomatic coxa magna in the
abduction orthosis is placed, and immediate mobili- severely deformed and incongruent femoral head.
zation with “Perthes exercises” are performed.
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Posttraumatic Lower Limb
Deformities in Children 30
Ashok N. Johari, Sandeep A. Patwardhan,
and Taral Vishanji Nagda
can be observed. In general, when the residual axial malalign-

Introduction ment of a lower extremity long bone exceeds 10°, correction
may be warranted.
Posttraumatic deformities in children are unique. They are
different from deformities that are congenital or secondary
to other causes since the skeletal and soft-tissue anatomy Causation and Etiological Factors
and physiology are often normal to start with. Hence, repar-
ative mechanisms and many management principles differ
in these patients.
Posttraumatic deformities in children are different from Box 30.1. Factors Related to Development of
adults. Children have the potential to influence future growth Posttraumatic Deformities
due to the possibility of substantial remodeling of a mal- • Injury related
united fracture. Such remodeling can occur from different – Soft tissue
sources including the adjacent physis and differential perios- Skin and soft-tissue loss
teal growth of the affected diaphysis (Fig. 30.1). On the other Tendon and muscle injury
hand, injury to a portion of the growth plate can lead to Nerve injury
asymmetric growth of the physis with progressive deformi- Vascular injury
ties in children. – Skeletal
Deformities following trauma to the extremities are not Type of fracture, fracture geometry, and comminution
uncommon and not always dependent on the level of care pro- Growth plate injury
vided. While some deformities are inevitable due to high- Bone loss
energy trauma and damage to the growing area of long bones, Intra-articular injury
other deformities occur because of suboptimal management • Treatment related
allowing the limb to be positioned abnormally or allowing cer- – Missed diagnosis of a fracture
tain muscles to get tight and create a secondary deformity. – Delayed presentation and management
Deformities that are not clinically significant or have the – Incorrect treatment choice
potential for substantial remodeling to an acceptable level – Suboptimal management
– Overtreatment
A.N. Johari, MS(Orth), M.Ch Orth, FRCS, FAMS (*) – Iatrogenic problems
Department of Paediatric orthopedics, Children’s orthopedic • Complication related
Centre, Bobby Apartment, L. J. Road, Mahim, – Infection
Mumbai, Maharashtra 400 016, India
– Loss of reduction
– Compartment syndrome
S.A. Patwardhan, MS(Orth), D Orth
– Postoperative stiffness
Department of Pediatric orthopedics, Sancheti Institute
for orthopedics and Rehabilitation, Pune, Maharashtra, India – Avascular necrosis
T.V. Nagda, MS, DNB, D Ortho (continued)
Department of Pediatric orthopedics, Hinduja Hospital Mumbai,
Mumbai, Maharashtra, India

570 A.N. Johari et al.
Box 30.1. (continued) Box 30.2. Etiological Mechanisms for Posttraumatic

• Patient-related factors Deformities
– Preexisting skeletal problems like dysplasia, • Postural or positional
syndromes • Muscle forces
– Neuromuscular problems like cerebral palsy, • Neural and vascular injury
poliomyelitis, muscular dystrophy • Poor skeletal stabilization
– Altered bone physiology due to osteogenesis • Damage to the growing area of bone
imperfecta, metabolic bone disease • Infection
– Pathological fracture with underlying tumor,
osteomyelitis
– Immunocompromise
– Malnutrition Deformities can be caused by one or more of the follow-
ing etiological factors:
1. Postural or positional: Lack of immobilization or faulty

immobilization may cause a deformity, often related to
contracture of the adjacent soft tissues. Examples are an
equinus deformity of the foot or a knee flexion deformity
because of contracture of the gastro-soleus or the ham-
strings, respectively.
2. Muscle forces: Varus deformity of the upper femur follow-
ing a fracture is a classic example of muscle forces at work.
The flexor-adductor predominance over the abductor-
extensor forces causes this deformity (e.g., Figs. 30.1
and 30.2).
3. Neural or vascular injury: Neural injury can create defor-
mity because of imbalance of muscle forces or by muscle
paralysis causing a postural deformity. Vascular injury may
give rise to infarction of muscle followed by its fibrosis and
eventually a contracture. Volkmann’s ischaemic contracture
is a typical example of such an etiology.
4. Poor skeletal stabilization, loss of reduction, and loss of
fixation can all give rise to posttraumatic deformities.
Factors such as poor bone quality, traumatic bone loss,
and fracture comminution may contribute to inadequate
skeletal stabilization (e.g., see Fig. 30.9).
5. Damage to the growing area of bone: Classic examples of
Fig. 30.1 Four-year-old child with femur fracture treated by traction these are partial physeal injuries with growth arrest giving
in a Thomas’ splint. (a) Injury film. (b) X-ray in Thomas’s splint. (c) At
rise to various angular deformities (e.g., see Fig. 30.7).
2 months fracture united with 40° angulation and 3 cm shortening. The
malunion is due to muscle forces that were not effectively managed Symmetric physeal damage would give rise to limb short-
ening. Damage to the growing area may be a consequence
of the mode of injury or may occur because of its poor
A posttraumatic deformity can be a result of initial soft- management. Salter–Harris type 6 injury is a perichon-
tissue and skeletal injury, its management, or complications. drial ring injury presenting with a normal X-ray. This is
In order to accurately identify the cause and anticipate the an example of the mode of injury giving rise to deformity
natural progression of the deformity, it is important to ascer- (e.g., see Fig. 30.10).
tain the mechanism of injury, initial soft-tissue and bony 6. Infection: Open skeletal injuries or iatrogenic issues can
injury, specifics of the treatment given—conservative and be complicated by infection which works by damaging
operative, postoperative care, and complications and course bone and cartilage, causing bone lysis and loss, growth
of the deformity thus far. plate damage and its sequelae, as well as joint ankylosis
and deformity.
30 Posttraumatic Lower Limb Deformities in Children 571
Classification X-rays in the plane of the deformity. Full-length standing

radiographs and scanograms are useful for monitoring
Posttraumatic deformities can be classified into different ways limb length.
to fully understand the current problems as well as potential MRI can give a lot of information about the growth plate
issues that may arise in the future. Such classification helps in (e.g., see Fig. 30.7f, g) and articular cartilage, periarticular
formulating the management plan for the individual patient. ligamentous injury, osteochondral fractures, and articular
alignment.
CT scans help define the fracture geometry, area of growth
Box 30.3. Classification of Posttraumatic Deformities
arrest, and presence of a bony bar. It is also a useful tool to
in Children
quantify torsional deformities.
Related to the area of bone affected Occasionally, specialized scans like the radioisotope
• Diaphyseal scans may be required to detect infection. Hematologic tests
• Metaphyseal including a white cell count, sedimentation rate, and
• Physeal C-reactive protein are helpful in such a situation. A more
• Epiphyseal comprehensive metabolic panel may be required in patients
with underlying metabolic bone diseases such as various
Related to the anatomical effect forms of rickets.
• Angulation
• Rotation
• Limb length Remodeling of Bony Deformities
• Nonunion
• Restriction of movement Remodeling may impact decision making for correcting
• Muscle weakness any bone deformity. The patient’s functional capacity and
Related to the tissue affected the surgeon’s experience should also be factors in deter-
• Soft tissue mining whether to depend on the remodeling capacity of
• Skin, fascia, muscle, capsule, or ligaments the specific fracture or to consider performing a more
• Skeletal aggressive, invasive technique to achieve a satisfactory
• Diaphyseal, growth plate, articular result [1].
In the typical long bone, 75 % of the remodeling occurs
Related to the progression by reorientation of the physis while appositional remodeling
• Progressive: Improving: Partial or complete of the diaphysis can only be expected to contribute 25 % to
• Progressive: Improving, then progressive the remodeling process.
Important factors influencing remodeling are as follows:
Clinical Manifestations 1. Age of the patient: The older the child the less is the
remodeling potential. In general, remodeling of posttrau-
Fixed deformity of the joint, joint stiffness and ankylosis, matic deformities occurs more effectively in children
shortening, angular and torsional deformities, and axial younger than 10 years.
malalignment are all potential clinical manifestations in a 2. Location of the malunion: Metaphyseal fractures remodel
posttraumatic situation. Meticulous clinical assessment may better than diaphyseal fractures. Fractures near the fast-
point to the static or progressive nature of the deformity. growing physes such as lower femur and upper tibia and
Presence or absence of infection is to be noted as it is an lower radius and upper humerus remodel better than their
important consideration in management. Examination for counterparts.
neural injury is important and can be challenging in young 3. The plane of the deformity: Angulation in the plane of
children, especially in the presence of fixed contractures. the joint axis corrects better. Rotations do not correct
for all practical purposes. In some fractures overgrowth
phenomenon gives rise to correction of limb
Investigations shortening.
4. Magnitude of angulation and translation.
Besides plain X-rays, MRI and CT scans may be useful in Some guidelines on acceptability of fracture reduction
some patients. and remodeling are given at the end of this chapter (see sec-
Plain X-rays are the simplest way of monitoring bony tion Acceptability Criteria for Reduction in Lower Limb
deformity. In addition to standard views one may need Fractures).
Case Study (Fig. 30.3)

Box 30.4. Factors Affecting Remodeling of Fractures
in Children Clinical Summary
• Age A 6-year-old girl presented 4 months after an open fracture of
• Location the tibial tuberosity with patellar tendon tear which was
• Plane of deformity debrided, repaired, and cast. She developed a stiff knee for
• Degree of deformity which the original surgeon did a manipulation under anaesthesia.
However, the local swelling and deformity increased. A Salter–
Harris type 1 injury was diagnosed at presentation. An MRI
revealed a posterior displacement of the distal femoral epiphysis
with loculated fluid collection along the posterior border.
Case Study (Fig. 30.2) Different options of management were considered but it
was decided to leave her alone in view of the good possibility
Clinical Summary of remodeling. For the stiff knee physical therapy with range
A 4-year-old child was treated for fracture shaft femur by of motion exercises was started. The sequential X-rays show
immediate spica. He had 2-cm shortening and varus defor- the excellent remodeling (Table 30.1). She regained nearly
mity 3 months post-fracture. Over a period of 2 years, the full knee movement 2 years after the injury.
deformity corrected and child had equal leg lengths.
Philosophy of Management
Philosophy of Treatment The distal femoral epiphysis is one of the fastest growing phy-
Fracture healing can stimulate bone growth in certain femo- ses with rapid remodeling in children. Undisplaced Salter–
ral shaft fractures. The amount of overgrowth varies in dif- Harris type 1 injuries need not be fixed, as long as they are
ferent reports from 0.4 to 2.7 cm. Overgrowth in femoral immobilized with restricted weight bearing and closely fol-
fractures appears to be independent of age, fracture level, lowed with serial radiographs. The ones with displacement
and position of the fracture at the time of healing. The effect may need a gentle reduction and fixation. We prefer that pins
of growth stimulation may continue for up to 3 years follow- not be introduced from the knee level upwards but rather in a
ing the fracture. reverse fashion from the metaphysis into the epiphysis as
Fig. 30.2 Effect of remodeling and growth: Fracture shaft femur in a 20 varus and 2.5 cm shortening. (d) At 3 months, united with persistent
4-year-old child. (a) Injury film. (b) Position in plaster after immediate shortening. (e) After 2 years, correction of deformity with equal limb
spica showing shortening and recurvatum. (c) At 6 weeks, uniting with lengths
Fig. 30.3 A 6-year-old with

malunited type 1 SH fracture.
(a) Four months from injury, Feb
2011. (b) MRI showing posterior
displacement of the physis. (c, d)
X-ray and clinical picture 5
months postinjury. (e) X-rays 10
months postinjury showing
remodeling and correction of
angulation and translation.
(f) Complete remodeling at
1.5 years postinjury in 2012
Table 30.1 Diaphyseal-epiphyseal angle

Table 30.2 presents a general schema of the types of
Feb 2011 38° interventions.
Mar 2011 42°
May 2011 54°
Aug 2011 70°
Nov 2011 82°
Important Posttraumatic Deformities:
Apr 2012 90°
Lower Limb
In this section we briefly discuss some unique posttraumatic

recommended by Wall [2]. It is important not to leave pins lower limb deformities in children and demonstrate a variety
sticking out of the skin if placed retrogradely. If placed in this of treatment strategies. However, as noted earlier, the deci-
fashion they should be buried under the skin for later removal. sion making should be individualized based on patient, sur-
geon, and environmental factors.
Management
Decision making in posttraumatic deformity situations is
based on a number of factors, including: Posttraumatic Chondrolysis of the Hip
and Avascular Necrosis
• Age of the patient
• Location of the deformity Acetabular injuries may be complicated by chondrolysis
• Morphological status of bone, soft tissue, and cartilage of the hip, and this may give rise to a fixed flexion and
• Functional loss and impairment adduction deformity. Likewise hip dislocations and frac-
• Abnormal mechanical loading with risk of joint tures of the femoral neck may be complicated by avascu-
degeneration lar necrosis. Avascular necrosis may follow the commonly
• Cosmetic aspect described patterns in the classifications of Kalamchi and
Table 30.2 General schema of the types of interventions

Type of deformity Nature of intervention
Joint contractures Manipulation
Splintage
Serial plasters
Soft-tissue release
External fixation
Osteotomy
Arthrodesis
Muscle imbalance Splintage
Tendon transfer
Arthrodesis
Angular and rotational bony deformities Osteotomy
Growth modulation
Physeal bar excision
Shortening Shoe raise
Limb lengthening
Contralateral epiphysiodesis
Limb shortening
Bone loss and nonunion Bone grafting
Bone transport
MacEwen [3] or of Bucholz and Ogden [4] and manage- fracture site caused by the body weight and muscle forces
ment would follow the lines described for such may be responsible for the mal- or nonunion.
conditions. Restoration of the normal neck–shaft angle is important
Arthrodiatasis [5, 6] is a useful modality for managing biomechanically and this would require correction of the
posttraumatic chondrolysis and stiffness of the hip and an malunion by a valgus osteotomy. For a nonunion osteosyn-
example is presented here. thesis may include internal fixation with or without bone
grafting and a valgus osteotomy.
Clinical Summary
A 15-year-old boy sustained a motor cycle accident in 1999. Clinical Summary
He suffered a fracture of the medial wall of the acetabulum Case of basicervical fracture of the femur in an 8-year-old
which was managed conservatively by traction. He gradually child treated conservatively who presented with a limp
developed a fixed flexion and adduction deformity of the hip related to a coxa vara deformity: The deformity was non-
along with stiffness and awkward gait. He presented 2 years progressive and uniplanar with the apex at the neck of
later for this problem having tried all conservative measures femur.
and physiotherapy. X-rays revealed narrowing of the right
hip joint space and MRI was not suggestive of infection or Philosophy of Treatment
inflammation. Both deformity correction and nonunion of femur neck frac-
ture were treated with a valgus osteotomy performed at the
Philosophy of Treatment subtrochanteric level and fixation with a plate.
He was managed by an adductor release and hip distraction.
The flexion adduction deformity improved and he gradually
regained his hip motion. The hinged distractor was in place
for 3 months and physiotherapy continued. On follow-up 8 Malunited Shaft Femur or Pseudarthrosis
years later he still retained excellent motion at the hip and with Deformity
was able to squat and sit cross legged.
Either situation will demand skeletal realignment and stabi-
Posttraumatic Coxa Vara lization by some form of internal or external fixation with or
Posttraumatic coxa vara may be caused by a malunion or without bone grafting depending on the nature of the
even a nonunion of the proximal femur. Shear forces at the pseudarthrosis.
Fig. 30.4 Hip deformity due to

posttraumatic chondrolysis in a
15-year-old. (a) Injury film
showing acetabular fracture. (b)
X-ray 2 months later, showing
healed fracture. (c) 1.5 years later
showing the marked adduction of
the hip and loss of joint space.
(d) Following arthrodiatasis with
an adductor release nearly 2
years postaccident. (e) X-rays 8
years postinjury. (f) Clinical
picture at this time
Fig. 30.5 Posttraumatic coxa

vara due to fracture neck femur
treated in spica. (a) Fibrous
nonunion in varus with a neck–
shaft angle of 100°. (b) Lateral
X-ray showing ununited fracture
line. (c) In situ fixation of
nonunion with subtrochanteric
valgus osteotomy. (d) Healing of
fracture as well as osteotomy in
3.5 months
Fig. 30.6 Maluniting fracture

shaft femur. (a) Fibrous nonunion
shaft of femur with triplanar
deformation. (b) Excision of
fibrous tissue, deformity
correction at apex, plating
without bone grafting. (c) Final
healing after plate removal at 1
year
Case Study (Fig. 30.6) avoid this problem it is recommended that the entry point is
at least 5 cm away from the growth plate. Damage to the
Clinical Summary peripheral growth plate and a resultant bar can give rise to
Fracture femur shaft which was treated conservatively pre- progressive angular deformity.
sented with fibrous nonunion with unacceptable deformity at Excision of bar is indicated if there is at least 2-year
the fracture level. growth left and area of the bar is less than 30 % of total phy-
The deformity was a triplanar deformity with malrotation seal area. The success of the surgery depends on the age of
of the femur, procurvatum of 90° and translation of about the patient, etiology, area of the bar, the bone involved, and
1 cm, and valgus angulation at the fracture site. the location of the bar [7] central or peripheral. The deformi-
ties if more than 30° are treated by osteotomy but if less than
Philosophy of Treatment 30° can often be treated by simultaneous growth modulation
Fibrous nonunion of shaft of femur was treated with resec- surgery.
tion and freshening of the edges and fixation along with bone
grafting. A corrective osteotomy was performed at the apex Case Study (Fig. 30.7)
of deformity, i.e., at the fracture site, and fixation was done
by a plate. Clinical Summary
A 11-year-old girl with progressive posttraumatic genu
valgum: She had sustained a fracture shaft femur at age 9
Distal Femoral Deformity Following Iatrogenic years which was treated elsewhere with a retrograde
Physeal Injury Rush nail.
A progressive deformity was noted after removal of the
Growth plate can get injured during insertion of elastic nail- nail. X-rays revealed a lateral growth arrest. MRI showed
ing for fracture shaft femur or during implant removal. To peripheral physeal arrest with area less than 20 %.
Fig. 30.7 A 11-year-old with fracture shaft femur treated by intramed- physeal arrest using a CT scan. (i) Intraoperative photo showing the
ullary nail with physeal injury of lower femur and genu valgum. (a) excision of bony bar using a burr. (j) Interposition of the defect with
Injury film. (b) X-rays showing fixation with a single intramedullary subcutaneous gluteal fat. (k) Intraoperative image to confirm adequacy
nail from lateral aspect. (c–e) Clinical photos and X-ray scanogram of the bar excision. (l) Postoperative X-ray showing hemiepiphysiode-
showing the deformity. (f, g) Gradient echo fat suppression MRI image sis plate and markers. (m, n) Nine months postoperative clinical photo
showing lateral growth arrest due to physeal bar. (h) Mapping of the and X-rays showing resumption of growth and correction of deformity
Philosophy of Treatment 52 % [11, 12]. Salter–Harris (SH) 1 injuries had the lowest
As she had more than 2 years of growth available and area of incidence of growth disturbance (36 %), SH2 58 %, and SH
the bar was less than 30 % of the underlying physis, she was 3 49 %, whereas SH 4 injuries had the highest rate of growth
treated with excision of physeal bar and fat interposition. At disturbance at 64 % [11].
the same time she underwent growth modulation by plate Physeal injuries require very accurate repositioning of the
hemiepiphysiodesis on medial side. growth plate and restoration of the articular alignment as
The medial hemiepiphysiodesis corrected the growth growth disturbances can result in significant angular defor-
with restoration of lateral growth by physeal bar excision. At mity. Both SH classification and displacement of the fracture
1 year the restoration of growth was evident by diverging are significant predictors of the final outcome. The treatment
screws of hemiepiphysiodesis plate and increase in the dis- method may influence the final outcome [13]. In relation to
tance between markers. Salter–Harris type 2 injuries, Ilharreborde and colleagues
stated that those with comminution and displacement had a
greater risk of growth arrest (75 %) compared with those
Physeal Injury of the Distal Femur without these risk factors (38 %) [14].
with Angular Deformity In a posttrauma situation angular deformities in children
are usually caused by a physeal injury. Such deformities can
Physeal injuries of the distal femur are notorious for the probe addressed by a hemiepiphysiodesis procedure utilizing
duction of deformity. Serious damage to the physis can occur staples, transphyseal screws, or extra-periosteal tension band
giving rise to angular deformity and/or shortening. The inci- plates. Appropriate selection of the age for intervention and
dence of angular deformity in such cases is 18–51 % in dif- time for removal of growth modulation devices is important.
ferent series and that of shortening is 36–68 % [7–10]. The It is important to avoid repeat corrections via multiple oste-
risk of growth arrest has been reported to be between 40 and otomies and hold the deformity constant in a progressive
situation. Final correction of deformity and limb length dis- open reduced, and fixed with a cancellous screw. Eight
crepancy can be done at skeletal maturity. months later a valgus deformity of the knee was noted that
Accurate reduction of a physeal injury is mandatory and was gradually progressive. At 10 years from injury, the
all efforts must be made to prevent articular step-off. Intra- deformity was progressive and very noticeable. A CT scan
articular injuries should be open reduced and stabilized and revealed the growth disturbance at the distal femur.
patients with physeal injuries should undergo rapid mobili-
zation of the adjacent joints, generally by the fourth week Philosophy of Management
postinjury, unless the fracture is very comminuted. An extra-periosteal tension band plate medial hemiepiphys-
iodesis was done to prevent further angulation. The defor-
Case Study (Fig. 30.8) mity remained unchanged till skeletal maturity but a residual
shortening of 7 cm resulted. Realignment was done by a dis-
Clinical Summary tal femoral osteotomy. The marked shortening was addressed
A 16-year-old boy had an open Salter–Harris type 2 injury of by a simultaneous gradual lengthening of the femur using
the left distal femur at 1.5 years age. The injury was debrided, external fixation
Fig. 30.8 Posttraumatic genu valgum and femoral shortening due to prevents progression of deformity holding LDFA to 66°. (h) Clinical
type 2 SH physeal injury of lower femur. (a) Injury film 1998. (b) picture, 2010. (i) Underwent medial closing-wedge distal femoral cor-
Postoperative X-ray showing fixation with screw. (c) X-ray taken 6 rective osteotomy with limb lengthening proximally using external fix-
months post-op showing valgus deformity. (d, e) Clinical photo and ation. (j, k) X-ray at the end of lengthening after consolidation of the
X-ray in 2007, 9 years postinjury showing progressive genu valgum. (f) regenerate. (l, m) 2014 final follow-up showing correction of limb
CT shows extensive lateral physeal arrest involving more than 50 % of lengths and deformity
the growth plate. (g) Hemiepiphysiodesis in 2007 using plates. This
Malunited Intra-articular Fractures Philosophy of Treatment

The echogradient fat suppression MRI showed that the area
If presenting early, maluniting fractures can be addressed of the bar was less than 25 % of the surface area of the
with open reduction and the osteochondral fragments reposi- underlying physis. He was 11 years and had more than 2
tioned to restore articular and physeal alignment. Late pre- years of active growth remaining. As he fulfilled the criteria
sentations may be fraught with the risks of poor results as for growth restoration, he was treated with physeal bar
articular congruity may be difficult to restore. excision and interposition of bone cement in the defect to
prevent reformation of the bar. Growth modulation was
Case Study (Fig. 30.9) added on lateral side with hemiepiphysiodesis plate to cor-
A 17-year-old boy sustained a comminuted fracture of the rect the deformity.
upper tibia. This was treated by internal fixation. The reduc-
tion and fixation were suboptimal. The fracture malunited Case Study (Fig. 30.11)
and he presented after implant removal for deformity and
loss of motion of the left knee. At this stage management Clinical Summary
options such as arthrodesis and deformity correction were Case of 17-year-old boy who had a fracture of the upper end
discussed but the family refused further treatment. of tibia at the age of 10 years for which he was treated with
above knee cast: He probably had an anterolateral proximal
tibial physeal arrest which was undiagnosed. It resulted in
Physeal Injury to Proximal Tibia progressive recurvatum and valgus deformity.
After 2 years of the injury, the patient presented with a
Injury to proximal tibial physis is less common than the dis- deformity of genu recurvatum which was progressive in
tal femur but can produce progressive deformities as it is also nature. There was anteroposterior instability of the knee
a fast-growing physis. The management options include with partial subluxation of the joint. Radiological exami-
excision of the physeal bar coupled with osteotomy or growth nation showed anterior physeal arrest of the proximal tib-
modulation. In case where growth cannot be restored, acute ial physis.
angular correction with an osteotomy combined with an epi- The apex of the deformity was located at the upper end of
physiodesis (to prevent recurrent deformity with future the tibia.
growth) or limb lengthening at a later date can be utilized.
Philosophy of Treatment
Case Study (Fig. 30.10) Very high tibial open-wedge osteotomy at apex to correct
deformity and filling up of the defect with bone graft: The
Clinical Summary deformity correction was done acutely with an anterior
A 11-year-old with progressive tibial varus presented with open-wedge osteotomy at the upper end of the tibia. The
blunt vehicular trauma with normal X-rays 2 years earlier. osteotomy was stabilized with a plate. Tricortical iliac
He developed progressive tibial varus due to probably a crest graft which was appropriately shaped was used.
Salter–Harris type 6 medial proximal tibial physeal injury Additional tricalcium phosphate interposition material
indicating injury to perichondrial ring. was used.
Fig. 30.9 Genu valgum due to malunited upper tibial fracture. (a) Injury film. (b) Postoperative X-ray. (c) Implant failure and malunion. (d, e)
X-rays and clinical picture after implant removal
Fig. 30.10 Type 6 Salter–Harris

injury to upper tibial physis
perichondrial ring with resultant
varus deformity. The child was
treated with bar excision and
growth modulation. (a, b)
Clinical picture and X-ray 2
years following the injury
showing varus deformity upper
tibia. (c) Gradient echo fat
suppression image showing
perichondrial ring injury (white
arrow) with formation of bony
bar (red arrow). (d) Postoperative
X-ray showing bone cement used
for interposition after excising
bony bar, markers, and
hemiepiphysiodesis plate. (e, f)
Clinical picture and X-rays 6
months after surgery. (g, h)
Clinical picture and scanogram
13 months postoperatively
showing correction of deformity
and restoration of growth. (i, j)
Intraoperative images before and
after removal of the plate. Note
the divergence of the screws
Case Study (Fig. 30.12) applied for gradual correction. Later a fibular osteotomy was
done and the same graft used to fill the osteotomy defect site,
Clinical Summary which healed and acted like an epiphysiodesis.
Case of posttraumatic tibia vara deformity secondary to frac-
ture of upper end of tibia in a young child: The deformity
was progressive and was biplanar with apex at the upper end Posttraumatic Tibia Valga
of tibia. There was severe tibia vara with mild procurvatum
of the tibia. An unusual complication of fractures of the upper tibia in
children is progressive tibial valgus. There has been a lot
Philosophy of Treatment of discussion on the mechanism of this valgus and a num-
Complex deformity in a postphyseal arrest tibia vara with ber of authors have advanced their hypotheses [15, 16].
progressive deformity was treated with staged gradual cor- Operative findings have disclosed soft-tissue interposition
rection with oblique osteotomy through apex and distractors in the form of periosteum, pes anserinus, or medial collateral
Fig. 30.11 A 17-year-old with injury to upper tibial growth plate with (d, e) Postoperative X-rays showing correction of deformity with oste-
recurvatum and varus deformity. (a, b) Anteroposterior X-rays showing otomy. (f–h) Six-month follow-up showing deformity correction and
the deformity. (c) Preoperative picture showing the extent of deformity. function with X-ray
Fig. 30.12 Severe posttraumatic tibia vara secondary to upper tibial physeal arrest. (a, b) Preoperative clinical picture and X-ray. (c, d) After
differential distraction by a minifixator. (e) After osteotomy and grafting. (f, g) Clinical picture and X-ray on healing
ligament. A consequence of this interposition is loss of peri- the growth modulation techniques can be used. Late presen-
osteal regulation of linear growth on the medial side of the tation may need an osteotomy [16].
tibia with consequent valgus.
Treatment of this condition is primarily watchful observa- Case Study (Fig. 30.13)
tion. Many authors have shown spontaneous resolution of A 3-year-old boy was seen in the outpatient clinic with a
the valgus with time. If the deformity does not resolve any of deformity of the right leg. He had sustained a fracture of the
Fig. 30.13 Posttraumatic coxa

valga following upper tibial
metaphyseal fracture. (a) At
trauma, May 2013, 1 year and 10
months of age. (b) X-ray in
plaster cast. (c) At 1 year, 11
months of age, June 2013, after
cast removal. (d) February 2014
at age 2 years, 6 months. (e, f)
X-ray and clinical photo at
presentation at 3 years of age,
July 2014, MPTA 102°, TFA 18°
right proximal tibial metaphysis at the age of 1 year and 10 At present patient walks well with no recurrence of defor-
months. He had had a cast treatment and there was no history mity and equal limb length at 5-year follow-up
of manipulation at that time. The deformity was apparent a
few months after the cast was removed. Case Study (Fig. 30.15)
Progressive deformity was noted that gradually increased
to the current magnitude. Clinical Summary
A 11-year-old male with trauma to ankle treated with cast
immobilization presenting with progressive valgus defor-
Physeal Injury Ankle with Malunion mity within 1 year of the original injury: MRI showed distal
fibular growth arrest with damage to lateral part of distal
The so-called MacFarland fracture is a Salter–Harris type 3 tibial physis.
or 4 fracture of the lower tibia and these fractures are associ-
ated with a high incidence of growth arrest and deformity. Philosophy of Treatment
Accurate repositioning of these fractures and accurate resto- The treatment was planned with very low medial transphy-
ration of the physeal plate and the joint surface are critical in seal osteotomy, and hemiepiphysiodesis of distal tibia to
reducing the incidence of growth arrest. Radiological obser- arrest medial growth to achieve correction, with percutane-
vation and investigative scans are necessary to determine the ous physeal drilling for fibular physis.
progression and location of the physeal arrest. Patient walks well with full range of ankle motion and no
recurrence of deformity at 16 months postsurgery.
Clinical Summary Soft-Tissue Loss with Skeletal Injury

A patient with fracture of distal end of tibia treated conserva-
tively presented with progressive varus deformity at the It poses a greater challenge for reconstruction as the manage-
ankle joint and shortening: The apex of the deformity was ment involves restoration of soft-tissue cover, muscle length,
present in the distal tibial physis. On radiological examina- and skeletal alignment.
tion there was a physeal bar present in the center of the distal This may need soft tissue procedures, use of distraction
tibial physis which was tenting it. with external fixation, and osteotomies.
Philosophy of Treatment Case Study (Fig. 30.16)

An intrafocal osteotomy was done at the distal end of tibia
and excision of the bar was done followed by fat graft. A cor- Clinical Summary
rective osteotomy was done at the distal end of tibia superior A 15-year-old male sustained a runover injury with soft-
to the previous osteotomy and an external fixator was applied tissue loss and bony injury (distal physeal arrest) to the lower
along with an above knee slab. leg 4 years earlier. He was initially treated with skin grafting
Fig. 30.14 Physeal injury ankle

with growth arrest. (a, b)
Postdistal tibial fracture—clinical
picture showing deformity and
MRI showing physeal central
growth arrest with tenting. (c)
Intraoperative C-arm picture
shows osteotomy and central bar
excision and fat graft. (d–f)
Postoperative X-ray showing
osteotomy and excision of bar
with external fixation. (g, h) Five
years postoperative clinical photo
and X-rays showing restoration
of physeal growth and absence of
deformity
Fig. 30.15 Twelve-year-old child with posttraumatic ankle valgus. lower tibial hemiepiphysiodesis. (e) X-rays at 16-month follow-up
(a–c) Clinical picture, X-rays, and MRI 1 year after injury. (d) showing good correction with physeal closure
Intraoperative C-arm image after fibular epiphysiodesis and medial
Fig. 30.16 Posttraumatic severe ankle valgus following physeal injury omy fixed with K wires. (e) Intraoperative picture showing mini exter-
and soft-tissue loss in 15-year-old. (a–c) Clinical pictures and X-rays at nal fixator. (f, g) Postoperative pictures on follow-up after fixator
presentation. (d) Intraoperative X-ray showing the intraphyseal osteot- removal showing good correction
and external fixation. He presented with a progressive valgus In Salter–Harris Types 3 and 4
deformity and soft-tissue contracture and was walking on the Anatomical reduction and ORIF
medial aspect of the foot.
Philosophy of Treatment Fracture Tibial Tuberosity

We did an oblique closed-wedge intraphyseal osteotomy of
distal tibia to correct the deformity and stop further progres- Only minimally displaced fractures with possible active
sion of deformity. This was combined with fibular resection extension of knee to 0° can be acceptable. Rest require open
and application of a simple distractor system for stretching reduction and internal fixation.
the lateral scarred soft tissues. We were able to achieve plan-
tigrade foot with this simple strategy.
Fracture Patella
Summary <3 mm articular step-off

<3 mm diastasis on X-ray
Posttraumatic deformity management requires a comprehen- Intact extensor mechanism
sive approach in terms of evaluation and management. Such
deformities vary in their complexity from a simple extraar-
ticular joint contracture to the more complex and progressive Fracture of Tibia and Fibula
deformities due to physeal injuries or bone loss. In children,
the potential for remodeling has to be kept in mind when Proximal Metaphysis
developing management strategy. Correction may demand Closed reduction to anatomic position or slight varus is
combination of techniques and multispecialty involvement. acceptable.
The aim must be to get an acceptable alignment and limb
length by skeletal maturity with adequate function of the Diaphysis
extremity. <8 years >8 years
Varus <10° <5°
Valgus <5° <5°
Acceptability Criteria for Reduction in Lower Ant. angulation <10° <5°
Limb Fractures Post. angulation <5° <0°
Shortening <10 mm <5 mm
Rotation <5° <5°
Fracture Neck Femur
Only anatomical reduction is acceptable. Distal Tibial Fractures

Salter–Harris Types 1 and 2
1. In patients with at least 2 years of growth remaining: <15°
Fracture Shaft Femur of posterior angulation, <10° of valgus angulation, 0° of
varus angulation
0–6 months of age: <1.5 cm of shortening, <30° angulation 2. In patients with less than 2 years of growth remaining
in varus valgus plane, <30° angulation in AP plane angulation in all planes <5°
6 months to 6 years: <2 cm of shortening, <15° angulation in
varus valgus plane, <20° anterior angulation Salter–Harris Types 3 and 4
6–10 years: <1.5 cm shortening, <10° varus valgus angula- <2 mm displacement
tion, <15° AP angulation
>10 years: <1 cm shortening, <5° varus valgus angulation,
<10° AP angulation
References
Fracture: Separation of Distal Physis of Femur 1. Wilkins KE. Principles of fracture remodeling in children. Injury.
2005;36 Suppl 1:A3–11.
2. Wall EJ, May MM. Growth plate fractures of the distal femur.
In Salter–Harris Types 1 and 2 J Pediatr Orthop. 2012; 32 Suppl 1:S40–6.
<10 years: <20° anterior or posterior angulation 3. Kalamchi A, MacEwen GD. Avascular necrosis following treat-
>10 years: Only minimal AP angulation ment of congenital dislocation of the hip. J Bone Joint Surg Am.
1980;62-A:876–88.
<5° varus valgus angulation
4. Bucholz RW, Ogden JA. Patterns of ischemic necrosis of the proxi- 11. Basener CJ, Mehlman CT, DiPasquale TG. Growth disturbance
mal femur in nonoperatively treated congenital hip disease. In: The after distal femoral growth plate fractures in children: a meta-
hip: proceedings of the sixth open scientific meeting of the hip soci- analysis. J Orthop Trauma. 2009;23(9):663–7.
ety. St Louis, MO: Mosby; 1978. p. 43–63. 12. Garrett BR, Hoffman EB, Carrara H. The effect of percutaneous pin
5. Kitakoji T, Hattori T, Ida K, Iwata H. Arthrodiatasis for chondroly- fixation in the treatment of distal femoral physeal fractures. J Bone
sis with hinge abduction: a case report. J Pediatr Orthop B. Joint Surg Br. 2011;93(5):689–94.
2000;9(3):198–200. 13. Arkader A, Warner Jr WC, Horn BD, Shaw RN, Wells L. Predicting
6. González-Herranz P. Articulated hip distraction or arthrodiatasis. J the outcome of physeal fractures of the distal femur. J Pediatr
Pediatr Orthop B. 2006;15(2):154. Orthop. 2007;27(6):703–8.
7. Eid AM, Hafez MA. Traumatic injuries of the distal femoral phy- 14. Ilharreborde B, Raquillet C, Morel E, Fitoussi F, Bensahel H,
sis. Retrospective study on 151 cases. Injury. 2002;33(3): Penneçot GF, Mazda K. Long-term prognosis of Salter-Harris type
251–5. 2 injuries of the distal femoral physis. J Pediatr Orthop B.
8. Czitrom AA, Salter RB, Willis RB. Fractures involving the distal 2006;15(6):433–8.
epiphyseal plate of the femur. Int Orthop. 1981;4(4):269–77. 15. Vrettakos AN, Evaggelidis DC, Kyrkos MJ, Tsatsos AV, Nenopoulos
9. Robert M, Moulies D, Longis B, Laufenburger A, Coville M, Alain A, Beslikas T. Lower limb deformity following proximal tibia phy-
JL. Traumatic epiphyseal separation of the lower end of the femur. seal injury: long-term follow-up. J Orthop Traumatol. 2012;13(1):7–
Rev Chir Orthop Reparatrice Appar Mot. 1988;74(1):69–78. 11. Epub 2012 Feb 11.
10. Thomson JD, Stricker SJ, Williams MM. Fractures of the distal 16. Dorman S, Jariwala A, Campbell D. Cozen’s phenomenon: a
femoral epiphyseal plate. J Pediatr Orthop. 1995;15(4):474–8. reminder. Scott Med J. 2013;58(3):e10–3.
Postinfectious Deformities
of the Lower Limb 31
In Ho Choi
reconstructive operations can ensure growth and develop-

Introduction ment of the lower limb by restoring the weight-bearing
mechanical axis as well as individual joint alignment, and
There is a diverse range of postinfectious residual deformi- thus providing the best possible joint mechanics at skeletal
ties of the lower limb depending on the site of infection, maturity. This chapter reviews the treatment principles and
patient’s age, infecting organism, host resistance, timing and reconstructive surgical modalities for long-term residual
adequacy of initial treatment, and severity of damage to the deformities of the lower limb after septic arthritis of the hip,
epiphysis and physis of the affected bone [1–8]. The epiphy- knee, and ankle, and osteomyelitis of the femur and tibia in
sis can be damaged extensively, particularly in the neonatal infancy or early childhood.
period, by various mechanisms including transphyseal
spread of infection from the metaphysis through the physis
and into the epiphysis via transphyseal vessels, or following Late Sequelae of Infantile Septic
the passage of organisms from the metaphysis to the joint in Arthritis of the Hip
intracapsular epiphyses such as the hip joint, or from the
epiphyses into the adjacent joint following transphyseal Residual deformities of infantile septic arthritis of the hip
spread [9]. As a result, most of the postinfectious juxta- occur due to necrosis of the articular cartilage, ischemic
articular and articular deformities are associated with phy- necrosis of the femoral head (FH), premature closure of the
seal damage and growth disturbance. triradiate cartilage, acetabular dysplasia, premature or asym-
Proper evaluation based on a thorough musculoskeletal metrical closure of the proximal femoral physis, subluxation,
examination and radiographic imaging allows the surgeon to dislocation, pseudarthrosis of the femoral neck, greater tro-
determine an effective treatment plan for deformity correc- chanteric overriding, and complete destruction of the FH and
tion. Meticulous analysis of the complex deformity of the neck [1–3, 6–9, 11–14]. The decision to perform either a
lower limb by determining the location, magnitude, and reconstructive or a salvage procedure should be individual-
direction of the deformity would be the first step in charac- ized depending on the type of residual deformity of the hip.
terizing the deformity. Mechanical axis deviation, mechani-
cal and anatomical alignment, and joint orientation should be
accurately determined [10]. In addition, LLD and difference Radiographic Classifications of Late
in the knee level and the presence of joint laxity and loss of Sequelae of Infantile Septic Arthritis
mobility secondary to soft-tissue contractures and/or juxta- of the Hip
articular skeletal deformity should be evaluated.
The management of severe deformities that occur as a Several radiographic classifications of late sequelae of
result of fulminant septic arthritis or osteomyelitis is clini- infantile septic arthritis of the hip have been reported in
cally challenging. However, timely and well-performed the literature. In 1982, Hunka et al. [11] first proposed a
classification to document the five types of residual defor-
mities of septic arthritis in a logical fashion: Type 1—
I.H. Choi, MD, PhD (*) absent or minimal FH changes; Type II—(A) deformity of
Division of Pediatric Orthopedics, Seoul National University
the FH, with an intact growth plate; (B) deformity of the
Children’s Hospital, 101 Daehak-ro, Jongno-gu, Seoul
110-744, Republic of Korea FH, with a premature fusion of the growth plate; Type
e-mail: [email protected] III—pseudarthrosis of the femoral neck; Type IV—(A)

588 I.H. Choi
Fig. 31.1 Classification of

sequelae of infantile septic
arthritis of the hip. Type I: No
residual deformity (Type IA) or
mild coxa magna (Type IB). Type
II: Coxa breva with a deformed
head (Type IIA) or progressive
coxa vara or valga due to
asymmetrical, premature physeal
closure (Type IIB). Type III:
Slipping at the femoral neck,
resulting in coxa vara or valga
with severe anteversion or
retroversion (Type IIIA) or
pseudarthrosis of the femoral
neck (Type IIIB). Type IV:
Destruction of the femoral head
and neck, with a small remnant
of the neck (Type IVA) or
complete loss of the femoral head
and neck and no articulation of
the hip (Type IVB)
complete destruction of the proximal femoral epiphysis, In 1990 the author devised a radiological classification
with a stable neck segment, (B) complete destruction of of residual proximal femoral deformities based on the
the proximal femoral epiphysis, with a small unstable nature and extent of damage and radiographic appearance
neck segment; Type V—complete destruction of the FH at the final follow-up or at maturity, and each of the four
and neck to the intertrochanteric line, with dislocation of major types of residual deformities was further divided into
the hip. two subtypes (Fig. 31.1) [1]. In 2002, Johari et al. [12, 13]
31 Postinfectious Deformities of the Lower Limb 589
also reported the classification of the sequelae of septic

hips. Group 1: loss of capital femoral epiphysis (CFE)/ Choi Type II and III Hips Causing
neck, metaphyseal spike present, stable; Group 2: loss of Femoroacetabular Impingement
CFE and neck, unstable; Group 3A: dislocation, CFE pres- and Secondary Hip Dysplasia
ent, unstable; Group 3B: subluxation, CFE present, unsta-
ble; Group 4, articular incongruity, avascular necrosis, coxa Type II and III hips are fundamentally the result of an abnor-
magna, physeal disturbance (coxa breva, coxa vara, coxa mally shaped, sized, or oriented FH and neck, altered head-
valga, and trochanteric overgrowth), stable; Group 5, neck offset, and acetabular dysplasia. A wide variety of
pseudarthrosis of the femoral neck, stable/unstable. In residual deformities of the FH have been observed in Type II
2008, Forlin and Milani [14] proposed a relatively simple and III hips, ranging from deformities similar to those in
radiological classification based on the relation between Perthes disease with coxa vara/valga, coxa plana, coxa breva,
the femur and the acetabulum, and the severity of the lesion coxa magna, coxa irregularis, relative greater trochanteric
of the proximal femur. Hips with sequelae grade 1 were overriding, sagging rope sign [15], and acetabular dysplasia
reduced hips, with FH preserved (1A) or absent (1B); hips to the more deformed proximal femur visualized as beard,
grade 2 were dislocated hips, with the FH preserved (2A) or collared, staghorn, or rugby ball-shaped FH [16, 17].
absent (2B). The classification systems proposed by other Children with Type II or III deformities may remain
authors [11–14] do not have a subtype equivalent to the asymptomatic in their younger age, but, in (pre)adolescence,
Choi Type IIIA (slipping at the femoral neck, resulting in these children often present with clinical problems such as
coxa vara or valga with severe anteversion or retroversion) pain, limp, joint instability or contracture, restriction of
(see Fig. 31.1) [1]. motion, LLD, spinal disorders, and even emotional distur-
One common drawback of all the aforementioned radio- bances. According to the author’s experience, in older chil-
logical classifications is that they were mainly devised based dren with Type II and III hips, a painful limp is usually
on the changes noted on the plain radiographs. Currently, associated with femoroacetabular impingement (FAI) with
with the help of modern, advanced imaging technologies, or without damaged acetabular cartilage and/or labrum.
e.g., MRI and 3D-CT, deformed osteocartilaginous geome- Figure 31.2 depicts a stepwise algorithmic approach to
try of the proximal femur can be better visualized in three FAI in Perthes-like deformities at the author’s institute. First,
dimensions, and the dynamics of articular motion between the extent of asphericity is assessed; if it is mild and the FH
the deformed FH and acetabulum can be better understood. is well contained within the acetabulum, osteochondroplasty
A new, comprehensive three-dimensional radiological clas- of the head and neck junction alone is indicated. Next, the
sification is anticipated considering the rapid advancement in presence or absence of a labral tear is determined. If a labral
the imaging technology. tear is present, labral debridement or refixation is necessary.
Similarly, the presence of coexisting acetabular dysplasia is
assessed. If the hip becomes congruent in abduction, relative
Late Operative Treatment Modalities femoral neck lengthening combined with periacetabular
for Residual Deformities of Septic osteotomy (PAO) or triple innominate osteotomy (TIO) is
Arthritis of the Hip indicated. In the author’s institute, Bernese PAO is preferred
for correction of acetabular dysplasia in adolescents and
Treatment modalities for the long-term sequelae of infantile young adults with closed triradiate cartilage (around 12 years
septic arthritis of the hip are outlined in Table 31.1. The rec- in girls and 14 years in boys) [18], whereas a TIO, in (pre)
ommended primary and secondary treatment methods are as adolescents with open triradiate cartilage [19]. If the hip
varied as the types of deformities. Unfortunately, no definite becomes congruent in adduction, proximal femoral valgus
primary treatment for a severely damaged hip joint makes osteotomy and PAO or TIO are indicated. The next step is to
the affected joint capable of withstanding the forces imposed check whether coxa breva and coxa vara associated with
on it by otherwise healthy children with their long-term relatively greater trochanteric overriding are severe enough
physical demands [1]. Thus, any surgical treatment for severe to cause FAI, and if so, a relative or true femoral neck
sequelae must be regarded as a measure that temporarily lengthening combined with distal advancement of the greater
improves the clinical function and delays the more definitive trochanter (GT) is indicated [20–23]. When the FH aspheric-
procedures that are reserved for adult patients [1, 2]. The ity is too severe and the FH is “irreducible,” the following
pearls and pitfalls in the management of late sequelae of procedures should be considered. If the hip becomes cong-
infantile septic arthritis of the hip are summarized in ruent in extension and adduction, femoral valgus-flexion
Table 31.2. osteotomy plus/minus PAO or TIO should be considered.
590 I.H. Choi
Table 31.1 Late operative treatment modalities for severe sequelae of infantile septic arthritis of the hip
Purpose Indications Procedures
To reduce/contain the hip joint Subluxation or dislocation Open reduction; soft-tissue release (adductor-iliopsoas tenotomy)
To optimize joint congruity Incongruity Proximal femoral realignment osteotomy (varus or valgus osteotomy with a
sagittal or rotational component); redirectional acetabular osteotomy
(Salter, Pemberton, Dega osteotomy, triple innominate osteotomy, Ganz
periacetabular osteotomy)
To improve femoral head Uncovered femoral head Redirectional acetabular osteotomy; salvage acetabuloplasty (e.g., Chiari,
coverage shelf operation); Ganz femoral head reduction osteotomy
To reduce cam or pincer lesion Femoroacetabular Osteochondroplasty via safe surgical hip dislocation or arthroscopy;
impingement excision of the femoral head and neck remnant (Choi Type IVA or mobile
nonunion type of IIIB)
To address intra-articular Torn labrum or articular Labral debridement or refixation; articular cartilage repair using
pathology of the cartilage or cartilage lesion microfracture technique or osteochondral grafting or chondrocyte
labrum transplantation
To correct acetabular dysplasia Dysplastic acetabulum Redirectional acetabular osteotomy (e.g., Salter, Pemberton, Dega, triple
innominate osteotomy, Ganz periacetabular osteotomy)
To correct abnormal version of Anteverted or retroverted Proximal femoral rotational/derotational osteotomy; redirectional acetabular
the proximal femur and femur and acetabulum osteotomy (e.g., triple innominate osteotomy, Ganz periacetabular
acetabulum osteotomy)
To improve abductor muscle Greater trochanteric Greater trochanteric advancement; true or relative femoral neck lengthening
function; to correct coxa brevis overriding; short femoral neck
To repair pseudarthrosis of the Femoral neck nonunion Bone grafting, often combined with proximal femoral valgus osteotomy
femoral neck
To reconstruct femoro-pelvic Destruction of the femoral Greater trochanteric arthroplasty; pelvic support osteotomy; Ilizarov hip
articulation head and neck reconstruction (Choi Type IVB or failed type IVA); L’Episcopo, Albee, or
Harmon operation, Ilizarov femoral neck lengthening, femoral neck
reconstruction using vascularized bone grafting (Choi Type IVA)
To equalize the lower limb Limb length shortening Lengthening by distraction osteogenesis; contralateral epiphysiodesis or
length shortening of the femur; Ilizarov hip reconstruction
To manage painful unstable Unstable hip with advanced Hip arthrodesis; total hip replacement arthroplasty; Ilizarov hip
hip as a last resort osteoarthritis not amenable to reconstruction after excision of the femoral head and neck remnant
the above-mentioned
reconstructive procedures
Table 31.2 Pearls and pitfalls in the management of late sequelae of infantile septic arthritis of the hip
Assessment The surgeon should be familiar with radiological classifications of femoral residual deformities. Use of advanced imaging
technologies (MRI, ultrasonography, arthrography, and 2-D/3D-CT) to visualize unossified cartilaginous structure of the
epiphysis, also to determine osteocartilaginous geometry of the proximal femur, and the dynamics of articular motion between
the deformed head and acetabulum.
Full-length standing radiograph (teleradiograph) visualizing the entire lower limbs is helpful not only to identify associated
sequelae at other sites, if any, but also to determine axial malalignment, joint malorientation, and limb length discrepancy.
Treatment Femoroacetabular impingement-related symptoms are often encountered in older children and young adults with severe
residual deformities.
The decision to perform either reconstructive or salvage procedures must be made on an individual basis, after reviewing the
clinical findings and imaging studies. Definitive and adjunctive surgical treatment methods can be used alone or in combination
(see Table 31.1).
Author’s stepwise algorithmic surgical approach to femoroacetabular impingement would be helpful in decision making of
treatment for Choi Type II and III hips (see Fig. 31.2).
Greater trochanteric arthroplasty for reconstruction of femoro-pelvic articulation is a technically demanding procedure with
unpredictable outcomes. If performed at younger age, the transferred greater trochanter can assume a globular shape, but with
no or incomplete formation of head-neck offset.
Ilizarov hip reconstruction is a highly effective treatment modality for the management of Choi Type IVB and IVA hips in
which the previous reconstructive surgery has failed, or there is late presentation with a severe abductor lurch and a
considerable limb length shortening, particularly in the adolescents and young adults in whom arthrodesis or hip arthroplasty is
not suitable.
Fig. 31.2 A schema showing the stepwise algorithmic approach to neck lengthening, PAO periacetabular osteotomy, PA posteroante-
femoroacetabular impingement associated with Choi Type II and rior, AP anteroposterior. Note: A triple innominate osteotomy is
III sequelae, which are similar to Perthes(-like) deformities (see performed, instead of PAO, in (pre)adolescents with open triradiate
text). RFNL relative femoral neck lengthening, TFNL true femoral cartilage [18]
If the hip becomes congruent in any rotational position, L’Episcopo [30], Harmon [31], or Albee [32] reconstruction
rotational osteotomy with or without PAO or TIO may be methods. When a reasonably sized unossified cartilaginous
indicated. If the FH with a central depression is too severely cap persists as a femoral neck remnant, open reduction can be
deformed such that the FH cannot be contained within the attempted. The repositioning of the femoral remnant into the
acetabulum in any position, Chiari osteotomy or FH reduc- acetabular socket can be facilitated using a modification of the
tion osteotomy [20, 21, 24] plus/minus PAO or TIO can be Harmon technique [31]. An incomplete, spring osteotomy is
considered. In addition, combination of osteochondroplasty, made at the base of the femoral remnant adjoining the greater
labral debridement/refixation, and distal advancement of the trochanter and the resulting opening wedged gap is filled with
greater trochanter should be performed, if necessary. a block of cartilage graft taken from the iliac apophysis. This
procedure produces a femoral neck-lengthening effect [28].
In older children, gradual lengthening of the remnant femoral
Late Treatment Options for Choi neck using the Ilizarov technique may be indicated.
Type IV Deformity Nonetheless, the author believes that open reduction in chil-
dren more than 6 years of age is not likely to be beneficial
The available reconstructive procedures for the treatment of because of the high risk of developing stiffness and pain.
Type IV hips are limited, and, in general, have not yielded Therefore, the author recommends that in children older than
satisfactory long-term results [1, 2, 7, 11, 25–29]. When only 6 years of age, Type IVA hips should be treated in the same
a remnant of the FH and neck is present, it is difficult to main- manner as hips with Type IVB sequelae [2].
tain a stable reduction; and if instability and dislocation per- Postponing the treatment of Type IVB hips will result in
sist, the surgeon can either leave the deformity uncorrected or proximal iliac dislocation with a marked abductor lurch, tele-
can attempt reconstruction of femoro-pelvic articulation [25]. scoping, limp, and LLD [8, 26]. Although these patients may
In young children in whom the FH and neck have been remain asymptomatic for a number of years [3, 7, 12] they are
destroyed, as in Type IVA sequelae, a new femoral neck can be at a risk of developing degenerative changes in the lumbosacral
fashioned to articulate with the acetabulum by utilizing the spine and hips [25]. Therefore, various treatment modalities for
592 I.H. Choi
reconstructing a femoro-pelvic articulation in Type IVB hips many drawbacks and limitations of GTA for reconstruction of
have been reported: hip reconstruction with vascularized iliac femoro-pelvic articulation due to the uniqueness of the tech-
crest grafting [33], arthrodesis, greater trochanteric arthro- nique. Previous reports have described the potential risk of
plasty (GTA) [25, 26, 34–39] with adjunctive or secondary pro- avascularity of the proximal segment after femoral varus oste-
cedures, pelvic support osteotomy (PSO) [40–44], and Ilizarov otomy, difficulty in osteosynthesis at the varus osteotomy
hip reconstruction (IHR) [44–58]. Of these treatment modali- site, gradual straightening of the proximal femur due to
ties, the two most commonly performed procedures are GTA remodeling at the osteotomy site, abductor weakness, stiff-
and IHR. ness, degenerative arthritis, and difficulty during conversion
to total hip arthroplasty in adulthood [1, 2, 25, 26, 28, 36, 37,
51]. According to the author’s observation, the transferred GT
Greater Trochanteric Arthroplasty Versus can assume a globular shape with time, but with no or incom-
Pelvic Support Osteotomy (Ilizarov Hip plete formation of head-neck offset. For this reason, even
Reconstruction) those children with satisfactory results at the midterm follow-
up often present with FAI-related symptoms (Fig. 31.3).
GTA was originally described by Colonna [34] and was pop- The two most important factors that determine the suc-
ularized by Westin [35] and others [25, 26, 36, 37]. The cess or failure of a procedure after GTA are preoperative
underlying concept of GTA is based on the following two abductor weakness and stiffness [2]. Although some authors
aspects: firstly, the GT is viable and therefore retains its have reported satisfactory results [25, 26, 34, 35, 37–39],
growth potential; and secondly, when the hyaline cartilage most of the experienced surgeons feel that GTA is a techni-
covering the GT is placed inside the acetabulum, the GT cally demanding procedure with highly unpredictable out-
assumes a globular shape, similar to that of an FH [1, 2, 18, comes. The author has noted that the result of GTA may also
26, 28, 29, 34–39, 44]. The detached abductor muscles must be correlated with the patient’s age at operation. In a retro-
be transferred distally in order to provide hip stability and spective study performed by the author [28], satisfactory
some degree of abductor function. However, the subsequent results were obtained only in those children who underwent
progressive subluxation observed in most of the patients GTA at less than 6 years of age. This observation may
necessitates additional procedures, such as femoral varus support the opinion that a patient should be old enough to
osteotomy, pelvic osteotomy including Chiari osteotomy, or follow the physical therapy instructions, and young enough
shelf acetabuloplasty, to improve FH coverage. There are to allow remodeling of the transferred GT [2].
Fig. 31.3 Greater trochanteric arthroplasty in a girl. (a) A radiograph shows laterally displaced, remodeled greater trochanter with multiple
in a 5-year- and 1-month-old girl showing Choi Type IVB sequelae in osteochondral prominences on its margin, suggestive of femoroacetab-
the right hip. (b) A radiograph showing trochanteric arthroplasty with ular impingement. (e) A radiograph showing total hip replacement
adjunctive femoral varus osteotomy. (c, d) Radiographs taken at arthroplasty which was performed to treat a painful limp. (f) A gross
32 years of age showing advanced degenerative arthritis of the right hip photograph of the remodeled greater trochanter; the articular cartilagi-
joint, despite a well-remodeled greater trochanter which had assumed a nous surface had been markedly degenerated, showing some fibrous
globular shape similar to that of a femoral head. 3D-CT image also adhesions
“Pelvic support osteotomy (PSO)” has a long history in performing IHR at an age before adolescence, as much as
orthopedic surgery. The technique was developed in 1910s 25°–40° of overcorrection should be empirically added to the
and quite popular until the advent of total hip arthroplasty valgus correction angle based on the single-leg stance drop
[40–44]. The basic concepts and goals of PSO are to enhance angle relative to the horizontal line of the pelvis due to the
femoro-pelvic stability by means of a proximal femoral val- possibility of straightening of the proximal femur after IHR
gus osteotomy and to improve hip biomechanics by displac- [44, 46, 51]. The author believes that IHR is still indicated
ing the center of gravity medially, resulting in an improvement even if patients are older than 6 years of age, despite the high
in the mechanical efficiency of the abductor muscles [44]. probability of repeat PSO, because a second femoral length-
However, the clinical application of traditional PSO is lim- ening is usually needed at or near skeletal maturity in patients
ited due to its indigenous shortcomings. It is difficult to with hip instability associated with marked LLD due to mul-
achieve the optimal extent of angulation. If the angular cor- tiple lower limb growth disturbances secondary to neonatal
rection is too large, excessive genu valgum, fixed pelvic sepsis. Another alternative is to perform femoral lengthening
obliquity, and impingement pain on adduction of the lower without a PSO, and insert half pins into the pelvis to prevent
limb to neutral position may occur. On the other hand, if the proximal migration of the femur at a younger age, and sub-
angular correction is too small, the result would lead to an sequently perform IHR when the patients are near skeletal
insufficient improvement in hip biomechanics, and most maturity [44, 55].
importantly, the issue of persisting LLD cannot be addressed.
To overcome the shortcomings of traditional PSO, Ilizarov
developed and popularized a modified PSO technique, the Late Sequelae After Septic Arthritis
so-called IHR, in which a second distal femoral osteotomy is and Osteomyelitis Around the Knee
performed in addition to proximal femoral valgus osteotomy and Ankle Joints
for pelvic support to realign the knee joint and to correct the
LLD [44–58]. Furthermore, Ilizarov emphasized the impor- The rate of overall unsatisfactory results in terms of residual
tance of extension of the proximal femoral osteotomy to cor- deformities after infantile or childhood septic arthritis of the
rect the fixed flexion deformity of the hip and to permit knee and ankle joints is less as compared to that after septic
locking of the hip joint [44–47, 49–58]. arthritis of the hip [5, 6], because the infection in these joints
Evidence has accumulated that IHR is highly effective in can be diagnosed sooner than that in the hip [9]. Long-term
eliminating or substantially reducing the Trendelenburg gait assessment is essential because radiographic improvement in
and in equalizing the lower limb length while improving the the involved epiphyseal region can occur several months to
stability and range of motion of the hip joint at the same time years after the infection. In younger children in whom the
[44, 47–58]. Hence, the author currently prefers IHR for the secondary ossification center has not yet developed, vascular
management of Type IVB and IVA hips in which the previ- damage can lead to a marked delay in the formation of the
ous reconstructive surgery has failed, or there is late presen- ossific center, but the cartilage model continues to grow.
tation with a severe abductor lurch and a considerable LLD, Therefore, even when bone deficiency is seen on plain radi-
particularly in the adolescents and young adults in whom ography, it is necessary to perform advanced imaging studies
arthrodesis or hip arthroplasty is not suitable [44]. such as ultrasonography, arthrography, or MR imaging [9] in
The reported complications of IHR in the literature order to better visualize the cartilaginous epiphysis (chon-
include knee stiffness, pin tract infection, delayed consolida- droepiphysis) and assess the potential growth of the adjacent
tion, refracture, obturator nerve entrapment, straightening of physis. When the appropriate treatment is delayed, growth
proximal valgus angulation, and persistent Trendelenburg disturbance with a resultant angular deformity, joint incon-
gait. There exists a possibility of ischiofemoral impingement gruity, impingement, and accompanying ligamentous laxity
if the apex of the proximal femoral valgus angulation site and joint instability may develop gradually. Postinfectious
directly abuts the ischium. The literature demonstrates that juxta-articular deformity of the knee usually develops as a
an average of 30 % (range, 0–62.5 %) of patients have a per- result of metaphyseal osteomyelitis or primary septic arthri-
sistent positive Trendelenburg sign after IHR, although it is tis of the knee, involving the distal femoral physis or the
reduced in severity in most of the patients [44]. proximal tibial physis, or both. The distal femur is more
IHR is not ideal for young children, because in accor- prone to growth-related sequelae than the proximal tibia.
dance with Wolff’s law, gradual straightening of the proxi- Angular deformities due to partial distal femoral physeal
mal femur tends to occur at the site of valgus angulation, destruction are far more common than complete symmetric
which may result in loss of pelvic support. Bone remodeling growth cessation [9]. The most serious growth sequelae
and subsequent gradual loss of valgus angulation remain occur following neonatal sepsis and to a lesser extent within
important considerations in proximal femoral osteotomy the first 2 years of life [6, 9] (Fig. 31.5). In particular,
[44, 46, 51, 52, 55, 56, 58] (Fig. 31.4). Therefore, when meningococcemia caused by Neisseria meningitides or
594 I.H. Choi
Fig. 31.4 (a) A radiograph in a 12-year- and 4-month-old boy with graph and 3D-CT taken at 17 years of age showing displacement of the
Choi Type IVB sequelae in the right hip. He had a previous history of lesser trochanter into the acetabulum and proximal femur with valgus
Ilizarov hip reconstruction with 40° of proximal valgus osteotomy for angulation near the ischium, which apparently enhances the femoro-
pelvic support and 4 cm of distal diaphyseal lengthening for leg length pelvic stability and improves abductor mechanics by displacing the
equalization performed at an age of 8 years and 1 month. It is notewor- center of gravity medially. (e) A teleradiograph taken at 17 years of age
thy to observe a completely remodeled, straightened proximal femur showing equalization of the lower limb length after surgery. He had a
showing no evidence of pelvic support. (b) A teleradiograph showing a negative Trendelenburg sign
repeated Ilizarov hip reconstruction osteotomy. (c, d) A plain radio-
meningococcus, which is found only in human respiratory contribute to abnormal loading of the affected joint, leading
secretions, is a devastating illness that primarily affects to compartmental osteoarthritis of the affected joint(s) [6, 62,
young children. The disseminated intravascular coagulation 63]. Juxta-articular realignment and reorientation osteotomy
and focal infections of the acute phase are primarily respon- are preferred because proximity of such an osteotomy to the
sible for the vascular injuries to the growing chondro-osseous adjacent joint leads to a more anatomic reorientation correc-
tissues and contiguous soft tissues. Ischemic changes also tion, without iatrogenic translational deformities. The resul-
selectively involve the physeal circulation, which adversely tant pain reduction and decreased wear rate of the articular
affect longitudinal and transverse growth of bone. Therefore, cartilage due to redistribution of joint reaction forces increase
children who survive meningococcal septicemia are prone to the longevity of the major weight-bearing joints [63]. The
develop complex deformities of the lower limb due to mul- pearls and pitfalls in the management of late sequelae after
tiple areas of growth disturbance [59–61]. septic arthritis and osteomyelitis around the knee and ankle
The ultimate goal of managing juxta-articular and intra- joints are summarized in Table 31.3.
articular deformity is prevention of osteoarthritis of the To establish an optimal treatment plan for restoring the
affected joint and the adjacent joint by restoring the weight- weight-bearing axis, for stabilizing the unstable joint, and
bearing mechanical axis and by normalizing the joint orien- for equalizing the lower limb length, preoperative three-
tation as early as possible, because both these factors dimensional assessment of the juxta-articular and articular
Fig. 31.5 (a) A 10-year- and 7-month-old girl presenting with 17.5 cm joint apparently caused by deformation of the femoral condyle second-
of shortening of the right femur and a concomitant 30° of flexion con- ary to central physeal arrest in the distal femur. (d, e) A concomitant
tracture of the knee joint after fulminant osteomyelitis of the right 10-cm lengthening and deformity correction could be achieved by dif-
femur extending to the hip and knee joints. Further flexion in the knee ferential distraction of the anterior and posterior motor rods. Subsequent
joint was only marginally limited. (b, c) A radiograph and photograph procedures included right tibial and fibular lengthening followed by left
showing the femoral and tibial Ilizarov frames constructed with one distal femoral epiphysiodesis, concomitant right femoral and tibial
proximal full ring and a distal two-third ring for concomitant femoral lengthening, and right greater trochanteric advancement. (f) At the last
lengthening and deformity correction at the distal femoral corticotomy follow-up at the age of 26 years, her right lower limb function and gait
site. Tibial frame was applied for the purpose of transarticular fixation were excellent with equalization of the lower limb length and no knee
during lengthening to prevent potential subluxation of the unstable knee flexion contracture despite the difference in the knee level
deformity is essential. The surgeon should recognize that Patients who have a juxta-articular deformity may also
frontal, sagittal, and oblique plane malalignment can result have a compensatory fixed deformity at an adjacent bone and
from a variety of sources such as ligamentous laxity, joint joint, and correction of this deformity must also be included
subluxation, and altered joint line secondary to physeal in the treatment plan. Correction of the malunion without
growth disturbance, and epiphyseal deficiency or deforma- addressing the compensatory deformity may result in a
tion. This may be particularly true in children with severe straight bone with a maloriented joint, leading to long-term
multifocal, angular, and/or rotational deformities, often disability due to altered biomechanics. For instance, when
associated with joint incongruity, joint instability, LLD, distal femoral valgus coexists with compensatory proximal
and surrounding soft-tissue scars and contractures. In such tibia varus or distal femoral varus in the presence of a com-
children, identification, characterization, and prioritization pensatory proximal tibial valgus deformity, the lower limb
of each component of the deformity are crucial. The deci- may appear aligned with no mechanical axis deviation
sion and timing of performing multilevel operations either despite malorientation of the knee joint. However, combina-
in one stage or in multiple stages must be made on an indi- tions of femoral valgus and tibial varus would produce shear
vidual basis. stress in the knee joint due to increased obliquity of the knee
596 I.H. Choi
Table 31.3 Pearls and pitfalls in the management of late sequelae after septic arthritis and osteomyelitis around the knee and ankle joints
Assessment In young children, it is necessary to perform studies assessing the cartilaginous structure of an epiphyseal region to assess
the growth potential by using ultrasonography, arthrography, bone scintigraphy, and MRI.
Meticulous deformity analysis of the complex deformity by determining the location, magnitude, and direction of the
deformity is the first step. Frontal, sagittal, and oblique plane malalignment can also result from ligamentous laxity, joint
subluxation, and joint line deformity.
It is essential to determine whether a juxta-articular deformity has a compensatory fixed deformity at an adjacent bone and
joint. When there exists a compensatory fixed deformity, correction of this deformity must also be included in the treatment
plan.
Careful assessment of physeal integrity, intra-articular epiphyseal irregularity, joint instability, surrounding soft-tissue
condition, and neurovascular condition is essential.
Treatment The decision whether to perform acute versus gradual deformity correction, and conventional osteotomy versus external
fixator-assisted plating or intramedullary nailing, should be made on an individual basis. When a technique of lengthening
over an intramedullary nail using external fixation is used, caution is required to prevent deep infection in patients who have
a past history of infection, and open trauma.
Juxta-articular realignment and reorientation osteotomy are preferred because the closer is each osteotomy to the adjacent
joint, the greater is the reorientation with angular correction.
When correcting a juxta-articular deformity by conventional metaphyseal osteotomy, the use of the Ilizarov technique with
an offset hinge system or external fixation which utilizes the six-axis deformity correction principle is preferred.
When correcting severe juxta-articular deformity, adjunctive procedures (e.g., peroneal nerve decompression and
compartment release) may be justified to prevent major complications.
In the management of juxta-articular ankle deformity, early stabilization of the talocrural joint by leveling the ankle and
restoration of the axial alignment and joint orientation are of paramount importance.
Intra-articular osteotomy is a very technically demanding procedure, and should be selectively performed.
to knee joint stability, buttressing against the lateral sublux-

ation forces [64, 65].
Correction of juxta-articular deformity by conventional
metaphyseal osteotomy is associated with many problems,
including fixation stability, geometrical considerations related
to the difference between the osteotomy site and the center of
the deformity, multilevel deformities caused by previous
osteotomies, and concomitant LLD. In addition, the disad-
vantages of acute correction using plate and screw fixation
include the requirement of extensive soft-tissue dissection,
limitation of early weight bearing and function, and inability
to correct a significant shortening deformity. It is well known
that realignment of the mechanical axis and joint orientation
by metaphyseal osteotomy requires a combination of angula-
tion and translation [62, 63]. In this regard, the Ilizarov tech-
nique with an offset (juxta-articular) hinge system is preferred
for accurately correcting and restoring the mechanical axis
and joint orientation. In recent years, the use of external fix-
ators which utilize the six-axis deformity correction principle
Fig. 31.6 A radiograph showing right distal femoral valgus and com-
pensatory proximal tibia varus in a 16-year-old boy. Although the lower using a software program has been advocated due to the con-
limb may appear aligned with little mechanical axis deviation despite venience and accuracy of such adjuncts in correcting complex
increased obliquity of the knee joint, this deformity can cause lateral juxta-articular deformities of the limb. External fixator-
subluxation of the proximal tibia associated with progressive degenera- assisted acute deformity correction and fixation using a lock-
tive arthritis in late adulthood
ing compression plate [66–68] is another surgical technique
for addressing such deformities. The potential advantages of
joint to the ground (Fig. 31.6). This deformity can cause lat- a fixator-assisted plating include correction of deformities
eral subluxation of the proximal tibia associated with pro- under direct visualization, rigid fixation, and its applicability
gressive degenerative arthritis in late adulthood. In contrast, in various anatomic locations and situations, e.g., open phy-
in the case of distal femoral varus and proximal tibial valgus, sis, narrow intramedullary canal, and bowing deformity of the
patients may tolerate well because obliquity of the joint adds femur and tibia. Similarly, external fixator-assisted acute
Circular external fixation is well suited for the management

of patients with complex deformities and/or bone loss with a
poor soft-tissue envelope and history or presence of infec-
tion. The specific advantage of external fixation is that
adjustments to achieve the desired alignment can be per-
formed gradually during the postoperative period. In certain
complex scenarios, external fixators are utilized in conjunc-
tion with internal fixation to provide additional support in
order to avoid hardware failure and development of a resid-
ual deformity [73].
Figure 31.8 depicts the author’s systematic approach for
assessment of the juxta-articular deformity of the ankle.
First, the length of the tibia relative to the fibula is assessed,
physeal integrity of the distal tibia and fibula is checked, and
Fig. 31.7 A radiograph showing asymmetrical premature physeal clo- orientation of the ankle joint in the coronal and sagittal
sure of the distal tibia in the presence of normally growing distal fibula planes is assessed. The location and extent of premature
in a 13-year- and 8-month-old boy physeal closure, angular or joint surface deformities, and
secondary osteoarthritis can be determined by using MRI,
bone realignment and fixation using an intramedullary lock- bone scintigraphy, and CT, together with plain radiographs
ing nail or fixator-assisted deformity correction and consecu- [74–76]. Documenting the magnitude of the angular deformity
tive lengthening over an intramedullary locking nail can also at other site(s) and LLD, if any, is also essential. When a
be performed in certain instances [69, 70]. However, while juxta-articular deformity of the ankle, with either a neutral
the technique of lengthening over an intramedullary nail heel or a valgus heel, is associated with a relatively short
using external fixation is used to reduce the time of external fibula due to partial deficiency or premature physeal arrest
fixation, caution is required to prevent deep infection in in the presence of intact physis of the distal tibia (Fig. 31.9),
patients who have a past history of bone and joint infection, it is imperative to determine whether lateral talar sublux-
or an open trauma [71, 72]. ation with or without compensatory subtalar varus defor-
Juxta-articular deformity of the ankle may develop as a mity and wedge-shaped epiphysis of the distal tibia coexist.
result of metaphyseal osteomyelitis of the distal tibia or a If possible, acute or gradual reduction of lateral talar sub-
primary septic arthritis of the ankle joint, involving the phy- luxation by external fixation should be performed first.
sis. Asymmetric or symmetric premature physeal closure of Other definitive and adjunctive surgical treatment methods
the distal tibia can be seen along with a normally growing that can be used alone or in combination include fibular
distal fibula (Fig. 31.7), or vice versa. When the length dis- lengthening [77, 78], supramalleolar corrective osteotomy
crepancy between the tibia and fibula is severe, secondary [79–81], hemiepiphysiodesis, and occasionally intra-articu-
distal tibiofibular dissociation can occur. Similar to the juxta- lar osteotomy [82]. Several techniques of supramalleolar
articular deformity of the knee, the ultimate goal of the treat- osteotomy for acute deformity correction have been
ment for juxta-articular deformity of the ankle is to obtain a reported, including oblique transphyseal supramalleolar
painless, stable plantigrade foot with a functional, mobile osteotomy [79], transverse supramalleolar osteotomy with
ankle joint. For this purpose, early stabilization of the talo- translation, Wiltse translational step-cut osteotomy [80],
crural joint by leveling the ankle and restoration of the axial and arc osteotomy. The oblique medial closing wedge oste-
alignment and joint orientation are of paramount importance. otomy allows for correction of the valgus deformity of the
Treatment of juxta-articular deformity of the ankle should be ankle and offers the advantage of improved bone healing as
individualized after considering the patient’s age, relative minimal periosteal stripping is necessary and the deformity
length discrepancy between the tibia and fibula, physeal is corrected by hinging the osteotomy at a point along the
integrity, presence or absence of lateral talar subluxation and bisector of the deformity [81]. Hemiepiphysiodesis of the
wedge-shaped epiphysis, intra-articular epiphyseal irregular- medial aspect of the distal tibia can be performed by using a
ity due to epiphyseal deformation, compensatory foot defor- transphyseal screw [83, 84], staple, or a tension-band plate
mity including subtalar joint subluxation/deformation, range [85, 86]. External fixator-assisted acute correction of ankle
of motion, surrounding soft-tissue condition, neurovascular valgus and fixation using a locking compression plate or
status, LLD, and other factors, in addition to the deformity multiple screws and gradual correction with external fixa-
itself. For instance, the use of internal fixation may not be tion are other viable options.
feasible in patients with a poor soft-tissue envelope and is In contrast, when a juxta-articular deformity of the
contraindicated in the presence of an active infection. ankle, with an apparently neutral heel, varus heel, and
598 I.H. Choi
Fig. 31.8 Author’s systematic approach for assessment of juxta-articular deformity of the ankle (see text). DT distal tibia, DF distal fibula
distal fibular lateral apex bowing, is associated with pre- Postinfectious Articular Deformity Causing
mature physeal arrest of the distal tibia in the presence of Knee and Ankle Joint Incongruity
intact physis of the distal fibula, the location and extent of
physeal damage of the distal tibia should be checked. In The residual deformity of septic arthritis of the knee and ankle
this situation, definitive and adjunctive surgical options joints may include partial epiphyseal destruction with a resul-
that can be used alone or in combination include physeal bar tant joint incongruity and accompanying joint instability, sub-
resection, supramalleolar corrective osteotomy [79, 81, 87], luxation, and impingement that may lead to rapid degenerative
distal fibular shortening and/or epiphysiodesis, tibial arthritis. In this situation, juxta-articular osteotomy alone can-
lengthening and subsequent epiphysiodesis, and occasion- not solve the problem of joint incongruity. Although intra-
ally an intra-articular osteotomy. When along with a juxta- articular osteotomy is not a common treatment option for the
articular deformity of the ankle there is a compensatory hip, knee, or ankle, intra-articular osteotomy can be selectively
deformity of the subtalar joint and/or calcaneus, the cor- performed to promote joint congruity and stability [82]. Paley
rection of this component must also be included in the [82] recently demonstrated, through case studies, that intra-
treatment plan. When supramalleolar corrective osteotomy articular osteotomy of the FH, the distal femur, or the tibial
is contemplated, the treatment option for acute versus plafond is technically feasible and can yield successful results
gradual deformity correction should be selected on an when performed for specific indications. When juxta-articular
individual basis after considering all the aforementioned deformity of the ankle is associated with partial epiphyseal
confounding variables. In patients who have previously deficiency or deformation causing joint incongruity, an intra-
undergone surgery for reorientation of the talocrural joint, articular osteotomy combined with or without juxta-articular
a second-stage lengthening of the proximal tibia and fibula osteotomy may be indicated. Theoretically, normalizing the
may be needed, near or at skeletal maturity for equalizing joint articulation between the two adjoining bones is desir-
the lower limb lengths. able for improving the joint congruency and stability.
Fig. 31.9 (a–c) A 25-year-old man presenting with a severe equinoval- and gradual distraction for correction of proximal angular deformity
gus deformity of the foot. He had a history of undergoing incision and using conventional Ilizarov construct and correction of distal angular
drainage and sequestrectomy for osteomyelitis of the entire fibula at deformity at the supramalleolar level using the hexapod system. Lateral
5 years of age. Compensatory proximal varus and distal anteromedial calcaneal closing wedge osteotomy was also performed. (e–g) At
bowing deformity of the tibia, and a calcaneal varus deformity, is seen 13 months after removal of the external fixator, which was applied for
(c). (d) Tibial osteotomy was made at three levels, for the purpose of 9 months, he regained full function of the lower limb with a plantigrade
acute deformity correction and plate fixation for diaphyseal angulation, foot and ankle joint motion from neutral to 20° of plantar flexion
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Iatrogenic Deformities
32
Austin T. Fragomen and S. Robert Rozbruch
are not significant with angular deformities less than 10° in

Introduction any plane. In over 25 years of observation small amounts of
shortening and angulation have not been associated with
As physicians we strive to help our patients improve their osteoarthritis or diminished function at a later date and are
pain and function. As orthopedic surgeons we do this through cosmetically acceptable to the patient [3]. Many patients
operative or nonoperative intervention. Our treatment choice were left with large rotation deformities of the femur and
is based on the standard of care and on our individual train- tibia, bone shortening, or angular and translational deformi-
ing and level of comfort. Despite the best intentions some ties (Fig. 32.2). Surgeons have moved toward more stringent
patients have deformities after treatments that are unaccept- criteria with less than 5° of angular deformity considered
able to the patients. Iatrogenic deformity refers to the cir- ideal [4–6]. The adoption of intramedullary nailing and mar-
cumstance where medical treatment contributed to the limb ginalization of casting as a standard of care for adult tibia
deformity. It may be that the management of a tibia fracture fractures has given surgeons the ability to obtain more accu-
with casting resulted in a deformity which the patient now rate fracture reductions [7]. Similarly, the management of
complains about as an adult (Fig. 32.1), or, perhaps, an ado- pediatric femur fractures has moved toward surgery making
lescent with Blount’s disease whose deformity was overcor- traction and casting less popular [8] (Fig. 32.3).
rected resulting in a valgus deformity.
What Are the Consequences

What Is Acceptable Alignment? of Malalignment?
There has been an evolution in what constitutes acceptable It is not uncommon to see adult patients with childhood
alignment among our peers. Through the 1990s all focus was deformities present to the office with knee or ankle arthritis
on achieving union with the following alignment dogma: secondary to the long-standing malalignment. This poor
less than 10° of angular deformity and less than 2 cm of alignment places abnormal or asymmetric stress across the
shortening were well tolerated [1, 2]. Sarmiento stated, “We adjacent joint(s) leading to premature cartilage breakdown
accept minor losses in length and alignment as small sacri- [7–10]. This contention is supported by Vallier et al. who
fices in an effort to provide early function and decreased found that a tibial malunion of more than 5° was significantly
morbidity.” Changes in stress patterns at the tibiotalar joint associated with knee and ankle pain [4]. Palmu found that
the treatment of femur fractures in children older than 10
years with traction led to a high incidence of deformities
A.T. Fragomen, MD (*) greater than 10° that he later correlated with the development
Department of orthopedics, Hospital for Special Surgery, of knee arthritis at a young age [8] (Fig. 32.4). We should
535 East 70th Street, New York, NY 10021, USA
strive to reestablish these normal parameters for bone align-
ment to avoid abnormal limb mechanics. This will help to
S.R. Rozbruch, MD
Department of orthopedics, Hospital for Special Surgery,
prevent articular cartilage wear and pelvic and low back
535 East 70th Street, New York, NY 10021, USA problems, and facilitate joint replacement, if and when
Weill Cornell Medical College, Cornell University,
needed. More importantly, if initial treatment results in unac-
New York, NY, USA ceptable alignment then we are obligated to consider the
e-mail: [email protected] long-term implications for this patient and suggest further

604 A.T. Fragomen and S.R. Rozbruch
Fig. 32.1 This segmental tibia fracture in an adolescent was treated

with casting and went onto malunion. The patient subsequently devel-
oped knee and ankle osteoarthritis
Fig. 32.3 This fracture was treated in adolescence with traction and
casting resulting in 16° of varus and 3 cm of shortening
Fig. 32.2 This distal tibial fracture was sustained during adolescence
and was treated with casting. The angulation of 10° was accepted and
the fracture healed uneventfully. The patient developed ankle arthritis
years later with more joint space narrowing seen in the medial aspect of Fig. 32.4 This varus femur malunion resulted in malalignment that
the tibiotalar joint developed medial compartment osteoarthritis years after the injury
32 Iatrogenic Deformities 605
surgical correction for this patient. If the surgeon is not If a child of advanced age presents with a displaced long bone
comfortable performing this “residual correction” then the fracture of the lower extremity that will likely go on to a mal-
patient should be referred to a specialized center. Many union (impending malunion) if treated with cast immobiliza-
patients will elect no further treatment or may not be candi- tion, the surgeon will often select operative treatment to help
dates for further intervention, but the malalignment needs to ensure acceptable alignment at skeletal maturity. The current
be acknowledged. criteria for “acceptable” alignment in pediatric fractures fall
into two groups. Children 8 years old or less often remodel
angular deformities of up to 10°. In children older than eight
What Are the Legal Implications and in adolescents, angular deformity of up to 5°, 1 cm of
of Iatrogenic Deformities? shortening, and less than 10° of rotational deformity is accept-
able [15]. Most remodeling occurs within 2 years after frac-
Nearly 15 % of orthopedic surgeons face a malpractice claim ture healing [15]. Due in part to a robust periosteum and rapid
annually [11]. Restricting the criteria for acceptable defor- healing, most tibia fractures in children can be treated with
mity is analogous to “narrowing the strike zone.” This places casting while still maintaining acceptable alignment, length,
increased responsibility on the surgeon to have a “perfect” and rotation [16]. However certain fractures are difficult to
result, thus making our job more difficult. If a malunion is a manage in a cast or are at high risk for displacement and mal-
failure and the criteria for malunion are now broadened then union and are often treated surgically: high-energy unstable
there are legal implications. Patients are glad to have their fractures, open fractures, fractures with soft tissue injury or
concerns about their deformity legitimized, but their next compartment syndrome, oblique fractures with an intact fib-
question is why the deformity occurred and whether this was ula, floating knee, and fractures in older children [16–19].
malpractice. Based on our experience, in most cases, the best Operative management of tibia factures is most commonly
policy is to concede that the result is not ideal, reassure that performed with elastic titanium intramedullary (IM) nails
every effort was made to provide an optimal result and that [20]. Circular external fixation has been shown to be superior
there was no medical negligence, and counsel that further to monolateral fixation for unstable diaphyseal fractures of
surgery to deliver a superior outcome is recommended. the tibia in children and adolescents and is gaining popularity
The greatest predictor of payment to a plaintiff is the severity [21]. Circular fixation may also be equivalent to elastic IM nail-
of the patient’s disability [12]. This implies that the best ing in adolescents [22]. External fixation may be superior to
course of action is to do everything possible to improve their casting as it allows for ease of monitoring and management of
condition and decrease disability. At no time should the sur- compartment syndrome, which remains one of the most com-
geon abandon or blame the patient. A good doctor-patient mon complications of pediatric tibia fractures [23, 24]. Use of
relationship and better preoperative discussions of known the programmable, six-axis circular fixator in high-energy
complications will help to calm the impulse to bring legal pediatric tibia fractures has proved successful affording very
action against the physician [13, 14]. Contact the hospital accurate alignment and early weight bearing [25, 26]. However,
legal department and identify this patient as “at risk” for liti- the use of external fixation is associated with a small incidence
gation. The hospital will often have patient services mitigate of refracture and fracture through the pin sites [27].
any feelings of discontent or help finance further treatment.
When a patient does seek legal action, an angry patient will Case 1
be less likely to settle the case. So, one must do as much as A 12-year-old male presented with a noticeable deformity of
possible to avoid a hostile relationship with the patient and the lower leg including varus, external rotation, and shorten-
his or her caretakers. One must keep in mind that we are ing. He had sustained a Salter–Harris type II fracture of the
judged not only by our successes but also by how we handle distal tibia at the age of 8 years. A metaphyseal osteotomy
adversity. These difficult situations are hurdles along the was performed for deformity correction and was stabilized
career path of every orthopedic surgeon. with a plate and screws. The growth plate became tethered
medially driving the epiphysis into varus and slowing the
growth of the entire physis (Fig. 32.5a, b). This was recog-
Common Iatrogenic Deformities nized by his orthopedist who had been following him yearly
after the injury. He was referred for deformity correction and
Tibia Malunion limb lengthening. The sum of these deformities led to sig-
nificant functional problems with walking and running and
The immature skeleton’s ability to remodel a “malunion” is put him at increased risk for developing arthritis of the ankle
determined by the number of years of growth remaining. If the and low back pain. He was noted to have limited ankle dorsi-
remodeling potential of an angulated bone is limited, then the flexion of the injured side compared with the unaffected left
fracture is considered a malunion and warrants intervention. ankle. His external rotation deformity of the tibia was 20°.
Fig. 32.5 (a) This patient has a malunion with shortening, external and double-level circular fixation for deformity correction and length-
rotation, and varus. (b) AP X-ray shows retained hardware, medial ening. (e, f) These X-rays show healing of both osteotomies with accu-
physeal tethering, and distal varus deformity. (c, d) AP and lateral rate correction of the underlying deformities. (g) Restoration of the
radiographs during treatment demonstrating the two-level osteotomy axial alignment of the tibia is assessed with the prone exam
He had a 2.5-cm shortening of the tibia. Surgical planning physical therapy 6 months after frame removal. Another option
included calculation of the future shortening that would would have been to lengthen the gastrocnemius muscle ten-
result from formal closure of the distal tibial physis. don for a more rapid recovery of ankle motion.
Correction with the six-axis circular fixator was considered a
good option. This would allow for deformity correction and Case 2
lengthening. The entire distal tibial growth plate was closed A high-energy closed tibia fracture in a 13-year-old male
surgically. A distal metaphyseal osteotomy was created with was treated with a monolateral pin-to-bar external fixation
an osteotome to allow for gradual correction of the varus and as the definitive stabilization. The reduction resulted in 2 cm
rotational deformities. Lengthening through the same oste- of lateral translation of the distal fragment and 10° of apex
otomy site has two disadvantages: the patient may develop a anterior angular deformity (Fig. 32.6a, b) The surgeon
significant equinus, and the time to consolidation of the explained that this was acceptable alignment and that he
lengthening regenerate would be lengthy. To address these was unable to improve it with closed reduction; because the
potential problems a second more proximal osteotomy was deformity was acceptable open reduction was not indicated.
performed for the lengthening (see Fig. 32.5c, d). The final The family sought a second opinion. This sagittal plane
result was correction of the deformities including length deformity is greater than 5° and will likely not remodel in
with a full functional recovery (see Fig. 32.5e–g) He had a this adolescent patient. The clinical concern about residual
contracture of the gastrocnemius that fully resolved with apex anterior deformity of the tibial shaft is that it may create
Fig. 32.6 (a, b) AP and lateral radiographs show a high-energy tibial bearing. (e) The prone exam is important to confirm that axial align-
fracture treated with temporizing external fixation. Soft-tissue swelling ment has been restored. (f, g) The final result is a healed fracture with
and damage made internal fixation a poor option. (c, d) The circular excellent alignment
external fixator provided superior stability and allowed for early weight
abnormal wear on the knee and ankle joints, increases stress Fibular Malunion
on the anterior cruciate ligament (ACL), and prevents full
leg extension. Trepidations about the fixation construct Fibular fractures in children and adolescents commonly heal
included further loss of reduction, impending malunion, and without incident. In rare circumstances growth arrest of the
even possible nonunion. Treatment with a six-axis circular distal fibular physis can occur causing progressive fibular
fixator using the Taylor Spatial Frame (TSF) (Smith & shortening. This leads to valgus orientation of the hindfoot,
Nephew, Memphis, TN) was provided. This device allowed widening of the syndesmosis, and early ankle joint degenera-
for a percutaneous surgery, an accurate and gradual correction [28–33]. Once recognized, this fibular shortening and
tion of the residual deformity, avoidance of the growth syndesmotic laxity can be corrected surgically [34–36].
plates, and weight bearing as tolerated ambulation during Manoudis et al. reported a case of fibular shortening from
the entire treatment period (see Fig. 32.6c–e) After 3 months physeal arrest corrected by open osteotomy, acute lengthen-
of wearing the external fixator, the final result was a nearing, grafting with tricortical iliac crest, and internal fixation
anatomic bony alignment and no functional impairment (see [37]. An open release of the syndesmosis was necessary to
Fig. 32.6f, g). reduce the fibular tip into its proper location. Other authors
Fig. 32.7 (a, b) AP radiographs

of the shortened right fibula and
the normal left fibula. Tibial
alignment is within normal. (c, d)
An external fixator was used to
lengthen the fibula using half
pins. (e) At the time of frame
removal the syndesmosis was
stabilized with two screws. (f)
Final radiographs show
restoration of the length of the
fibula and removal of the screws
have reported on the same technique without the need for unacceptable as it would lead to asymmetric wear of the lateral
syndesmotic dissection [29]. Alternatively, a percutaneous talus and possibly arthritis. Fibular shortening of 1.5 cm was
technique can be employed to both lengthen the fibula measured. The treatment plan included lengthening of the
through distraction osteogenesis and utilize distraction his- fibula. The tibia was included in the proximal fibular ring block
tiogenesis to stretch the syndesmotic ligaments and achieve to protect the proximal tibiofibular joint from subluxation and
distal migration of the fibular tip. ensure distal migration and reduction of the fibular tip
(see Fig. 32.7c, d). The fibula was lengthened with the exter-
Case 3 nal fixator. When the correct length was obtained the patient
A 13-year-old female presented with a valgus deformity of the was brought back to the operating room for insertion of syn-
hindfoot after premature fibular physeal closure. The patient desmotic screws and early removal of the frame (see Fig. 32.7e).
had previously undergone curettage of a benign distal fibular The patient’s hindfoot alignment was corrected with no loss of
bone cyst (without apparent physeal involvement) with pro- ankle motion (see Fig. 32.7f).
phylactic plating to prevent fracture of the distal fibula. The
hardware was removed 1 year later without incident. The dis-
tal fibular physis closed prematurely postoperatively leading Transphyseal ACL Reconstruction Causes
to a shortening deformity of the fibula over time. The etiology Premature Physeal Closure
of the physeal injury is not clear but an iatrogenic cause could
not be excluded. The patient’s hindfoot drifted into valgus An option for ACL reconstruction in older children (age
when compared with the other side. Radiographs revealed a 11–14 years) is the transphyseal tunnel technique. Most
shortened fibula with a distal physeal closure. X-rays of the authors report minimal complications with this method
contralateral ankle were taken to establish the normal fibular including an average growth plate disturbance risk of 3 %
length and talocrural angle (Fig. 32.7a, b). This deformity was [38–44]. Kumar et al. reported on 32 children with an average
Fig. 32.8 (a) This clinical photo shows a proximal tibial deformity fixator mimicking the deformity. The radiograph was taken near the
with shortening, varus, and malrotation. (b, c) AP and lateral X-rays end of the correction showing the proximal tibial lengthening. (f–h)
show varus and recurvatum angulation with closure of the anterome- Final clinical photo and radiographs demonstrate full correction of
dial physis. (d, e) This photo shows the early postoperative external the deformity
age of 11.25 years where the transphyseal reconstruction disturbance around the knee is small, its occurrence in some
was used with one case of mild valgus and no leg length cases is undeniable and such iatrogenic angular deformities
discrepancy [38]. Similarly, transphyseal surgery in a series may require surgical correction.
of 15 patients, average age 12.8 years, resulted in one case of
valgus malalignment of the knee [39]. Asymmetric growth Case 4
of the distal femoral physis is the most commonly reported A 15-year-old male presented with a deformity of the proximal
mechanism for the valgus deformity [41]. Other series report tibia including varus, recurvatum, and shortening. At age 12
no adverse trauma to the physis with this technique, but he sustained an ACL injury. The ACL was reconstructed
authors caution to make sure that the graft bone blocks are not using the transphyseal technique in the tibia and an over-the-
in contact with the physis [42–44]. Physeal sparing recon- top technique in the femur. The anterior-medial proximal
struction carries minimal risk to the growth plate but may not tibial physis was likely damaged. A progressive angular
be as reliable with regard to maintenance of ligamentous deformity developed with significant retardation of physeal
stability of the knee [45]. Although the risk of growth plate growth (Fig. 32.8a–c). The radiologic deformities included
15° of varus, 28° of apex posterior (recurvatum), and 4.5 cm increasing difficulty playing due to the lower limb deformity
of shortening. The clinical deformity included 10° of hyper- and knee pain. Partial closure of the lateral distal femoral
extension of the knee and normal knee ligamentous stability. physis caused a valgus deformity and 3 cm of shortening
Growth remaining discrepancy was calculated to be an addi- (Fig. 32.9a, b). The patient was at risk for knee arthritis, hip,
tional 1 cm. Treatment was closure of the remainder of the and low back problems. The proposed treatment would
proximal tibial physis to prevent further deformity, proximal address both the valgus deformity and the femur shortening.
tibial osteotomy, and TSF application for deformity correc- A distal osteotomy with lengthening is best done with a circu-
tion and lengthening (see Fig. 32.8d, e). The latest follow-up lar fixator, but the frame would need to be on the leg for a long
at 12 months post-frame removal shows restoration of length time and femoral rings are very uncomfortable. The LAP
and alignment, and a full functional recovery is expected (lengthening and plating) technique was selected. A femoral
(see Fig. 32.8f–h). osteotomy was created and stabilized with a TSF (see Fig. 32.9c).
The correction was performed gradually with lengthening.
Once the acceptable length and alignment were reached, the
Femur Malunion and Growth Disturbance patient was brought back to surgery for insertion of a plate and
screws and removal of the frame (see Fig. 32.9d, e). The femur
Distal femur physeal fractures are afflicted by a high rate of healed and the plate was later removed (see Fig. 32.9f, g).
physeal arrest and subsequent deformity [46]. Displaced frac- He returned to play soccer without any limitations.
tures and Salter–Harris II injuries are predictors of a poor out-
come with a 40 % risk of physeal bar formation [47]. Surgical Case 6
reduction and fixation are recommended, and smooth A 34-year-old female presented with a deformity of the right
Steinman pins crossing the physis do not seem to further femur and tibia. She had a femur fracture when she was 11
increase the already high risk of physeal arrest [46, 48]. years old that was treated with skeletal traction followed a
Distal femur malunion without growth plate arrest is few days later by casting. The traction pin was placed into the
another complication of pediatric femur fractures. Although proximal tibia at that time. She presented with a deformity of
the skeletally immature femur has a tremendous remodeling the right femur including valgus and shortening, and defor-
potential, angulation and shortening may not be tolerated as mity of the right tibia including recurvatum and shortening.
well as many surgeons believe. In children over 10 years old On examination she had knee hyperextension of 20°, valgus
varus-valgus angulation of greater than 5° and procurvatum- alignment, and lateral knee instability. The radiographs
recurvatum of greater than 10° should not be accepted revealed a 20° apex posterior deformity of the proximal tibia
because such deformities can be associated with premature with 15-mm shortening and 6° of valgus, relative proximal
knee arthritis [8]. migration of the proximal fibula, and 5° valgus deformity of
Treatment of diaphyseal and distal femoral deformities the distal femur diaphysis with 20 mm of shortening
that occur during childhood and subsequently present in (Fig. 32.10a, b). The patient has knee instability and pain.
adulthood can be accomplished with various implants includ- She was at a high risk for developing premature knee arthri-
ing IM nail, plate and screws, and external fixation. The tis and would have likely required living a life of activity
challenge is managing the femoral shortening that is often modification. The surgical plan included femur surgery to
present in these individuals. External fixation allows for both correct the valgus deformity and lengthen the femur.
deformity correction and lengthening simultaneously [49]. This was accomplished with a retrograde IM internal length-
Integrated fixation techniques provide the best of both ening nail. The plan also included a gradual correction of the
devices using external fixation to achieve length and defor- recurvatum and valgus deformities of the tibia with lengthen-
mity correction and then converting to internal fixation while ing. This included translating the intact fibula distally to
the immature bone consolidates [50–52]. The newer internal re-tension the lateral collateral ligament (see Fig. 32.10c, d).
lengthening nails provide an all-internal correction of defor- After removal of the hardware the patient is recovering well.
mity and bone lengthening solution [53]. The deformities have been corrected and the knee is stable
with normal extension (Fig. 32.10e–g).
Case 5
A 19-year-old male presented with femoral valgus and short-
ening. He had been struck by a vehicle when he was 11 years Vascularized Fibula Graft Nonunion and Valgus
old sustaining a distal femur physeal fracture. The fracture Ankle Deformity from the Fibular Donor Site
was stabilized with internal fixation. It is not known whether
the fracture was reduced anatomically or was stabilized with Reconstruction of the tibia after tumor resection with contra-
residual valgus angulation. If the initial postoperative align- lateral vascularized fibular autograft is an effective limb-
ment was appropriate then this may not qualify as an iatro- sparing procedure [54]. The rate of nonunion can be as high
genic deformity. He was an avid soccer player but was having as 20 % [55]. When the metaphyseal segment is small,
Fig. 32.9 (a, b) This clinical photo and radiograph show a valgus mal- ferently to expose the lateral femur for subsequent plating. Here the
union of the distal femur with shortening. The two medial screws sug- plate is being inserted. (e) This radiograph shows the plate spanning
gest a Salter–Harris 2 fracture with fixation. This X-ray was taken with the new regenerate bone. (f, g) Final radiographs show full union of the
the patient standing on blocks. (c) External fixation was used to distract osteotomy and ideal alignment
and angulate the distal femur. (d) The external fixator was mounted dif-
achieving stability with internal fixation can be challenging. effective in the short term but can be complicated by recurrence
Circular fixation is especially effective in these situations. of the deformity [57]. In this case report the guided-growth
Harvesting of the fibula for reconstruction of a bone defect technique is used to restore the distal tibial alignment resulting
with a vascularized fibular autograft can be associated with in normal ankle joint orientation at maturity.
valgus deformity of the donor-side ankle [56–58]. Tibia-fibula
syndesmosis synostosis has been recommended to prevent this Case 7
deformity, but valgus can occur even after this measure [59]. A 10-year-old male was referred for management of a non-
Prevention is also possible and has been successful with stabi- union and deformity at the junction of the proximal tibial
lization of the fibula to the tibia using a distal syndesmotic metaphysis and a vascularized fibular autograft. The patient
screw [58]. Correction of the iatrogenic valgus deformity had an osteosarcoma excision of the proximal tibia
with external fixation through a supramalleolar osteotomy is reconstructed with contralateral fibular autograft and flap.
Fig. 32.10 (a, b) These radiographs show valgus of the distal femur and recurvatum as well as the superior migration of the fibula were all
and proximal tibia as well as a 20° recurvatum deformity of the proxi- corrected with a tibial osteotomy and external fixation. (e–g) Final
mal tibia. (c, d) Correction of the valgus femur malunion and shorten- radiographs and a clinical photo show full healing and a successful
ing was performed with an internal lengthening nail. The tibia valgus correction of the deformities
Fig. 32.11 (a–c) The radiographs show tibial shortening and a nonunion Valgus deformity was developing at the donor-side ankle, which was
with angulation of the proximal graft-host junction. The vascularized treated with guided growth of the physis. (h, i) The final results were
fibular graft has hypertrophied but has failed to unite. The patient had a correction of the deformity, bony union, and correction of the donor-side
mobile nonunion with bowing and a lateral thrust. (d, e) Fixation with ankle valgus
fine wires afforded enough stability for bone healing to progress. (f, g)
The patient did not have recurrence of the tumor and the nonunion of the proximal graft junction (Fig. 32.11a–c).
diaphyseal junction of the graft healed satisfactorily. He had The treatment plan was to repair the nonunion and correct
pain, a varus and flexion deformity of the proximal tibia, and the deformity with fine-wire fixation. The proximal tibial
difficulty walking despite bracing. Radiographs revealed a segment was very small making it well suited for this technique.
Fig. 32.12 (a, b) A patient

underwent bilateral tibial
lengthening surgery by
lengthening the tibia with an
external fixator over an IM nail.
Once the desired length was
achieved the fixator was removed
and the patient started weight
bearing too early. These AP and
lateral (respectively) radiographs
show collapse and angulation
through the tibial lengthening
site. The immature bone could
not support weight bearing and
failed with angulation of the
regenerate and fracture of the
internal fixation
A percutaneous repair of the nonunion was performed by Deformity from Tibial Lengthening
making a small incision over the nonunion site and passing
a drillbit multiple times and in different planes to stimulate Lengthening of the tibia from congenital or acquired short-
the adjacent bone to bleed. A curette was then passed through ening can be accomplished using the methods of Ilizarov.
the drill holes to scrape the bone surfaces and further stimu- However there are unbalanced forces from the posterior
late bony bleeding. This approach stimulated a healing and lateral muscle groups acting upon the bone that cause
response while minimizing trauma to the poor soft tissue predictable deformity: valgus and procurvatum. The chal-
envelope (see Fig. 32.11d). The minimally invasive wires lenge of distraction osteogenesis is to create a straight
and pins accomplished the same goals of soft tissue preser- regenerate, thereby avoiding the creation of a tibial defor-
vation and provided excellent bony fixation. The deformities mity. This iatrogenic deformity needs to be anticipated
were corrected with the TSF and the nonunion was com- whether using external fixation or an internal lengthening
pressed (see Fig. 32.11e). Weight-bearing ambulation was nail. A further problem is bending of the new regenerate
allowed postoperatively. The patient was also noted to have bone when the external fixator is removed prematurely.
an iatrogenic deformity of the contralateral ankle. After the Even if the lengthening is done correctly, the new bone is
fibula was harvested the ankle had drifted into a valgus still at risk for developing angular deformity (Fig. 32.12a,
deformity. Radiographs showed a valgus deformity of the b). Both deformities that occur during lengthening and
distal tibia. This was addressed with guided growth using a after lengthening are considered malunions and are consid-
small extraperiosteal plate and non-locking screws to tether ered iatrogenic. The treatment of these malunions is either
the medial physis and improve the distal tibial valgus (see casting to prevent further angulation, internal fixation with
Fig. 32.11f, g) After frame removal of the side with the non- osteotomy to correct the current deformity, or external fixa-
union, the graft-host junction of the proximal tibia had healed tion with osteotomy. If the immature lengthening regener-
and the deformities were corrected including the contralat- ate bends and is managed early enough, it can be manually
eral distal tibial alignment. The patient underwent a distal realigned without osteotomy and stabilized with external or
femoral lengthening of the extremity with the proximal tibial internal fixation.
autograft 2 years later to make up for the residual leg length Contracture of the ankle and knee joints is a common
discrepancy. It was felt that the ipsilateral tibia had already complication of tibial lengthening surgery. Tightening of the
suffered enough trauma making a femoral osteotomy a wiser gastrocnemius muscle and fascia pulls the ankle into equinus
course of action (Fig. 32.11h, i). and the knee into flexion. A tibial lengthening of greater than
13 % of the length of the tibia bone and congenital etiology and desirable. Patients will often need reassurance that the
were risk factors for an equinus contracture. Further, gastro- translational “deformity” was intended.
soleus recession restored preoperative motion in all cases
[60]. Acute correction of these contractures should be Case 9
avoided to prevent undue stretching on the already tight A 42-year-old female had a correction of Blount’s disease at
nerves, especially the tibial nerve at the tarsal tunnel. When the age of 10 years. A varus and flexion deformity recurred
these contractures occur before the desired tibial length has over time and led to medial compartment arthritis (Fig. 32.14a,
been achieved then consideration should be given to stop- b). Preoperative radiographs showed a medial mechanical
ping or slowing down the lengthening process to allow for axis deviation (MAD), 17° of varus, and the apex of the
improved range of joint motion. deformity at the knee joint line (see Fig. 32.14c, d). The oste-
otomy performed distal to the tibial tubercle would require a
Case 8 lateral translation of 24 mm (see Fig. 32.14e). This would cre-
A 10-year-old male presented with shortening and deformity ate an iatrogenic deformity of the tibia in order to restore the
of the left tibia. He had no previous trauma, no syndrome, and mechanical axis and the proper joint orientation angles, the
no femoral involvement. He was felt to have a very mild fibu- medial proximal tibial angle (MPTA). A circular fixator was
lar hemimelia resulting in angular deformity and shortening used to achieve a gradual correction of the deformity and
of the affected tibia and fibula (Fig. 32.13a–c). He had normal translate the distal fragment laterally (see Fig. 32.14f). Final
range of motion and stability of both ankle and knee joints. radiographs demonstrate an apparent iatrogenic deformity of
There was 8 cm of shortening with additional shortening the proximal tibia (see Fig. 32.14g–j). However, the MAD
expected as growth continued. The goal was to obtain at least and MPTA have been restored, and the bone healed without
5 cm of shortening, which was an 18 % lengthening of the incident. It is likely that this translation, although technically
tibia. The patient was treated with double-level tibia osteoto- correct, will cause difficulty with conversion to a total knee
mies lengthened at 1.0 mm per day for a total of 2.0 mm per replacement.
day of limb lengthening (see Fig. 32.13d, e). This rate was
chosen to prevent premature consolidation at either lengthen-
ing site. The result of this rapid lengthening was equinus con-
tracture of the ankle and knee flexion contracture (see Box 32.1. Highlights
Fig. 32.13f). The patient and his mother were unable to stretch • The ability to correct deformity has improved, making
the contracture despite physical therapy. The patient under- anatomic restoration of bone more attainable.
went a gastro-soleus recession at the mid-distal-leg level with • Posttraumatic malunions are often seen with dis-
attachment of a foot ring and hinges. The ankle joint was displaced pediatric tibial shaft fractures and have directed
tracted acutely 3 mm to prevent articular cartilage compres- initial treatment in some adolescents toward surgical
sion. The correction of the equinus contracture was performed fixation rather than casting.
gradually with the foot ring. A spring-loaded hinged brace • Transphyseal ACL reconstruction can, in rare cases,
was attached to the tibial ring to help correct this knee flexion result in growth disturbance.
deformity (see Fig. 32.13g). Further lengthening was stopped. • Limb lengthening often creates predictable defor-
After frame removal a total of 5 cm of length had been mity that the surgeon must be able to anticipate,
achieved, and ankle and knee range of motion were eventu- recognize, and treat.
ally normal (see Fig. 32.13h, i). The patient can undergo • Intentional translation after osteotomy for periar-
further lengthening in the future. ticular deformities maintains proper limb align-
ment, but the bone may appear deformed to the
untrained eye.
Translational Deformity
Iatrogenic translational deformity can occur when an oste-

otomy is performed at a location away from the apex of the Summary
deformity. These osteotomies require a deliberate translation
of the bone fragments to ensure that the joints remain in line Iatrogenic deformity takes many forms (Box 32.1). The sur-
with the long axis of the tibia and the mechanical axis of the geon must consider factors such as the location and magni-
femur. These intentional translations may look like an acci- tude of the deformity and the growth remaining when
dental iatrogenic deformity when in fact they are planned deciding on treatment options. The tools available to repair
Fig. 32.13 (a–c) These radiographs show a two-level, congenital lengthening process. (g) The foot ring is seen in an overcorrected
deformity of the left tibia with significant shortening. (d, e) The proxi- position to prevent recurrence. The dynamic, spring-loaded brace is
mal osteotomy allowed for mild deformity correction and lengthening. seen bolted onto the proximal tibial ring delivering an extension
The distal osteotomy allowed for deformity correction and mild moment. (h, i) Final X-rays show full healing, correction of the defor-
lengthening. (f) Ankle and knee contractures are apparent during the mity, and successful attainment of length
malunions and restore alignment have improved, and the joint replacement. Further research will help determine more
outcome of surgical reconstruction has become more pre- accurate parameters for acceptable alignment. Advances in
dictable. Patients with deformity need not suffer for the technology will continue to make anatomic restoration of
remainder of their lives or until they are old enough for a deformities and fractures more easily attainable.
Fig. 32.14 (a, b) Preoperative radiographs demonstrate varus and flex- in addition to the angular correction of 17°. (f) Circular fixation is ideal
ion deformities of the right proximal tibia. (c, d) The MAD is 52 mm for executing angular and translational movements of the bone. (g, h)
medial to the midline, and the MPTA is 68°. (e) This represents a 17° Final radiographs after frame removal show what looks like an extreme
varus deformity. The CORA is near the knee joint line. The planned amount of translation at the osteotomy site. (i, j) Mechanical axis and
osteotomy is below the tibial tubercle. In order to prevent a translational joint orientation analysis exhibit a slight lateral MAD and an MPTA of
deformity the distal bone fragment must be translated 24 mm laterally 87°; both were ideal for this patient
22. Gordon J, Gregush R, Schoenecker P, et al. Complications after

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Index
A physeal injury, angular deformity secondary, 84

Acetabular dysplasia, 535 planning, governing rules, 89–90
Acetabuloplasties, 538 proximal femur, 86–88
Achondroplasia, 192–194, 319–321 screws, 93
Achterman and Kalamchi classification, 428–429 shortening osteotomy, 83–84, 89
ACL. See Anterior cruciate ligament (ACL) supracutaneous locked plating, 96, 99
Acute complications tension band wiring, 93–94
compartment syndrome, 98–99 tibial deformity correction, 90–91
iatrogenic fractures, 99 Adolescent Blount’s disease, 510–511
neurological, 98, 99 intermediate types, 514
physeal injury, 99 treatment considerations
skin and wound problems, 99 acute correction with internal fixation, 513–514
vascular injury, 99 acute/gradual correction with external fixation, 514
Acute deformity correction associated deformities, 512–513
acute vs. gradual correction, 101 obesity and impact, 511–512
age considerations, 79, 80 AER. See Apical ectodermal ridge (AER)
bone gap creation, 79 Aitken classification, 368–369
long-standing deformities, 79 Amputation management
lower limb alignment, 79, 81 cheetah type carbon blades, 199, 200
osteotomy congenital femoral deficiency
acetabulum, 88 Gillespie type A, 201, 202
acute complications (see Acute complications) Gillespie types B and C, 202–204
adjuvant fibular, 81, 85 Hamanishi classification, 201, 202
adjuvant procedures, 88–89 Paley classification, 201, 203
angular correction and translation, 82–83 congenital fibular deficiency
blade plate, 94 Birch classification, 205
bone void fillers, 95 Brown procedure, 205
closed wedge osteotomy, 81, 82 Symes amputation, 205–206
Cozen’s phenomenon, 84, 85 congenital pseudarthrosis, 206, 208, 209
deformity memory effect, 84, 85 congenital tibial deficiency
diaphysis, 86, 87 Jones type 1a classification, 206, 208
disease-specific indications, 89 Jones type 2 classification, 206, 208, 209
dome osteotomy, 81, 85 Jones type 4 classification, 206, 209
epiphyseal, 85 radiologic description, 206, 207
external fixation, 94–95 fibular hemimelia, 199, 200
FAP technique, 95–98 Ilizarov approach, 199
femoral deformity correction, 91–92 knee fusion, 199, 200
influence of, 81 marked anterolateral bowing, 199, 201
intramedullary devices, 94, 95 patient and family management, 201
juxta-apophyseal, 85, 87 short tibia, 199, 201
Kirschner wires, 93 trauma, 210, 211
knee, 88 treatment concepts, 199–200
late complications (see Late complications) tumor reconstruction, 208, 210–211
LCP, 94 two ray foot, 199, 201
length discrepancy, 88, 89 volume changes, 210
lengthening osteotomy, 83–84 Androgens, 528
limb reconstruction systems, 94–95 Angular correction
mango slice effect, 84 bilateral, 52
metaphysis, 85, 86 frontal
multiple bony deformities, 92–93 anatomic angles, 54
oblique osteotomy, 83 epiphysiodesis, 53
opening wedge osteotomy, 81, 82 follow-up, 54–56

DOI 10.1007/978-3-319-17097-8, © Springer International Publishing Switzerland 2016
622 Index
Angular correction (cont.) ASTYMT, 184, 188, 194

guided growth, 54, 58–60 Autogenous cancellous bone grafting, 408
Keith needle insertion, 54 Avascular necrosis (AVN), 11, 573–575
longest practical screw, 54
mechanical axis, 53, 54
open/percutaneous techniques, 53 B
pathologic, 53 Beckwith–Wiedemann syndrome, 7–8
physiologic, 52 Benign bone lesions
management, 60, 61 dysplasia epiphysealis hemimelica, 343–344
osteotomy, 82–83 enchondromatosis, 338–340
patient examination, 52 fibrous dysplasia
sagittal/oblique anteroposterior, 334, 335
equinus/posterior plate, 58 bisphosphonate agents, 336
knee, 56–58 definition, 333
osteotomy, 58 diagnosis, 333
progressive ankle valgus, 56–58 differential diagnosis, 335
staples, 51 flame-shaped appearance, 334
Ankle and foot ground glass appearance, 334
decision making limb length discrepancy, 336
assessment tools and indications, 160–161 malignant degeneration, 337
cosmetic improvements, 158 Mazabraud’s syndrome, 334
diagnostic matrix, 153, 154 McCune Albright syndrome, 334
gait cycle (see Gait cycle) monostotic and polyostotic fibrous dysplasia, 334
intraoperative assessment, foot pusher, 159 orthopedic management, 335
level I, II and III deformities, 159 osteotomy, 336, 337
segmental malalignment patterns, 157–158 pathological fractures, 335, 336
skeletal surgeries, 158–159 RANKL, 336
soft tissue surgeries, 158 recurrence, 335
equinocavovarus deformity, 273, 275 scoliosis, 336
equinoplanovalgus deformity, 272, 274 shepherd’s crook deformity, 335–336
equinus contracture, 272, 274 MHE, 341–343
myelomeningocele (spina bifida), 290 nonossifying fibroma, 333, 334
normal and abducted foot, 273, 274 osteochondromas, 341
orthopedic procedure osteofibrous dysplasia, 337–338
foot osteotomy, 288–289 Benign tumors, 4, 6
gastrocnemius and soleus lengthening, 287–288 2-β-(3-hydroxypropoxy)- 1α, 25-dihydroxyvitamin D3 (ED-71), 526
SPLATT, 288 Birch classification, 205, 429–431
SPOTT, 288 Bisphosphonates, 256, 260, 264, 491–492, 528
Anprolene®, 221 Blocking screws, 113, 138
Antegrade femoral lengthening Blount’s disease, 503
patient indications, 138–139 adolescent (see Adolescent Blount’s disease)
planning for, 139 classification, 503, 504
surgical technique, 139–141 differential diagnosis, 503, 505
Anterior cruciate ligament (ACL), 363 gradual deformity correction, 108
Anterolateral tibial bowing, 9 late-onset/adolescent, 10–11
Anteromedial tibial bowing, 9 natural history, 505–506
Apical ectodermal ridge (AER), 361 physeal growth disturbance, 67
Arthrogryposis physeal injuries, 79, 80
ambulation, 309 proximal tibia intra-articular deformity, 46
clinical picture, 300–301 quad plates, 51
congenital contracture, 297, 298 treatment
etiology, 297 acute deformity correction, 507
genetic aspects, 300 atraumatic meniscal tears, 509
Hall classification system bracing, 506
group 1, 298 growth modulation, 506–507
group 2, 298–299 hemi-plateau elevation, 509
group 3, 299–300 high tibial osteotomy risks, 507
intellectual skills, 300 physeal arrest resection, 507–508
lower limb (see Lower limb management) physeal bar resection, 508
physical examination, 300 Body mass index (BMI), 16
prevalence, 297 Bone grafting methods, 408
prognosis, 301 Bone marrow aspirate concentrate (BMAC), 522
rehabilitation, 309, 310 Bone marrow cells (BMCs), 522
timing, 308–309 Bone morphogenetic protein (BMP), 489, 491
upper limb, 308 Bone transport, 125–127
Arthrogryposis multiplex congenita, 4, 6 Bovie cord technique, 96
Index 623
C mechanical axis, 28–29

Calcitonin, 528–529 oblique plane deformities, 32–34
Campanacci’s disease. See Osteofibrous dysplasia single-exposure weight-bearing teleroentgenogram, 28
Center of rotation of angulation (CORA) rickets, chemical findings, 34, 35
femoral deformity correction, 91–92 routine radiographic screening, 22
osteotomy, influence of, 81 version diagnostic imaging
sub-capital realignment osteotomy, 87 acetabular version, 25–26
tibial deformity correction, 90–91 femoral version, 26
Cerebral palsy (CP) tibial torsion, 26
ankle and foot (see Ankle and foot) Closed wedge osteotomy, 81, 82
causes, 267 Clubfoot, 225–226
DMD, 293 Computerized tomography (CT)
GMFCS, 267–268 acetabular version, 25–26
hereditary motor sensory neuropathy CFT, 367
diagnosis, 291 femoral version, 26
foot deformity, 292 limb length assessment, 24
hip deformity, 292–293 physeal arrest, extent of, 71–72
HSMN type 2/CMT type 2, 291 tibial torsion, 26
HSMN type 1/CMT type 1A, 291 Congenital coxa vara, 9
knee and lower leg segment (see Knee and lower leg segment) Congenital dislocation, 9
lower limb assessment (see Lower limb assessment) Congenital disorders
musculoskeletal pathology, 268–269 congenital coxa vara, 9
myelomeningocele (spina bifida), 289 congenital dislocation, 9
ankle and foot deformity, 290 congenital femoral deficiency, 8
hip dysplasia, 290 congenital fibular deficiency, 8
knee flexion deformity and tibial torsion, 290 hemihypertrophy and hemiatrophy, 7–8
optimal timing, 282 longitudinal tibial deficiency, 8
pelvis and hip (see Hip) tibia bowing, 9, 10
poliomyelitis, 290–291 Congenital femoral deficiency (CFD), 8, 191, 192
prevalence, 267 classification systems
risk factors, 267 Aitken, 368–369
treatment principles, 282 Gillespie and Torode, 369–371
CFD. See Congenital femoral deficiency (CFD) Hamanishi, 371
Charcot–Marie–Tooth disease (CMT), 5–6, 16, 291, 292 Kalamachi, 371–372
Cheetah type carbon blades, 199, 200 Paley, 372
Chondroectodermal dysplasia, 329–330 Pappas, 369
Cidex®, 221 complications and treatment
Clinical evaluation fractures, 409, 411
coronal plane angulation, 27–28 hip subluxation/dislocation, 408–410
deformity, definition of, 15 incomplete osteotomy and premature consolidation, 408
history, 15–16 joint stiffness and contracture, 413
limb length assessment, 23 knee subluxation/dislocation, 409
AP and lateral alignment radiographs, 23, 24 limb malalignment, 409
CT scanograms, 24 limb reconstructive surgery, 419–420
orthoroentgenography, 25 nerve injury, 407
single-exposure standing teleroentgenogram, 22, 24 pelvic support osteotomy, 415, 418–419
standardized radiographic technique, 22, 24 poor bone formation, 407–408
three-view scanogram, 24–25 prosthetic reconstructive surgery, 420–422
X-ray, 24 superhip 1.5 technique, 413–415
pelvic radiograph, 22 superhip 2 technique, 414–416
physical examination type 2 treatment, 413, 414
equinus, 18 type 3 treatment, 419
foot progression angle, 17 deformity and pathoanatomy
neurologic, 17 ligamentous structures, 363–364
prone exam (see Prone exam) muscle, 364–365
short leg gait, 18 osseous deformities, 363
spine and trunk, 17 vascular, 365–366
supine exam, 18–19 embryology, 361–362
trendelenburg sign, 18 evaluation
upper extremity, 17 CT, 367
vital signs, 16–17 history, 366
quantitative deformity analysis MRI, 367
anatomic axis, 29, 30 physical exam, 366
deformity components, 28 radiographic examination, 366–367
deformity detection, 29, 31 Gillespie type A, 201, 202
joint orientation angles (see Joint orientation angles) Gillespie types B and C, 202–204
624 Index
Congenital femoral deficiency (CFD) (cont.) Congenital posteromedial bowing

Hamanishi classification, 201, 202 deformity
knee considerations in, 391 ankle and foot, 496, 497
knee flexion contracture release, 394–396 fibula, 496, 497
Langenskiöld patellar realignment, 394 tibia, 496
often-cited incidence, 361 demographic features, 495
Paley classification, 201, 203 diagnosis and management, 501, 502
Paley type 1 etiology, 495
botulinum toxin injection, 401 evaluation, 498–499
femur/tibia, serial lengthenings, 375–376 history
hemiepiphysiodesis and epiphysiodesis, 376 residual deformity, 497, 498
hip and knee, preparatory surgery, 375 shortening, 497
knee instability, 401 spontaneous remodeling, 497, 498
limb lengthening, soft tissue releases, 399–401 weak plantar flexion, 498, 499
osteotomy level, 398 incidence, 495
superhip procedure, 377 treatment
Paley type 2 treatment, 413, 414 calcaneo-valgus foot deformity, 499–500
pathophysiology and genetics, 362–363 coronal deformity, 500
pelvic osteotomies indications, 499
Paley-Dega osteotomy, 388–389 limb length inequality, 500, 501
Paley PATO, 389–390 motivation, 499
PAO, 390–391 muscle imbalance, 500, 501
peroneal nerve decompression, 396–397 sagittal deformity, 500
preparatory surgery, 391 torsional deformity, 500
superhip surgical technique treatment planning, 501
abductor muscle slide, 385 valgus ankle, 500
arthrogram, 386 Congenital pseudarthrosis of the tibia (CPT)
blade plate insertion, 386 amputation, 488, 491
BMP insertion, 387 anatomy and pathology, 483, 484
closure, 387–388 associations, 483
distal femur fixation, 387 bisphosphonates, 491–492
external rotation contracture release, 385 BMP, 489, 491
fascia lata release, 384–385 bone marrow grafting, 492
femoral shortening, 386–387 combined approach, 492
flap elevation, 384 Crawford classification system, 484
guide wire insertion, 386 electrical stimulation, 492
hip flexion contracture releases, 385 FVF, 487
iliac wing osteotomy, 387 Ilizarov circular external fixator, 487–488
incision, 384 imaging, 485
muscle repairs and transfers, 387 intramedullary nailing, 486–487
pelvic osteotomy, 387 Masquelet’s procedure, 488, 491
periosteum release, 386 medical management, 489
positioning, prepping, and draping, 378–384 modified McFarland bypass graft technique, 485–486
postoperative course, 388 non-operative management, 485
quadriceps elevation, 386 operative management, 485
subtrochanteric osteotomy, 386 periosteal grafting, 488–490
superknee surgical technique, 392–394 prognostic factors, 485
surgical technique, 401–402 Congenital tibial deficiency
fixator removal and rodding, 406 Jones type 1a classification, 206, 208
Ilizarov™ fixator technique, 402–403 Jones type 2 classification, 206, 208, 209
modular rail system technique, 404–405 Jones type 4 classification, 206, 209
Orthofix™ fixator technique, 403–404 radiologic description, 206, 207
prophylactic rodding, 406–407 Continuous passive motion (CPM) machine, 560
rehabilitation and follow-up during lengthening, 405–406 CORA. See Center of rotation of angulation (CORA)
valgus ± flexion deformity, 405 Cortical allograft, 408
treatment options Coventry and Johnson classification, 428, 429
limb lengthening, 373–375 Cover-up test, 18–19
rotationplasty, 372–373 Coxa breva, 535, 551–552
syme amputation, 373 Morscher osteotomy
type 3 deformity correction and fixation, 552–554
limb reconstructive surgery, 419–420 positioning and exposure, 552
prosthetic reconstructive surgery, 420–422 postoperative care, 554–556
treatment, 419 Coxa magna, 535, 556–557
Congenital fibular deficiency, 8 femoral head reduction osteotomy
Birch classification, 205 deformity correction and fixation, 558–560
Brown procedure, 205 positioning and exposure, 557–558
Symes amputation, 205–206 postoperative care, 560, 561
Index 625
Cozen’s fracture, 42 FGF-2, 526

Cozen’s phenomenon, 52, 84, 85 GH, 527–528
CP. See Cerebral palsy (CP) growth factors, 523
CPT. See Congenital pseudarthrosis of the tibia (CPT) hyperbaric oxygen therapy, 528
Crawford classification system, 484 injection technique, 524–526
Custom knee device (CKD), 185–187 local application-BMP, 522
optimal timing, 526–527
osteogenic progenitor stem cell culture
D technique, 524
Decision making PTH, 527
clinical evaluation, 37–38 scaffolds, 523–524
contraindications, 39–41 stem cells, 523
equinus contracture TP510, 524, 526
external fixation, 167–169 disadvantages, 357
muscles and tendons, lengthening of, 165–167 indication, 349, 350
nonoperative, 164–165 physical stimulation
knee extension contractures LIPUS, 520–521
nonoperative technique, 176 mechanical stimulation, 520
quadricepsplasty, 176–178 PEMF, 521
knee flexion contracture, 171 regenerate bone monitoring, 519–520
bony correction options, 173–174 type I, 349, 350, 355, 356
gradual soft tissue correction, 174–175 type II, 351
nonoperative methods, 172 type III, 352
soft tissue releases, 173 type IV, 353
osteotomy (see Osteotomy) DO. See Distraction osteogenesis (DO)
in pediatric foot and ankle surgery Dome osteotomy, 81, 85
assessment tools and indications, 160–161 Drill hole technique, 236, 239
cosmetic improvements, 158 Duchenne muscular dystrophy (DMD), 293
diagnostic matrix, 153, 154 Duncan–Ely (DE) sign, 280
gait cycle, ankle and foot function (see Gait cycle) Dunn procedure
intraoperative assessment, foot pusher, 159 deformity correction and fixation, 545–546
level I, II and III deformities, 159 positioning and exposure, 545
segmental malalignment patterns, 157–158 postoperative care, 546–547
skeletal surgeries, 158–159 Dysplasia epiphysealis hemimelica, 343–344
soft tissue surgeries, 158
physeal bar resection, 42
advantages, 40 E
complete epiphysiodesis, 40 Ellis-van Creveld (EvC) syndrome, 329–330
disadvantages, 40 Endocrine disorders, 3
hemiepiphysiodesis, 40, 42 Equinocavovarus, 157, 158
oblique plane deformity, 42, 44 Equinoplanovalgus, 157
proximal tibial valgus deformity, 42, 45 Equinus contracture, 157, 163
in right distal femur, 40, 43 clinical evaluation, 163
problem lists, 38 imaging, 164, 165
soft-tissue modification, 40, 42 management strategy and decision making
surgeon skills and patient/caretaker preferences, 37 external fixation, 167–169
surgical indications, 38–39 muscles and tendons, lengthening of, 165–167
ankle, 39, 40 nonoperative, 164–165
knee, 39 pathoanatomy, 163–164
Dega osteotomy, 538 Estimated blood volume (EBV), 220
Developmental dysplasia of the hip (DDH), 11, 20 Extensor hallucis longus (EHL), 189
Diastrophic dysplasia, 299
with hitch hiker thumbs, 323, 325
laterally dislocated patellae, 323, 325 F
operative correction, 324, 326 Familial hypophosphatemic rickets. See Vitamin D-resistant rickets
rigid clubfeet and hallux varus, 323, 325 FAP technique. See Fixator-assisted plating (FAP) technique
rigid equinovarus foot, 324, 326 Femoral deformity correction, 91–92
supramalleolar osteotomies, 324, 326 Femoral head reduction osteotomy
Distraction osteogenesis (DO), 106, 121, 132, 519 deformity correction and fixation, 558–560
advantages, 357 positioning and exposure, 557–558
anticatabolic therapy postoperative care, 560, 561
bisphosphonate, 528 Femoral metaphyseal-diaphyseal angle (FMDA), 27
calcitonin, 528–529 Femoral neck osteoplasty, 549–550
biological stimulation, 522 percutaneous osteotomy and external fixation, 550
androgens, 528 postoperative care, 550
bone marrow cells/platelet-rich plasma, 522, 523 Femoral-tibial ratio (FTR), 27
ED-71, 526 Femur malunion, 608
626 Index
FH. See Fibular hemimelia (FH) type 3b, 447, 448

Fibroblast growth factor-2 (FGF-2), 526 osteotomy, 444, 448
Fibrous dysplasia positioning, 444
benign bone lesions surgical approach, 444
anteroposterior, 334, 335 type 3c, 448, 449
bisphosphonate agents, 336 osteotomy, 448
definition, 333 positioning, 448
diagnosis, 333 surgical approach, 448
differential diagnosis, 335 type 3d, 449–450
flame-shaped appearance, 334 upper extremity manifestations, 427
ground glass appearance, 334 Fibular malunion, 605–606
limb length discrepancy, 336 Fibular osteotomy, 81, 85
malignant degeneration, 337 FITBONE device, 133, 145
Mazabraud’s syndrome, 334 Fixator-assisted plating (FAP) technique, 95–96
McCune Albright syndrome, 334 advantage, 96
monostotic and polyostotic fibrous dysplasia, 334 CORA, 96
orthopedic management, 335 disadvantage, 96
osteotomy, 336, 337 external fixator pins, 96
pathological fractures, 335, 336 femoral shaft, 96, 97
RANKL, 336 Ilizarov frame, 96, 98
recurrence, 335 indications, 95
scoliosis, 336 LCP/DCP, 95
shepherd’s crook deformity, 335–336 locked plates, 95, 96
genu valgum, 11 metaphyseal plates, 95
Fibular hemimelia (FH), 191–192, 199, 200. See also Congenital monoplanar external fixator, 95
fibular deficiency proximal and distal pins, 96
classification straight narrow plates, 96
Achterman and Kalamchi, 428–429 Taylor spatial frame/multiaxial correction frame, 96
Birch, 429–431 Fixed knee flexion deformity (FKFD), 56–58
Coventry and Johnson, 428, 429 Free vascularized fibular (FVF) graft, 487
Paley, 431–433
Stanitski, 429, 430
clinical assessment, 433, 434 G
diagnosis, 449 Gabapentin (Neurontin™), 189
incidence, 427 Gait cycle
lower extremity anomalies, 427, 428 adduction/abduction, 153
outcomes, 451–453 external rotation, 153
Paley type 2 lengthening plus ankle realignment, 441, 444 foot columns, 153, 154
Paley type 3 lengthening plus super-ankle reconstruction, 441 foot segment, 153, 154
Paley type 1 lengthening procedure internal rotation, 153
full-length standing, 438, 440, 441 pronation, 153
LON technique, 440–442 skeletal alignment, 155
open Vulpius procedure, 438, 439 stance phase, 153
PRECICE, 441, 443 first/heel rocker, 155
prophylactic anterior compartment fasciotomy, 438, 440 second/ankle rocker, 155, 156
post-surgical care, 450–451 terminal stance, 155
prevalence, 427 third/forefoot rocker, 155
radiographic assessment supination, 153
arthrogram, 434, 438 swing phase, 153, 156
classic “C” sign, 434, 436 Ganz periacetabular osteotomy (PAO), 390–391, 539, 540
full-length standing AP view, 433, 434 Gastrocnemius tightness, 196
full-length standing lateral view, 433, 434 Genetic disorders, 4
with moderate deformity, 434, 435 NF, 4–5
Paley type 3, 434, 437 osteogenesis imperfecta, 3–4
with severe deformity, 434, 435 Genu valgum, 11
talo-calcaneal coalition, 434, 436 Genu varum, 4
talus and calcaneus overlap, 434, 435 Blount’s disease, 10–11
talus and calcaneus side by side, 434, 436 physiologic genu varum, 9
talus and calcaneus stack, 434, 435 tibia vara, 10, 11
treatment, 427, 436–438 Gigli saw osteotomy technique, 236, 239
type 4, 450–452 Gillespie type A classification, 201, 202, 369–371
type 3a, 445–446 Gillespie types B and C classification, 202–204, 369–371
frame application, 444 Gradual deformity correction
osteotomy, 444 analysis of
positioning, 442 alignment, 109
surgical approach, 442, 444 AP and lateral radiographs, 109
Index 627
bone density, 109 HSMN. See Hereditary motor sensory neuropathy (HSMN)
degree and location, 109 Hydrotherapy, 187–188
delayed healing secondary, 109, 112 Hyperbaric oxygen therapy, 528
hemangiomatosis and tibial bowing, 109, 111 Hypertonia, 268–269
length measurement, 109 Hypophosphatasia, 232
planning, 108–109 ALP, 244
unossified epiphyses, 110 with bilateral femoral varus deformities, 244–246
biology, 106 with genu valgum deformity, 244, 246–248
Blount disease, 108 with genu varum deformity, 244, 248–250
complications, 108 TNSALP, 244
Ellis-van Creveld disease, 106, 108 X-linked rickets, 244, 251–252
history, 105
hypophosphatemic rickets, 106, 107
internal lengthening devices, 113 I
intramedullary rod, blocking screws, 113, 116 Iatrogenic deformities
joint subluxation and dislocation, 112, 115 acceptable alignment, 601, 602
limb length inequality, 106, 109 femur malunion and growth disturbance, 608
locking plate, 113 fibular malunion, 605–606
MACT, 112–113 legal implications, 603
monolateral fixation device, 112, 115 malalignment, consequences, 601–603
osteotomy, 110, 112, 113 premature physeal closure, 606–608
postoperative care, 114, 116 tibial lengthening, 612–613
potential complications, 116–119 tibia malunion, 603–605
recurvatum, 114 translational deformity, 613–615
severe deformity, 106 vascularized fibula graft nonunion and valgus ankle deformity,
translation and angular deformity, 106, 107 608–612
untreated clubfoot, 114 Iatrogenic injury, 4, 12, 576–577
Graston™, 184 Idiopathic hemihypertrophy (IH), 7
Greater trochanteric arthroplasty (GTA), 592–593 Ilizarov approach, 199
Gross motor function classification system (GMFCS), 267–268 Ilizarov™ fixator technique, 402–403
Growth hormone (GH), 527–528 Ilizarov hip reconstruction, 415
Ilizarov method, 121, 167
Inflammatory disorders, 4, 6
H Informed consent, 219
Hamanishi classification, 201, 202, 371 Infratrochanteric torsion (ITT), 26
Health Inter Network Access to Research Initiative (HINARI), 228 Internal lengthening devices, 194–195
HemoCue device, 219 Internal limb lengtheners, 132–134
Hereditary motor sensory neuropathy (HSMN) Intramedullary skeletal kinetic distractor (ISKD), 132
diagnosis, 291 Intraoperative nail testing, 144–145
foot deformity, 292 I-STAT, 219
hip deformity, 292–293
HSMN type 2/CMT type 2, 291
HSMN type 1/CMT type 1A, 291 J
Hip Joint line congruency angle (JLCA), 39
coxa valga, 270, 271 Joint orientation angles
distraction treatment in adults, 30
application, 564 anatomic/mechanical, 30, 32
core decompression, 563 deformity magnitude, 30
patient positioning, 562 femur and tibia, 30, 31
postoperative care, 564, 565 long segment method, 30, 32
soft tissue releases, 563–564 short segment method, 30, 32, 33
stem cell injection, 563 Jones classification, 206, 208, 209
dysplasia, 290 Juxta-articular deformity
femoral anteversion, 270, 271 ankle development, 597
femoral head dislocation, 270, 272 correction of, 596
hip at risk progress, 270, 272 joint incongruity, 598
orthopedic procedures knee development, 593
adductor releases, 285 systematic approach, assessment, 598
femoral derotation, 285 ultimate goal of, 594
femoral head resection, 286
iliopsoas lengthening, 285
periacetabular pelvic osteotomy, 285–286 K
varus derotational osteotomy, 285 Kalamachi classification, 371–372
quantifying hip displacement and acetabular dysplasia, Knee and lower leg segment
270, 271 bilateral patella alta, 272, 273
windswept hips, 269, 270 functional contracture, 271
628 Index
Knee and lower leg segment (cont.) type I, 349, 351

gastrocnemius contracture, 271 type II, 352
orthopedic procedures type III, 353
anterior hemiepiphysiodesis, 286 type IV, 354, 355–357
distal femoral extension osteotomy, 286 type V, 354–355
distal tibial (supramalleolar) derotational osteotomies, 287, 288 type VI, 355
hamstring lengthening, 286 Locked plates (LCP), 94
patellar tendon shortening/advancement, 286, 287 Longitudinal tibial deficiency, 8
rectus femoris transfer, 287 Lower limb assessment
posterior capsular contracture, 271 ambulant child (GMFCS levels I-III)
torsional malalignment, 272, 273 apparent equinus pattern, 276, 277
true coronal plane deformity, 272 bony alignment, 280
weak plantar flexion-knee extension couple, 272 crouch gait, 277
Knee flexion contracture, 172 gait observation, 275
anterior cortical lines, 171, 172 improve independence and physical function, 281
biomechanics, 170 internal rotation gait pattern, 278
bony correction options, 173–174 jump gait pattern, 276
clinical evaluation, 170 muscle strength and selective control, 280–281
decision making, 171 muscle tone and length, 278–280
etiology, 169 optimize gait efficiency, 281
gradual soft tissue correction, external fixation, 174–175 stiff knee pattern, 277
nonoperative methods, 172 true equinus pattern, 276
radiographic evaluation, 171 unilateral CP (hemiplegia), 278, 279
soft tissue releases, 173 non-ambulant child (GMFCS levels IV-V), 281
Lower limb management
foot, 301–304
L hip, 307–308
Larsen syndrome, 299, 308 knee
Late complications with external fixator, 305, 306
cast-related complications, 100–101 knee extension contractures, 305–306
hardware-related complications, 100, 101 management, 304
joint stiffness, 100 quadricepsplasty, 306
muscle weakness, 101 supracondylar osteotomy, 304, 305
non-union and delayed union, 100 Low-intensity pulsed ultrasound (LIPUS), 520–521
recurrent deformity, 100 Lyrica™, 189
vascular, 100
Lateral distal femur angle (LDFA), 39
Lateral distal tibial angle (LDTA), 56, 58 M
Legg–Calve–Perthes disease, 190–191, 550–551 Maffucci’s syndrome, 338
Leg length discrepancy (LLD), 195 Magnetic resonance imaging (MRI)
Lengthening and Then Nailing (LATN), 127, 128 CFT, 367
Lengthening and Then Plating (LAP), 125, 126 CPT, 485
Lengthening osteotomy, 83–84 physeal arrest, extent of, 72
Lengthening over Nail (LON) technique, 121–123, 236, 238, 440–442 posttraumatic deformities, 571
Limb lengthening Malignant tumors, 4, 6
anisomelia Malunited intra-articular fractures, 579
complications, 61, 65 Malunited shaft femur, 574, 576
pathologic, 60 Masquelet’s procedure, 488, 491
physiologic, 60 Maximum allowable blood loss (MABL), 219
screw placement, 61, 65 Mechanical axis deviation (MAD), 28, 29, 109
timing, 60–64 Medial proximal tibial angle (MPTA), 39
complication, 121 Metabolic disorders, 4
hybrid techniques clinical manifestations, 231
bone transport, 125–127 definition, 231
LAP, 125, 126 deformity analysis, 236, 240
LATN, 127, 128 Drill hole technique, 236, 239
lengthening and screw fixation, 127, 128 drug-induced rickets, 234
LON, 121–123 Gigli saw osteotomy technique, 236, 239
PAL, 123–124 interference screws, 239, 242
physical therapy (see Physical therapy) intramedullary static fixation, 236, 239, 242
prolonged treatment, 121 long sagittal bowing deformity, 236, 238
staples, 51 medulla, 239, 241
Limb reconstructive surgery, 419–420 monolateral external fixator, 236, 241
Liquid nitrogen (LN) method nutritional rickets, 233, 234
advantages, 357 orthopedic treatment
disadvantages, 357 anterolateral bowing, 234, 235
Index 629
intramedullary and plate fixation, 234, 236, 237 Myelomeningocele (spina bifida), 289
LON, 236, 238 ankle and foot deformity, 290
monolateral/circular external fixator, 236, 238 CP, 289
recurrent deformity, 234, 238 ankle and foot deformity, 290
tibial deformity, 234, 235 hip dysplasia, 290
pathophysiology, 231–232 knee flexion deformity and tibial torsion, 290
pediatric metabolic conditions, 231 hip dysplasia, 290
prematurity, 234 knee flexion deformity and tibial torsion, 290
renal osteodystrophy, 3
angular deformity, 243
avascular necrosis, 244 N
clinical manifestations, 241 Neurofibromatoses (NF), 4–5
features, 242 Neuromuscular disorders, 4
medical treatment, 243 CMT, 4–5
prevalence of, 241 CP (see cerebral palsy (CP))
rachitic changes, 242–243 Non-physeal fractures, 4, 12
SCFE, 243
serum vitamin D and calcium levels, 234, 243
rickets, 3, 4 O
transverse skin incision, 236, 241 Oblique osteotomy, 83
tumor-related hypophosphatemic rickets, 241 Ollier’s disease, 338
two parallel Schanz screws, 236, 240, 241 Opening wedge osteotomy, 81, 82
unilateral external fixator, 236, 239 Orthofix™ fixator technique, 403–404
varus bowing deformity, 236, 238 Osseous deformities, 363
vitamin D-resistant rickets Osteofibrous dysplasia, 337–338
biochemical abnormality, 233, 234 Osteogenesis imperfecta (OI), 109
distal tibial varus deformity, 233, 235 anesthesia, 256
medical treatment, 233–234 bone deformity(ies), 255, 256
severe genu valgum deformity, 233, 234 braces
severe genu varum deformity, 233, 234 AFOs, 262
Metaphyseal chondrodysplasia, 328–329 HKAFOs, 261
Metaphyseal-diaphyseal angle (MDA), 27, 28 KAFOs, 261–262
Modified McFarland bypass graft technique, 485–486 molding of, 262
Modular rail system technique, 404–405 SMOs, 262
Morquio syndrome, 324–325, 327, 328 coxa vara correction, 261–263
Morscher osteotomy difficult intubations, 256
deformity correction and fixation, 552–554 femoral rodding technique, 258–260
positioning and exposure, 552 fracture, 256
postoperative care, 554–556 hemorrhage, 256
Motorized intramedullary limb lengthening long-term immobilization, 260
complications of, 145–148 neurologic complications, 256
reverse planning method, 134–135 open/percutaneous osteotomy, 257–258
Shanz pins, 136, 137 patient positioning, 260–262
trochanteric entry, 136, 137 postoperative rehabilitation, 261
venting, canal, 135–136 rod types
visual aids, 135, 136 regular rods, 257
Multi-axial correcting (MACT) system, 112–113 sliding rods, 257, 258
Multiple bony deformities, 92–93 telescopic rods, 257
Multiple epiphyseal dysplasia (MED), 323, 324 soft tissue problems, 255–256
Multiple hereditary osteochondromatosis/exostoses (MHE), 341–343 surgical correction, 256–257
Multiple pterygium syndromes, 299 tibial rodding technique, 259–261
Muscle contractures tight muscles stretching, 261
analgesic medications, 185 Osteogenic progenitor stem cell culture technique, 524
CKD, 185–187 Osteoporosis, 231
femoral lengthening, 184 Osteotomy
myofascial tissues, 184 acute correction
passive stretching, 185 blade and locking plates, 44, 47
positioning and splinting strategies, 185, 186 cast immobilization, 42
prolonged stretch, 184 conventional plates, 44–45
removable thigh cuff, 186, 187 distal femoral valgus deformity, 44, 47
soft tissue mobilization, 184 implants determination, 42
tibial lengthening, 184 intramedullary fixation, 42, 44
tight pin wraps, 185 intramedullary stabilization, 42, 44, 46
walking shoe modification, 185, 186 Kirschner wire fixation, 42
Muscular dystrophy, 293 screw fixation, 44
Musculoskeletal infections, 4, 12 structural graft, 44, 46
630 Index
Osteotomy (cont.) Pelvic support osteotomy (PSO), 592–593

acute deformity correction Pemberton osteotomy, 538
acetabulum, 88 Percutaneous epiphysiodesis using transphyseal screws (PETS), 52
acute complications (see Acute complications) Periacetabular triple osteotomy (Paley PATO), 389–390
adjuvant procedures, 88–89 deformity correction and fixation, 541–543
angular correction and translation, 82–83 osteotomies, 540–542
angular deformity secondary, 84 positioning and exposure, 540, 541
blade plate, 94 postoperative care, 544
bone void fillers, 95 Peroneal nerve decompression, 396–397
closed wedge osteotomy, 81, 82 Perthes disease, 190–191
Cozen’s phenomenon, 84, 85 Physeal bar excision
deformity memory effect, 84, 85 abnormal physis, assessment of
diaphysis, 86, 87 growth remaining, 72
disease-specific indications, 89 partial vs. complete physeal arrest, 70
dome osteotomy, 81, 85 physeal arrest location, 70–71
epiphyseal, 85 timely identification, 70
external fixation, 94–95 anterior physeal bar, 75
FAP technique, 95–96 cannulated ACL reamer, 75
femoral deformity correction, 91–92 clinical outcome, 76
influence of, 81 etiology and prognosis
intramedullary devices, 94, 95 altered proximal humeral physeal growth, 69
juxta-apophyseal, 85, 87 Harris growth arrest lines, 67, 68
Kirschner wires, 93 infection, 68, 69
knee, 88 neonatal sepsis, 67, 68
late complications (see Late complications) neoplasm and tumorlike conditions, 68–69
LCP, 94 physeal arrest, 67
length discrepancy, 88, 89 repetitive stress, 69
lengthening osteotomy, 83–84 trauma, 67–69
limb reconstruction systems, 94–95 vascular insult, 69
mango slice effect, 84 fiber-optic lighted suction, dental mirrors and fluoroscopy, 75
metaphysis, 85, 86 interposition material, 74, 76
multiple bony deformities, 92–93 local fat harvest, 75
oblique osteotomy, 83 osteotomy role, 73, 75
opening wedge osteotomy, 81, 82 physeal arrest, extent of, 71–72
planning, governing rules, 89–90 radiographic markers, 76
proximal femur, 86–88 radio-opaque dye, 75
screws, 93 recurvatum deformity, 75
shortening osteotomy, 83–84, 89 surgical approaches
supracutaneous locked plating, 96, 99 bony bar, 73
tension band wiring, 93–94 central bars, 73, 74
tibial deformity correction, 90–91 peripheral bars, 73
disadvantage, 51 type C bars, 73, 74
fibrous dysplasia, 336, 337 surgical indications, 72–73
gradual deformity correction, 45, 48–49, 110, 112, 113 Physeal bar resection, 42
physeal bar excision, 73 advantages, 40
complete epiphysiodesis, 40
disadvantages, 40
P hemiepiphysiodesis, 40, 42
Paley classification, 201, 203, 372, 431–433 oblique plane deformity, 42, 44
Paley-Dega osteotomy, 388–389 proximal tibial valgus deformity, 42, 45
Pappas classification, 369 in right distal femur, 40, 43
Parathyroid hormone (PTH), 527 Physeal injury, 4, 11–12, 84, 577–578
Pediatric joint contractures ankle with malunion, 582
equinus contracture (see Equinus contracture) distal femur with angular deformity, 577–578
knee extension contractures proximal tibia, 579–580
clinical evaluation, 176 Physical therapy
etiology, 176 equinus contracture, 164
knee stiffness, 176 knee extension contractures, 176
nonoperative technique, 176 knee flexion contracture, 172
quadricepsplasty, 176–178 limb lengthening and reconstruction surgery
radiographs, 176 achondroplasia, 192–194
knee flexion contracture (see Knee flexion contracture) CFD, 191, 192
Pelvic osteotomies consolidation phase, 182, 183
Paley-Dega osteotomy, 388–389 fibular hemimelia, 191–192
Paley PATO, 389–390 gait considerations, 195–196
PAO, 390–391 handling techniques, 184
Index 631
inpatient phase, 181, 182 R

internal lengthening devices, 194–195 Reaming techniques
joint stiffness, 186 conventional reaming, 136
joint subluxation, 188 rigid reaming, 137–138
Legg–Calve Perthes disease, 190–191 Receptor activator of nuclear factor kappa-B ligand (RANKL)
lengthening/correction phase, 181–182 inhibitors, 336
muscle contractures (see Muscle contractures) Reimer’s migration percentage (MP), 270, 271
muscle weakness, 186–188 Renal osteodystrophy, 3
nerve injury, 189 clinical manifestations, 241
post frame removal, 182–183 features, 242
temporary soft tissue dysfunction, 183 medical treatment, 243
weight-bearing considerations, 189–190 orthopedic treatment
Physiologic external rotation contracture of infancy angular deformity, 243
(PERCI), 20 avascular necrosis, 244
Pixel value ratio (PVR), 520 SCFE, 243
Plate-assisted lengthening (PAL), 123–124 prevalence of, 241
Platelet-rich plasma (PRP), 522 rachitic changes, 242–243
Poliomyelitis, 290–291 serum vitamin D and calcium levels, 234, 243
Popliteal pterygium syndrome, 299, 308 Residual femoral head subluxation
Posteromedial tibial bowing, 9, 10 application, 564
Postinfectious deformities, 587 core decompression, 563
Choi type II and III hips, 589, 591 patient positioning, 562
Choi type IV deformity, treatment options for, 591–592 postoperative care, 564, 565
GTA vs. PSO, 592–593 soft tissue releases, 563–564
infantile septic arthritis, 587 stem cell injection, 563
knee and ankle joints Residual hip deformities, 4, 11
incongruity, 598–599 coxa breva, 551–552
septic arthritis and osteomyelitis, 593–598 coxa magna, 556–557
radiographic classifications, 587–589 developmental dysplasia, 536
treatment modalities, 589, 590 femoral head reduction osteotomy
Posttraumatic chondrolysis, 573–575 deformity correction and fixation, 558–560
Posttraumatic deformities, 569 positioning and exposure, 557–558
bony deformities remodeling, 571–573 postoperative care, 560, 561
causation and etiological factors, 569–570 femoral neck osteoplasty, 549–550
classification, 571 percutaneous osteotomy and external fixation, 550
clinical manifestations, 571 postoperative care, 550
distal femoral deformity, 576–577 hip distraction treatment
investigations, 571 application, 564
lower limb, 573 core decompression, 563
malunited intra-articular fractures, 579 patient positioning, 562
malunited shaft femur/pseudarthrosis with deformity, 574, 576 postoperative care, 564, 565
physeal injury (see Physeal injury) soft tissue releases, 563–564
posttraumatic chondrolysis and avascular necrosis, 573–575 stem cell injection, 563
posttraumatic tibia valga, 580–582 Legg–Calve–Perthes disease, 550–551
soft-tissue loss with skeletal injury, 582–584 modified Dunn procedure
Posttraumatic tibia valga, 580–582 deformity correction and fixation, 545–546
PRECICE, 133, 134, 148, 440 positioning and exposure, 545
Pressure specified sensory device (PSSD), 189 postoperative care, 546–547
Prone exam surgical hip dislocation, 545
axial foot deformities, 21–22 modified Southwick osteotomy, 548, 549
femoral version and antetorsion, 19–21 deformity correction and fixation, 548
tibial torsion, 21 positioning and exposure, 548
Prosthetic management Morscher osteotomy
children, 210–212 deformity correction and fixation, 552–554
end weight-bearing, 213 positioning and exposure, 552
first prosthesis, 210 postoperative care, 554–556
future developments, 213 periacetabular triple osteotomy
residual limb length, 211–212 deformity correction and fixation, 541–543
residual limb overgrowth, 212–213 osteotomies, 540–542
Prosthetic reconstructive surgery, 420–422 positioning and exposure, 540, 541
Proximal focal femoral deficiency (PFFD), 20 postoperative care, 544
Pulsed electromagnetic field (PEMF), 521 residual femoral head subluxation, 562
SCFE, 544
treatment
Q patients after closure, 538–539
Quadricepsplasty, 176–178 patients <6 months of age, 536
632 Index
Residual hip deformities (cont.) limb lengthening, 317–318

patients >2 years until closure, 537–538 MED, 323, 324
patients >6 months to 2 years, 536–537 metaphyseal chondrodysplasia, 328–329
Resource-challenged environments Morquio syndrome, 324–325, 327, 328
Clubfoot, 225–226 postoperative considerations, 318
educational resources, 227–228 SEDC, 319, 321–323
international collaboration surgical considerations
credentialing rules, 217–218 anesthesia, 315–316
good working relationship, 217 imaging, 315
hosting, 216, 217 implant size and design, 316
medicolegal issues, 218 patient positioning, 316
motivation, 217 preoperative evaluation, 315
program commitment, 216 systemic abnormalities, 313, 314
volunteers, 216–217 Skeletal injury, 582–584
operative challenges Slipped capital femoral epiphysis (SCFE), 3, 20, 22, 243, 544
Cidex®, 221 Soft tissue surgery, 158, 173
cordless power, 223–224 Southwick osteotomy, 548, 549
ethylene oxide, 221 deformity correction and fixation, 548
indications, 218–219 positioning and exposure, 548
internal vs. external fixation, 222 Spica Cast, 227
operating room set up, 221–222 Split tibialis anterior tendon transfer (SPLATT), 288
orthopedics, 224–225 Split tibialis posterior tendon transfer (SPOTT), 288
perioperative and anesthetic considerations, 219–221 Spondyloepiphyseal dysplasia congenital (SEDC), 319, 321–323
postoperative care, 224 Stanitski classification, 429, 430
reusing implants and external fixation components, 222–223 Superhip surgical technique
sterility, 221 abductor muscle slide, 385
surgical modus operandi, 222 arthrogram, 386
population pyramids, 215, 216 blade plate insertion, 386
prevalence, 215 BMP insertion, 387
severely progressive disparity, 215, 216 closure, 387–388
SIGN nail, 226–227 distal femur fixation, 387
Spica Cast, 227 external rotation contracture release, 385
Retrograde femoral lengthening fascia lata release, 384–385
patient indications, 141–142 femoral shortening, 386–387
planning, 142 flap elevation, 384
surgical technique, 142–143 guide wire insertion, 386
Reverse planning method, 134–135, 142 hip flexion contracture releases, 385
Rickets, 3, 4 iliac wing osteotomy, 387
Ru Li classification, 520 incision, 384
muscle repairs and transfers, 387
pelvic osteotomy, 387
S periosteum release, 386
Salter-Harris type II, III, and IV injuries, 67, 69 positioning, prepping, and draping, 378–384
San Diegoplasty osteotomy, 538 postoperative course, 388
Scoliosis, 308 quadriceps elevation, 386
SEDC. See Spondyloepiphyseal dysplasia congenital (SEDC) subtrochanteric osteotomy, 386
Shanz pins, 136, 137 Superknee surgical technique, 392–394
Shortening osteotomy, 83–84, 89 Supracutaneous locked plating, 96, 99
SIGN. See Surgical Implant Generation Network (SIGN) nail Supratrochanteric torsion (STT), 26
Single-event multilevel orthopedic surgery (SEMLS), 282 Surgical Implant Generation Network (SIGN) nail, 226–227
Skeletal dysplasia, 4, 6
achondroplasia, 319–321
characteristic, 313, 314 T
deformity correction Taylor Spatial Frame™, 169, 174, 175
acute methods, 316–317 Taylor spatial frame (TSF), 605
with external fixation, 317 Tibia bowing, 9, 10
with guided growth, 317 Tibial deformity correction, 90–91
diastrophic dysplasia Tibial hemimelia, 455
with hitch hiker thumbs, 323, 325 genetics, 455–456
laterally dislocated patellae, 323, 325 horizons and strategy, 461–462
operative correction, 324, 326 Jones classification, 456, 457
rigid clubfeet and hallux varus, 323, 325 limb reconstruction surgery, 463
rigid equinovarus foot, 324, 326 Paley classification, 457, 458, 462
supramalleolar osteotomies, 324, 326 centralization, fibula, 477, 479
EvC syndrome, 329–330 synostosis and ankle fusion, 473
genetic mutation and mode of inheritance, 313, 314 type 1, 463–464
Index 633
type 2, 464–467 Trans-iliac osteotomy, 88

type 3a, 467 Transmalleolar axis (TMA), 21
type 4a, 467–469 Transphyseal ACL reconstruction, 606–608
type 5a, 473, 474 Trevor’s disease. See Dysplasia epiphysealis hemimelica
type 3b, 467 Triphalangeal thumb-polysyndactyly syndrome (TPTPS), 455
type 4b, 469–472
type 5bi, 476
type 5bii, 476–477, 478 V
type 4c, 472–473 Valgus ankle deformity, 608–612
pathoanatomy, 456 Vascularized fibula graft nonunion, 608–612
treatment history Vascular malformations, 4, 7
amputation, 460 Visual aids, 135
Brown procedure, 459–460 Vitamin D-resistant rickets
distal tibia and ankle stabilization, 460–461 biochemical abnormality, 233, 234
early treatments, 458–459 distal tibial varus deformity, 233, 235
limb lengthening, 461 medical treatment, 233–234
options, 461 severe genu valgum deformity, 233, 234
tibiofibular synostosis, 460 severe genu varum deformity, 233, 234
Weber patelloplasty, 461
Weber patellar arthroplasty, 473–476
Tibial hemimelia-polysyndactyly-triphalangeal thumb syndrome, 455 W
Tibialis posterior tendon lengthening, 288 Weber patelloplasty, 461
Tibial lengthening Wingless-type (Wnt) signaling pathway, 362
patient indications, 143 Wolff’s law, 79, 497
planning, 143 World Health Organization has created a Surgical Safety
surgical technique, 144 Checklist, 220
Tibial metaphyseal-diaphyseal angle (TMDA), 27
Tibia malunion, 603–605
Tibia vara, 10, 11 X
TMA. See Transmalleolar axis (TMA) X-rays, 571
TMDA. See Tibial metaphyseal-diaphyseal angle (TMDA)
Torode classification, 369–371
TP510, 524, 526 Z
TPTPS. See Triphalangeal thumb-polysyndactyly syndrome (TPTPS) Zone of polarizing activity (ZPA), 361

Pediatric Lower Limb Deformities 2016

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Pediatric Lower Limb Deformities 2016

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Sanjeev Sabharwal

Principles and Techniques

Pediatric Lower Limb

ISBN 978-3-319-17096-1 ISBN 978-3-319-17097-8 (eBook)

Library of Congress Control Number: 2015954086

Springer Cham Heidelberg New York Dordrecht London

Printed on acid-free paper

Springer International Publishing AG Switzerland is part of Springer Science+Business Media (www.springer.com)

Orange, CA, USA Stuart A. Green, MD

Newark, NJ, USA Sanjeev Sabharwal, MD, MPH

Part I General Principles and Techniques

1 Etiology of Lower Limb Deformity ........................................................................ 3

Part II Related Concepts and Management Options

10 Biomechanically Based Clinical Decision Making in Pediatric Foot

14 Working in Resource-Challenged Environments .................................................. 215

Part III Underlying Conditions

15 Metabolic Disorders ................................................................................................. 231

Part IV Congenital and Developmental Disorders

22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery ....... 361

Part V Sequelae and Complications

28 Methods to Enhance Bone Formation in Distraction Osteogenesis .................... 519

30 Posttraumatic Lower Limb Deformities in Children............................................ 569

Index .................................................................................................................................. 621

Corinna C. Franklin, MD Shriners Hospital for Children, Philadelphia, PA, USA

Hiroyuki Tsuchiya, MD, PD Department of Orthopedic Surgery, Kanazawa University

slipped capital femoral epiphysis (SCFE). These same disor-

S. Sabharwal (ed.), Pediatric Lower Limb Deformities, 3

Table 1.1 General categorization of the types of lower limb deformi-

Fig. 1.3 A 17-year-old male patient with Charcot-Marie-Tooth dis-

Fig. 1.2 A 3-year-old female patient with neuroﬁbromatosis type 1

and peroneus brevis) muscle weakness and imbalance. The

Arthrogryposis Multiplex Congenita

Arthrogryposis multiplex congenita (multiple congenital

Table 1.4 Common overgrowth and undergrowth syndromes

Various generalized and local vascular malformations are

Hemihypertrophy and Hemiatrophy

There are no clear guidelines in literature as to what consti-

Fig. 1.6 (a, b) An 18-month-old

Congenital Bowing of the Tibia

Anterolateral Bowing of the Tibia

Fig. 1.8 (a, b) A 9-year-old male

Tibia Vara/Blount’s Disease

Table 1.5 Common causes of genu varum

Table 1.6 Common causes of genu valgum

Acknowledgements The authors would like to thank Dr. Hitesh Shah

Fractures do not necessarily have to directly involve the

Do the parents or grandparents want to “do something

S. Sabharwal (ed.), Pediatric Lower Limb Deformities, 15

Fig. 2.1 The development of the

Winter’s Pediatric orthopedics,

The examiner should watch the child walk. Sometimes children

Spine and Trunk Foot Progression Angle

Fig. 2.2 The foot progression

Short Leg Gait Table 2.2 Etiologies of lower extremity deformity

and adolescents should be asked to report what amount of

Femoral Version and Antetorsion

Fig. 2.5 Assessment of hip

epiphysis (SCFE), coxa vara, and proximal focal femoral

Fig. 2.7 A 30° valgus deformity

The TFA averages 5° internal (range 30° to +20°) in

out disorders such as hip dysplasia and SCFE in a child

Assessment of Limb Length

Several techniques have been developed for the measurement

Table 2.3 Comparison of methods for assessing leg length discrepancya