Professional Documents
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Pediatric Lower Limb Deformities 2016
Pediatric Lower Limb Deformities 2016
Editor
Pediatric Lower
Limb Deformities
123
Pediatric Lower Limb Deformities
Sanjeev Sabharwal
Editor
“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!
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.
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
xiii
xiv Contents
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
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
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
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.
Vascular Malformations
Congenital
(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].
1 Etiology of Lower Limb Deformity 9
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.
18. Birch JG. Blount disease. J Am Acad Orthop Surg. 2013;21:408–18. 31. Crawford AH, Schorry EK. Neurofibromatosis in children: the role
19. Hoyme HE, Seaver LH, Jones KL, Procopio F, Crooks W, Feingold of the orthopaedist. J Am Acad Orthop Surg. 1999;7:217–30.
M. Isolated hemihyperplasia (hemihypertrophy): report of a pro- 32. Holt JF. 1977 Edward B. D. Neuhauser lecture: neurofibromatosis
spective multicenter study of the incidence of neoplasia and review. in children. Am J Roentgenol. 1978;130:615–39.
Am J Med Genet. 1998;79:274–8. 33. Hofmann A, Wenger DR. Posteromedial bowing of the tibia.
20. Clericuzio CL, Martin RA. Diagnostic criteria and tumor screening Progression of discrepancy in leg lengths. J Bone Joint Surg Am.
for individuals with isolated hemihyperplasia. Genet Med. 2009; 1981;63:384–8.
11:220–2. 34. Kocher MS, Saxon HS, Hovis WD, Hawkins RJ. Management and
21. Shuman C, Smith AC, Steele L, Ray PN, Clericuzio C, Zackai E, complications of anterior cruciate ligament injuries in skeletally
et al. Constitutional UPD for chromosome 11p15 in individuals immature patients: survey of the Herodicus Society and The ACL
with isolated hemihyperplasia is associated with high tumor risk Study Group. J Pediatr Orthop. 2002;22:452–7.
and occurs following assisted reproductive technologies. Am J Med 35. Jackson DW, Cozen L. Genu valgum as a complication of proximal
Genet A. 2006;140:1497–503. tibial metaphyseal fractures in children. J Bone Joint Surg Am.
22. Wiedemann HR. Familial malformation complex with umbilical 1971;53:1571–8.
hernia and macroglossia – a “New Syndrome”? J Genet Hum. 36. Enjolras O, Chapot R, Merland JJ. Vascular anomalies and the
1964;13:223–32. growth of limbs: a review. J Pediatr Orthop B. 2004;13:349–57.
23. Beckwith JB. Macroglossia, omphalocele, adrenal cytomegaly, 37. Cook SD, Lavernia CJ, Burke SW, Skinner HB, Haddad Jr RJ. A
gigantism, and hyperplastic visceromegaly. Birth Def. 1969;5:188. biomechanical analysis of the etiology of tibia vara. J Pediatr
24. Elliott M, Maher ER. Beckwith-Wiedemann syndrome. J Med Orthop. 1983;3:449–54.
Genet. 1994;31:560–4. 38. Salenius P, Vankka E. The development of the tibiofemoral angle in
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and natural history of Beckwith-Wiedemann syndrome: presenta- 39. Violas P, Salmeron F, Chapuis M, Sales de Gauzy J, Bracq H,
tion of 74 new cases. Clin Genet. 1994;46:168–74. Cahuzac JP. Simple bone cysts of the proximal humerus compli-
26. Schoenecker PL, Capelli AM, Millar EA, Sheen MR, Haher T, cated with growth arrest. Acta Orthop Belg. 2004;70:166–70.
Aiona MD, et al. Congenital longitudinal deficiency of the tibia. 40. Bell SN, Campbell PE, Cole WG, Menelaus MB. Tibia vara caused
J Bone Joint Surg Am. 1989;71:278–87. by focal fibrocartilaginous dysplasia. Three case reports. J Bone
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Clinical Evaluation Including Imaging
2
Joseph 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 ).
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
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
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.
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
2 Clinical Evaluation Including Imaging 25
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
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
2 Clinical Evaluation Including Imaging 31
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
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
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.
suspected lower extremity deformity, the clinician must first
9. Jonson SR, Gross MT. Intraexaminer reliability, interexaminer reli-
determine if there is true deformity or simply normal pediat- ability, and mean values for nine lower extremity skeletal measures
ric limb development. The latter needs no treatment but can in healthy naval midshipmen. J Orthop Sports Phys Ther. 1997;
safely be observed for natural resolution while giving reas- 25(4):253–63.
10. Woerman AL, Binder-Macleod SA. Leg length discrepancy assess-
surance to the family. This determination can often be
ment: accuracy and precision in five clinical methods of evalua-
performed by a history and physical examination. If the com- tion*. J Orthop Sports Phys Ther. 1984;5(5):230–9.
ponents of the history and physical are consistent with the 11. Birch JG. Blount disease. J Am Acad Orthop Surg. 2013;
diagnosis of normal childhood development, imaging studies 21(7):408–18.
are not necessary. When imaging studies are deemed to be 12. Cheema JI, Grissom LE, Harcke HT. Radiographic characteristics
of lower-extremity bowing in children. Radiographics. 2003;
necessary, they must be performed using techniques which 23(4):871–80.
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14. Salenius P, Vankka E. The development of the tibiofemoral angle in
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16. Kling Jr TF, Hensinger RN. Angular and torsional deformities of the
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Decision Making in Lower Extremity
Deformity Correction 3
Mark L. Miller and J. Eric 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.
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).
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. 3.6 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
3 Decision Making in Lower Extremity Deformity Correction 45
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
48 M.L. Miller and J.E. Gordon
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).
3 Decision Making in Lower Extremity Deformity Correction 49
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
4. Gabriel KR, Crawford AH, Roy DR, True MS, Sauntry S. 13. Feldman DS, Madan SS, Ruchelsman DE, Sala DA, Lehman
Percutaneous epiphysiodesis. J Pediatr Orthop. 1994;14(3): WB. Accuracy of correction of tibia vara: acute versus gradual cor-
358–62. rection. J Pediatr Orthop. 2006;26(6):794–8.
5. Skytta ET, Savolainen HA, Kautiainen HJ, Belt EA. Long-term 14. Feldman DS, Madan SS, Koval KJ, van Bosse HJ, Bazzi J, Lehman
results of leg length discrepancy treated with temporary epiphyseal WB. Correction of tibia vara with six-axis deformity analysis and
stapling in children with juvenile chronic arthritis. Clin Exp the Taylor Spatial Frame. J Pediatr Orthop. 2003;23(3):387–91.
Rheumatol. 2003;21(5):669–71. 15. Feldman DS, Shin SS, Madan S, Koval KJ. Correction of tibial mal-
6. Mielke CH, Stevens PM. Hemiepiphyseal stapling for knee defor- union and nonunion with six-axis analysis deformity correction using
mities in children younger than 10 years: a preliminary report. the Taylor Spatial Frame. J Orthop Trauma. 2003;17(8):549–54.
J Pediatr Orthop. 1996;16(4):423–9. 16. Gordon JE, Jani M, Dobbs M, Luhmann SJ, Szymanski DA,
7. Stevens PM, Klatt JB. Guided growth for pathological physes: Schoenecker PL. Treatment of rigid hypertrophic posttraumatic
radiographic improvement during realignment. J Pediatr Orthop. pseudarthrosis of the tibia in children using distraction osteogene-
2008;28(6):632–9. sis. J Pediatr Orthop. 2002;22(4):419–23.
8. Chapman MW, Dwellius PW, Bray TJ, Gordon JE. Closed intra- 17. Camathias C, Valderrabano V, Oberli H. Routine pin tract care in
medullary femoral osteotomy: shortening and derotation proce- external fixation is unnecessary: a randomised, prospective, blinded
dures. Clin Orthop. 1993;287:245–51. controlled study. Injury. 2012;43(11):1969–73.
9. Gordon JE, Pappademos PC, Schoenecker PL, Dobbs MB, 18. Dayton P, Prins DB, Hensley N, Wienke Jr J. A user-friendly
Luhmann SJ. Diaphyseal derotational osteotomy with intramedul- method of pin site management for external fixators. Foot Ankle
lary fixation for correction of excessive femoral anteversion in chil- Spec. 2011;4(6):370–2.
dren. J Pediatr Orthop. 2005;25:548. 19. Gordon JE, Kelly-Hahn J, Carpenter CJ, Schoenecker PL. Pin site
10. Schoenecker PL, Johnston R, Rich MM, Capelli AM. Elevation of care during external fixation in children: results of a nihilistic
the medical plateau of the tibia in the treatment of Blount disease. J approach. J Pediatr Orthop. 2000;20(2):163–5.
Bone Joint Surg Am. 1992;74(3):351–8. 20. Jennison T, McNally M, Pandit H. Prevention of infection in exter-
11. Gordon JE, Heidenreich FP, Carpenter CJ, Kelly-Hahn J, nal fixator pin sites. Acta Biomater. 2014;10(2):595–603.
Schoenecker PL. Comprehensive treatment of late-onset tibia vara. 21. Lee CK, Chua YP, Saw A. Antimicrobial gauze as a dressing
J Bone Joint Surg Am. 2005;87(7):1561–70. reduces pin site infection: a randomized controlled trial. Clin
12. Schiedel FM, Vogt B, Tretow HL, Schuhknecht B, Gosheger G, Orthop Rel Res. 2012;470(2):610–5.
Horter MJ, et al. How precise is the PRECICE compared to the 22. Park SS, Gordon JE, Luhmann SJ, Dobbs MB, Schoenecker
ISKD in intramedullary limb lengthening? Acta Orthop. 2014;85(3): PL. Outcome of hemiepiphyseal stapling for late-onset tibia vara. J
293–8. Bone Joint Surg Am. 2005;87(10):2259–66.
Growth Modulation for Angular
and Length Correction 4
Peter M. Stevens
General
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
Technique/Angular
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
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.
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
4 Growth Modulation for Angular and Length Correction 59
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
Pathologic
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
4 Growth Modulation for Angular and Length Correction 61
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
4 Growth Modulation for Angular and Length Correction 63
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-
J Bone Joint Surg Am. 1977;59(2):174–9. rior radiographs replace the scanogram for measurement of limb
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.
alternative to current methods. J Pediatr Orthop. 2013;33(5): Orthopedics. 1989;12(11):1457–63.
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
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. 5.2 (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
Vascular Insult
Timely Identification
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
72 K.E. Rathjen and A.I. Riccio
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
5 Physeal Bar Excision 73
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)
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
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
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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
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
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
84 V. Madhuri and S. Gangadharan
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).
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.
Indications
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).
86 V. Madhuri and S. Gangadharan
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
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.
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.
90 V. Madhuri and S. Gangadharan
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.
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
6 Acute Deformity Correction Using an Osteotomy 91
(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
92 V. Madhuri and S. Gangadharan
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).
6 Acute Deformity Correction Using an Osteotomy 93
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
Blade Plate
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 intraopera- tive 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.
6 Acute Deformity Correction Using an Osteotomy 97
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.
98 V. Madhuri and S. Gangadharan
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.
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
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]
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
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
7 Gradual Deformity Correction 109
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
110 D.S. Feldman et al.
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
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,
112 D.S. Feldman et al.
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
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).
114 D.S. Feldman et al.
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.
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
116 D.S. Feldman et al.
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
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
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
7 Gradual Deformity Correction 119
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Fig. 7.17 Syndesmotic screw through distal tibia and fibula to prevent in distraction osteogenesis. Ortop Traumatol Rehabil. 2013;15(6):
fibular migration during deformity correction. Radiographic view, syn- 591–9.
desmotic screw 22. Watanabe K, Tsuchiya H, Sakurakichi K, Yamashiro T, Matsubara
H, Tomita K. Treatment of lower limb deformities and limb-length
discrepancies with the external fixator in Ollier’s disease. J Orthop
Sci. 2007;12(5):471–5.
23. Nogami H, Ono Y, Katoh R, Oohira A. Microvascular and cellular
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1. Feldman DS, Madan SS, Ruchelsman DE, Sala DA, Lehman WB. 24. Coogan PG, Fox JA, Fitch RD. Treatment of adolescent Blount dis-
Accuracy of correction of tibia vara: acute versus gradual correc- ease with the circular external fixation device and distraction osteo-
tion. J Pediatr Orthop. 2006;26(6):794–8. genesis. J Pediatr Orthop. 1996;16(4):450–4.
2. Feldman DS, Madan SS, Koval KJ, van Bosse HJ, Bazzi J, Lehman 25. Solomin LN, Paley D, Shchepkina EA, Vilensky VA, Skomoroshko
WB. Correction of tibia vara with six-axis deformity analysis and PV. A comparative study of the correction of femoral deformity
the Taylor Spatial Frame. J Pediatr Orthop. 2003;23(3):387–91. between the Ilizarov apparatus and Ortho-SUV Frame. Int Orthop.
3. Codivilla A. On the means of lengthening, in the lower limbs, the 2014;38(4):865–72.
muscles and tissues which are shortened through deformity. Am J 26. Paley D. The correction of complex foot deformities using Ilizarov’s
Orthop Surg. 1905;22(4):353–69. distraction osteotomies. Clin Orthop Relat Res. 1993;293:97–111.
4. Ozerdem OR, Kivanc O, Tuncer I, Acarturk S, Gocenler L, 27. Paley D, Lamm BM. Correction of the cavus foot using external
Gumurdulu D. Callotasis in nonvascularized periosteal bone grafts fixation. Foot Ankle Clin. 2004;9(3):611–24.
and the role of periosteum: a new contribution to the concept of 28. McCarthy JJ, Ranade A, Davidson RS. Pediatric deformity correc-
distraction osteogenesis. Ann Plast Surg. 1998;41(2):148–55. tion using a multiaxial correction fixator. Clin Orthop Relat Res.
5. Reina-Romo E, Gomez-Benito MJ, Garcia-Aznar JM, Dominguez 2008;466(12):3011–7.
J, Doblare M. Modeling distraction osteogenesis: analysis of the 29. Clarke SE, McCarthy JJ, Davidson RS. Treatment of Blount dis-
distraction rate. Biomech Model Mechanobiol. 2009;8(4):323–35. ease: a comparison between the multiaxial correction system and
6. Ilizarov GA. The tension-stress effect on the genesis and growth of other external fixators. J Pediatr Orthop. 2009;29(2):103–9.
tissues: Part II. The influence of the rate and frequency of distrac- 30. Pandya NK, Clarke SE, McCarthy JJ, Horn BD, Hosalkar
tion. Clin Orthop Relat Res. 1989;239:263–85. HS. Correction of Blount’s disease by a multi-axial external fixa-
7. Al Ruhaimi KA. Comparison of different distraction rates in the tion system. J Child Orthop. 2009;3(4):291–9.
mandible: an experimental investigation. Int J Oral Maxillofac 31. Iobst CA, Dahl MT. Limb lengthening with submuscular plate sta-
Surg. 2001;30(3):220–7. bilization: a case series and description of the technique. J Pediatr
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tissues. Part I. The influence of stability of fixation and soft-tissue 32. Baumgart R. The reverse planning method for lengthening of the
preservation. Clin Orthop Relat Res. 1989;238:249–81. lower limb using a straight intramedullary nail with or without
9. Aronson J, Good B, Stewart C, Harrison B, Harp J. Preliminary deformity correction. A new method. Oper Orthop Traumatol.
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10. Aronson J. the biology of distraction osteogenesis. In: Bianchi- deformity correction in children and young adults using Taylor
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Assoc. 2003;12(2):46–54. 2006;15(6):387–95.
Hybrid Techniques for Limb Length
and Deformity Correction 8
Mark T. Dahl and Chang-Wug 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
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
124 M.T. Dahl and C.-W. Oh
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
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
126 M.T. Dahl and C.-W. Oh
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 con- struct, 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.
8 Hybrid Techniques for Limb Length and Deformity Correction 127
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
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
8 Hybrid Techniques for Limb Length and Deformity Correction 129
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].
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
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.
9 Motorized Intramedullary Lengthening, an Emerging Technology for Limb Length and Deformity Correction 137
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.
Patient Indications
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
9 Motorized Intramedullary Lengthening, an Emerging Technology for Limb Length and Deformity Correction 139
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
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
9 Motorized Intramedullary Lengthening, an Emerging Technology for Limb Length and Deformity Correction 143
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
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
Summary
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
Mead NJ). 2008;37(9):E150–4.
nal fixators [38]. Reports of mechanical device failure such
5. Dahl MT, Gulli B, Berg T. Complications of limb lengthening.
as nail breakage of the FITBONE are rare (4 in 2,000 cases) A learning curve. Clin Orthop Relat Res. 1994;301:10–8.
(Roman Stauch, Intens, personal communication). Nail 6. Burghardt RD, Paley D, Specht SC, Herzenberg JE. The effect on
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medullary telescopic nail. J Bone Joint Surg Br.
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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
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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
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. 10.3 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
Fig. 10.6 Plain radiographs of the foot in a child with equinoplanoval- gus 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
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)
primarily on stress radiographs, which requires the use of a 3. Perry J. Gait analysis: normal and pathological function. Thorofare,
NJ: Slack Inc.; 1992.
“foot pusher” device to obtain simulated weight bearing 4. Davids JR. Orthopaedic treatment of foot deformities. In: Gage J,
views of the foot (see Fig. 10.9). Postoperative assessment, Schwartz M, Koop S, Novacheck T, editors. The identification and
once recovery, healing, and rehabilitation following surgery treatment of gait problems in cerebral palsy. 2nd ed. London:
have been completed, should be as quantitative as possible, MacKeith Press; 2009. p. 514–33.
5. Inman VT. The human foot. Manit Med Rev. 1966;46(8):513–5.
mirroring the preoperative assessment to allow objective 6. Inman VT. The influence of the foot-ankle complex on the proximal
assessment of outcome in multiple domains. Finally, surveil- skeletal structures. Artif Limbs. 1969;13(1):59–65.
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.
been identified. 8. Ponseti IV, El-Khoury GY, Ippolito E, Weinstein SL. A radio-
graphic study of skeletal deformities in treated clubfeet. Clin
Box 10.4 Orthop. 1981;160:30–42.
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
range of data by the use of a diagnostic matrix, which after surgical treatment for contracture of the triceps surae: a com-
considers and integrates data from five domains. puter simulation study. J Orthop Res. 1995;13(1):96–104.
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
surgically than uncoupled segmental malalignments. and biomechanical study in human cadavers. J Bone Joint Surg Am.
• Different types of ankle and foot deformities are 2013;95(16):1489–96.
best evaluated with different combinations of 12. Davids JR. The foot and ankle in cerebral palsy. Orthop Clin North
Am. 2010;41(4):579–93.
assessment tools at different points in the course of 13. Mosca VS. The child’s foot: principles of management. J Pediatr
management Orthop. 1998;18(3):281–2.
14. Kadhim M, Holmes Jr L, Miller F. Long-term outcome of planoval-
gus foot surgical correction in children with cerebral palsy. J Foot
Ankle Surg. 2013;52(6):697–703.
15. Shore BJ, Smith KR, Riazi A, Symons SB, Khot A, Graham
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ified technique in the setting of single event multilevel surgery.
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and quantitative analysis. In: Drennan J, McCarthy J, editors. 16. Astion DJ, Deland JT, Otis JC, Kenneally S. Motion of the hindfoot
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Lippincott Williams and Wilkins; 2009. p. 54–63. 241–6.
2. Inman VT, Ralston HJ, Todd F. Human walking. Baltimore, MD: 17. Barnes MJ, Herring JA. Combined split anterior tibial-tendon
Williams & Wilkins; 1981. transfer and intramuscular lengthening of the posterior tibial ten-
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don. Results in patients who have a varus deformity of the foot due with hemiplegic cerebral palsy. J Pediatr Orthop. 2008;28(3):
to spastic cerebral palsy. J Bone Joint Surg Am. 1991;73(5): 359–65.
734–8. 27. Stevens PM. Effect of ankle valgus on radiographic appearance of
18. Hoffer MM, Barakat G, Koffman M. 10-year follow-up of split the hindfoot. J Pediatr Orthop. 1988;8(2):184–6.
anterior tibial tendon transfer in cerebral palsied patients with spas- 28. Davis RB, Jameson EG, Davids JR, Christopher LM, Rogozinski
tic equinovarus deformity. J Pediatr Orthop. 1985;5(4):432–4. BM, Anderson JP. The design, development, and initial evaluation
19. Scott AC, Scarborough N. The use of dynamic EMG in predicting of a multisegment foot model for routine clinical gait analysis. In:
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hemiplegia. J Pediatr Orthop. 2006;26(6):777–80. sis: clinical treatment and technology. Boca Raton, FL: CRC Press;
20. Davids JR, McBrayer D, Blackhurst DW. Juvenile hallux valgus 2007. p. 425–44.
deformity: surgical management by lateral hemiepiphyseodesis of 29. Davis RBOS, Tyburski D, Gage JR. A gait analysis data collection
the great toe metatarsal. J Pediatr Orthop. 2007;27(7):826–30. and reduction technique. Hum Mov Sci. 1991;10:575–87.
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BL. Surgical management of ankle valgus in children: use of a tromyography as an aid in planning tendon transfers in children
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3–8. 31. Sutherland DH. Varus foot in cerebral palsy: an overview. Instr
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Pediatr Orthop. 1997;17(1):9–12. 32. Jameson EG, Davids JR, Anderson JP, Davis 3rd RB, Blackhurst
23. Mosca VS. Calcaneal lengthening for valgus deformity of the hind- DW, Christopher LM. Dynamic pedobarography for children: use
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26. Westberry DE, Davids JR, Roush TF, Pugh LI. Qualitative versus in children: the relationship between static alignment and dynamic
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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
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. 11.4 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
11 Pediatric Joint Contractures 167
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
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.
11 Pediatric Joint Contractures 171
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 sur- ing 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
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
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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.
long-term outcomes following a modified Thompson’s quadri- 45. Wang JH, Zhao JZ, He YH. A new treatment strategy for severe
arthrofibrosis of the knee. J Bone Joint Surg. 2006;88:1245–50.
Physical Therapy During Limb
Lengthening and Deformity 12
Correction: Principles and Techniques
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
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.
Muscle Contractures
Joint Stiffness
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.
Weight-Bearing Considerations
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
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.
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.
12 Physical Therapy During Limb Lengthening and Deformity Correction: Principles and Techniques 193
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
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. 13.5 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
13 Amputation and Prosthetic Management: Amputation as a Reconstructive Option 203
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
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
Tumor Reconstruction
Prosthetic Considerations
First Prosthesis
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
13 Amputation and Prosthetic Management: Amputation as a Reconstructive Option 211
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).
13 Amputation and Prosthetic Management: Amputation as a Reconstructive Option 213
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Working in Resource-Challenged
Environments 14
Scott C. Nelson and Hugh G. Watts
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).
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.
14 Working in Resource-Challenged Environments 219
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. 14.3 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
14 Working in Resource-Challenged Environments 221
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
14 Working in Resource-Challenged Environments 223
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.
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
14 Working in Resource-Challenged Environments 225
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
226 S.C. Nelson and H.G. Watts
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
14 Working in Resource-Challenged Environments 227
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 sta- in 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
228 S.C. Nelson and H.G. Watts
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
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.
15 Metabolic Disorders 235
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
236 M. Kocaoglu et al.
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. 15.14 The deformity has been acutely corrected with use of an
unilateral external fixator
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).
240 M. Kocaoglu et al.
Fig. 15.19 Two parallel Schanz screws distally at the femur Fig. 15.20 Two parallel Schanz screws proximally at the femur
15 Metabolic Disorders 241
Fig. 15.21 Schanz screws must leave enough space for intramedullary
nailing
Fig. 15.23 Transverse skin incision over the ligamentum patella
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].
242 M. Kocaoglu et al.
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.
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
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. 15.33 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. 15.37 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
248 M. Kocaoglu et al.
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
15 Metabolic Disorders 249
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. 15.53 Orthoroentgenogram of a patient with X-linked rickets Fig. 15.55 Orthoroentgenogram of the same patient after 3 months
prior to treatment later
24. Eralp L, Kocaoglu M, Cakmak M, Ozden VE. A correction of 28. Bardin T. Renal osteodystrophy, disorders of vitamin D metabo-
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
Orthop. 1992;12:291–9. alkaline phosphatase proteins associated with neonatal hypophos-
26. Stanitski DF. Treatment of deformity secondary to metabolic bone phatasia using green fluorescent protein chimeras. J Clin Endocrinol
disease with the Ilizarov technique. Clin Orthop Relat Res. Metab. 1998;83(11):3936–42.
1994;301:38–41. 30. Whyte MP, Kurtzberg J, McAlister WH, Mumm S, Podgornik MN,
27. Barrett IR, Papadimitriou DG. Skeletal disorders in children with Coburn SP, et al. Marrow cell transplantation for infantile hypo-
renal failure. J Pediatr Orthop. 1996;16:264–71. phosphatasia. J Bone Miner Res. 2003;18(4):624–36.
Osteogenesis Imperfecta
16
François R. Fassier
Surgical Correction
Why?
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
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].
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
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
16 Osteogenesis Imperfecta 261
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
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.
16 Osteogenesis Imperfecta 263
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
MM, et al. Correction of lower extremity angular deformities in
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.
References 21. Nicolaou N, Bowe JD, Wilkinson JM, Fernandes JA, Bell MJ. Use
of the Sheffield telescopic intramedullary rod system for the man-
1. Sofield HA, Millar EA. Fragmentation, realignment, and intramed- agement of osteogenesis imperfecta: clinical outcomes at an aver-
ullary rod fixation of deformities of the long bones in children: a age follow-up of nineteen years. J Bone Joint Surg Am.
ten-year appraisal. J Bone Joint Surg Am. 1959;41:1371–91. 2011;93(21):1994–2000.
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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
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
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
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:
17 Lower Limb Deformity in Neuromuscular Disorders: Pathophysiology, Assessment, Goals, and Principles of Management 275
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. 17.15 (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
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
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
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
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].
17 Lower Limb Deformity in Neuromuscular Disorders: Pathophysiology, Assessment, Goals, and Principles of Management 287
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
17 Lower Limb Deformity in Neuromuscular Disorders: Pathophysiology, Assessment, Goals, and Principles of Management 289
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 17.3 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
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
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
e-mail: [email protected]
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].
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].
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.
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
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
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. 18.9 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
304 R. Hamdy and N. Dahan-Oliel
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
306 R. Hamdy and N. Dahan-Oliel
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
• 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
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).
308 R. Hamdy and N. Dahan-Oliel
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.
Summary
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-
Genet. 1982;11(2):185–239.
alized and all the affected joints of the upper and lower limbs 11. Sells JM, Jaffe KM, Hall JG. Amyoplasia, the most common type
and spine should be comprehensively evaluated. of arthrogryposis: the potential for good outcome. Pediatrics.
Multidisciplinary treatment of the child with arthrogryposis 1996;97(2):225–31.
12. Alvarado DM, Buchan JG, Gurnett CA, Dobbs MB. Exome
with a focus on early and continued clinical evaluation is
sequencing identifies an MYH3 mutation in a family with distal
warranted for a positive prognosis and to optimize the arthrogryposis type 1. J Bone Joint Surg Am. 2011;93(11):
affected child’s integration in society and improve his or her 1045–50.
quality of life. 13. Attali R, Warwar N, Israel A, Gurt I, McNally E, Puckelwartz M,
et al. Mutation of SYNE-1, encoding an essential component of the
nuclear lamina, is responsible for autosomal recessive arthrogrypo-
sis. Hum Mol Genet. 2009;18(18):3462–9.
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et al. 7 Mb de novo deletion within 8q21 in a patient with distal
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Limb Lengthening and Deformity
Correction in Patients with Skeletal 19
Dysplasias
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
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
316 M.M. Thacker et al.
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-
19 Limb Lengthening and Deformity Correction in Patients with Skeletal Dysplasias 317
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.
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
19 Limb Lengthening and Deformity Correction in Patients with Skeletal Dysplasias 319
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
19 Limb Lengthening and Deformity Correction in Patients with Skeletal Dysplasias 321
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
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
322 M.M. Thacker et al.
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).
19 Limb Lengthening and Deformity Correction in Patients with Skeletal Dysplasias 323
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 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
324 M.M. Thacker et al.
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).
19 Limb Lengthening and Deformity Correction in Patients with Skeletal Dysplasias 325
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
Fig. 19.11 (a, b) Clinical
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
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].
19 Limb Lengthening and Deformity Correction in Patients with Skeletal Dysplasias 329
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
330 M.M. Thacker et al.
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disease. Am J Hum Genet. 1991;49(5):1091–3. single-nucleotide polymorphisms. Clin Genet. 2002;61(2):146–51.
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18–21. in the Ellis-van Creveld syndrome. J Child Orthop. 2014;8(1):61–9.
Lower Extremity Benign Bone Lesions
and Related Conditions 20
Lori Karol
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].
20 Lower Extremity Benign Bone Lesions and Related Conditions 339
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.
20 Lower Extremity Benign Bone Lesions and Related Conditions 343
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Management of Juxtaphyseal
Malignant Bone Tumors Around 21
the Knee Joint: New Concepts
in Limb-Sparing Surgery
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. 21.4 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.
352 H. Matsubara and H. Tsuchiya
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.
21 Management of Juxtaphyseal Malignant Bone Tumors Around the Knee Joint… 353
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.
354 H. Matsubara and H. Tsuchiya
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.
21 Management of Juxtaphyseal Malignant Bone Tumors Around the Knee Joint… 355
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.
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.
21 Management of Juxtaphyseal Malignant Bone Tumors Around the Knee Joint… 357
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
358 H. Matsubara and H. Tsuchiya
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
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
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.
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).
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 367
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.
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 369
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
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 371
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
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].
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 377
(continued) (continued)
378 D. Paley et al.
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 381
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-
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 383
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 385
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 dis- tinct 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,
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 387
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.
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 391
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.
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 395
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 397
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.
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.
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 401
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 403
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 405
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 407
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.
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. 22.23 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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 411
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 413
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.
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 415
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 417
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 419
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.
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
22 Congenital Femoral Deficiency Reconstruction and Lengthening Surgery 421
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
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
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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,
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Fibular Hemimelia: Principles
and Techniques of Management 23
John E. Herzenberg, Lior Shabtai, and Shawn C. Standard
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
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
Tibiotalar joint
H V S
(Horizontal - (Valgus) (Spherical)
Normal)
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.
23 Fibular Hemimelia: Principles and Techniques of Management 431
6–10%
< 6%
Contralateral
side
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
23 Fibular Hemimelia: Principles and Techniques of Management 433
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
434 J.E. Herzenberg et al.
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).
23 Fibular Hemimelia: Principles and Techniques of Management 435
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
436 J.E. Herzenberg et al.
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
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
23 Fibular Hemimelia: Principles and Techniques of Management 439
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
440 J.E. Herzenberg et al.
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
23 Fibular Hemimelia: Principles and Techniques of Management 441
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.
442 J.E. Herzenberg et al.
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
444 J.E. Herzenberg et al.
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
23 Fibular Hemimelia: Principles and Techniques of Management 445
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
23 Fibular Hemimelia: Principles and Techniques of Management 447
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
448 J.E. Herzenberg et al.
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.
23 Fibular Hemimelia: Principles and Techniques of Management 449
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
Opening Bone
wedge graft
osteotomy
Wire
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.
452 J.E. Herzenberg et al.
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.
23 Fibular Hemimelia: Principles and Techniques of Management 453
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.
14. El-Sayed MM, Correll J, Pohlig K. Limb sparing reconstruc-
87.5 %.
tive surgery and Ilizarov lengthening in fibular hemimelia of
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.
plantigrade feet and two patients had equinus deformity. The 15. Das S, Ganesh GS, Pradhan S, Mohanty RN. Outcome of
eight-plate hemiepiphysiodesis on genu valgum and height correc-
other patients had minor complications (pain and pin-tract
tion in bilateral fibular hemimelia. J Pediatr Orthop B. 2014;23(1):
infection). 67–72.
There is still a major debate amongst pediatric orthopedic 16. Herzenberg JE, Paley D, Gillespie R. Limb deficiency. In: Staheli
practitioners regarding the relative value of lengthening LT, editor. Pediatric orthopaedic secrets. 2nd ed. Philadelphia, PA:
Hanley & Belfus, Inc.; 2003. p. 406–16.
versus amputation for higher grades of FH. Regardless of the
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.
References 22. Radler C, Myers AK, Hunter RJ, Arrabal PP, Herzenberg
JE. Prenatal diagnosis of congenital femoral deficiency and fibular
1. Boakes JL, Stevens PM, Moseley RF. Treatment of genu valgus hemimelia. Prenat Diagn. 2014;34(10):940–5.
deformity in congenital absence of the fibula. J Pediatr Orthop. 23. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method
1991;11(6):721–4. for predicting limb-length discrepancy. J Bone Joint Surg Am.
2. Froster UG, Baird PA. Congenital defects of lower limbs and asso- 2000;82-A(10):1432–46.
ciated malformations: a population based study. Am J Med Genet. 24. Paley J, Talor J, Levin A, Bhave A, Paley D, Herzenberg JE. The
1993;45(1):60–4. multiplier method for prediction of adult height. J Pediatr Orthop.
3. Rogala EJ, Wynne-Davies R, Littlejohn A, Gormley J. Congenital 2004;24(6):732–7.
limb anomalies: frequency and aetiological factors. Data from the 25. Shabtai L, Specht SC, Standard SC, Herzenberg JE. Internal length-
Edinburgh Register of the Newborn (1964-68). J Med Genet. ening device for congenital femoral deficiency and fibular hemime-
1974;11(3):221–33. lia. Clin Orthop Relat Res. 2014;472(12):3860–8.
4. Stevens PM, Arms D. Postaxial hypoplasia of the lower extremity. 26. Belthur MV, Paley D, Jindal G, Burghardt RD, Specht SC, Herzenberg
J Pediatr Orthop. 2000;20(2):166–72. JE. Tibial lengthening: extraarticular calcaneotibial screw to prevent
5. Birch JG, Lincoln TL, Mack PW, Birch CM. Congenital fibular ankle equinus. Clin Orthop Relat Res. 2008;466(12):3003–10.
deficiency: a review of thirty years’ experience at one institution 27. Choi IH, Kumar SJ, Bowen JR. Amputation or limb-lengthening
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.
6. Rodriguez-Ramirez A, Thacker MM, Becerra LC, Riddle EC, 28. Dahl MT, Fischer DA. Lower extremity lengthening by Wagner’s
Mackenzie WG. Limb length discrepancy and congenital limb anom- method and by callus distraction. Orthop Clin North Am.
alies in fibular hemimelia. J Pediatr Orthop B. 2010;19(5):436–40. 1991;22(4):643–9.
7. Roux MO, Carlioz H. Clinical examination and investigation of the 29. Cheng JC, Cheung KW, Ng BK. Severe progressive deformities
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Orthop. 1999;19(2):247–51. Surg Br. 1998;80(5):772–6.
8. McCarthy JJ, Glancy GL, Chnag FM, Eilert RE. Fibular hemime- 30. Catagni MA, Bolano L, Cattaneo R. Management of fibular hemi-
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9. Changulani M, Ali F, Mulgrew E, Day JB, Zenios M. Outcome of 31. El-Tayeby HM, Ahmed AA. Ankle reconstruction in type II fibular
limb lengthening in fibular hemimelia and a functional foot. J Child hemimelia. Strategies Trauma Limb Reconstr. 2012;7(1):23–6.
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-
NA. Limb lengthening and deformity correction by the Ilizarov lia type II: can it be an alternative to amputation? J Pediatr Orthop
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tion. Clin Orthop Relat Res. 2011;469(4):1175–80. 33. Başbozkurt M, Yildiz C, Kömürcü M, Demiralp B, Kürklü M,
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317–9. 46–53. Turkish.
Tibial Hemimelia
24
Dror Paley and David 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
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
458 D. Paley and D.Y. Chong
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
460 D. Paley and D.Y. Chong
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.
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
24 Tibial Hemimelia 461
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
462 D. Paley and D.Y. Chong
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
464 D. Paley and D.Y. Chong
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
24 Tibial Hemimelia 465
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
466 D. Paley and D.Y. Chong
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
24 Tibial Hemimelia 467
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.
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
24 Tibial Hemimelia 469
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
24 Tibial Hemimelia 471
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
472 D. Paley and D.Y. Chong
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
24 Tibial Hemimelia 473
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,
474 D. Paley and D.Y. Chong
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
476 D. Paley and D.Y. Chong
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.
478 D. Paley and D.Y. Chong
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
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
480 D. Paley and D.Y. Chong
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Congenital Pseudarthrosis of the Tibia
25
Corinna C. Franklin and Richard 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.
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
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
25 Congenital Pseudarthrosis of the Tibia 487
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
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.
490 C.C. Franklin and R.S. Davidson
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
25 Congenital Pseudarthrosis of the Tibia 491
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
Bisphosphonates
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].
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.
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Congenital Posteromedial Bowing
of the Tibia 26
Benjamin Joseph, Hitesh Shah, and N.D. Siddesh
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-
e-mail: [email protected]
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.
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
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
30
Slow resolution
20
Plateau
10
0
0 1 2 3 4 5 6
Age (years)
Treatment
Indications
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
Decision Making
A A‘ A A‘
PB PB
TP TP
Peroneus Longus
Peroneus Longus
Achilles Achilles
tendon tendon
S‘ S‘
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
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.
27 Controversies in Blount’s Disease 507
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
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
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
Associated Deformities
Growth Modulation
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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
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
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.
522 H.-R. Song et al.
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
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).
28 Methods to Enhance Bone Formation in Distraction Osteogenesis 527
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].
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
28 Methods to Enhance Bone Formation in Distraction Osteogenesis 529
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Residual Deformities of the Hip
29
Shawn C. Standard and Daniel K. Ruggles
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
540 S.C. Standard and D.K. Ruggles
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
29 Residual Deformities of the Hip 541
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.
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.
544 S.C. Standard and D.K. Ruggles
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.
29 Residual Deformities of the Hip 545
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
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.
29 Residual Deformities of the Hip 547
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
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
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
550 S.C. Standard and D.K. Ruggles
Postoperative Care
Coxa Breva
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.
Deformity Correction and Fixation
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).
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
29 Residual Deformities of the Hip 553
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 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
29 Residual Deformities of the Hip 555
Fig. 29.36 Step-by-step schematic summary of the Morscher osteotomy. Reprinted with permission from the Rubin Institute for Advanced
Orthopedics, Sinai Hospital of Baltimore
556 S.C. Standard and D.K. Ruggles
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
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
558 S.C. Standard and D.K. Ruggles
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
29 Residual Deformities of the Hip 559
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).
Postoperative Care
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
562 S.C. Standard and D.K. Ruggles
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.
29 Residual Deformities of the Hip 563
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
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).
29 Residual Deformities of the Hip 565
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
566 S.C. Standard and D.K. Ruggles
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2014;437(4):1274–83.
Posttraumatic Lower Limb
Deformities in Children 30
Ashok N. Johari, Sandeep A. Patwardhan,
and Taral Vishanji Nagda
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).
572 A.N. Johari et al.
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
30 Posttraumatic Lower Limb Deformities in Children 573
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.
Case Study (Fig. 30.4)
Case Study (Fig. 30.5)
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.
30 Posttraumatic Lower Limb Deformities in Children 575
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 %.
30 Posttraumatic Lower Limb Deformities in Children 577
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 pro- be 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
578 A.N. Johari et al.
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
30 Posttraumatic Lower Limb Deformities in Children 579
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
580 A.N. Johari et al.
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
30 Posttraumatic Lower Limb Deformities in Children 581
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
582 A.N. Johari et al.
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.
Case Study (Fig. 30.14)
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
584 A.N. Johari et al.
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.
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
30 Posttraumatic Lower Limb Deformities in Children 585
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
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
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.
31 Postinfectious Deformities of the Lower Limb 591
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
31 Postinfectious Deformities of the Lower Limb 593
“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
31 Postinfectious Deformities of the Lower Limb 595
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.
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.
31 Postinfectious Deformities of the Lower Limb 599
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
However, an intra-articular osteotomy is a technically demand- 7. Fabry G, Meire E. Septic arthritis of the hip in children: poor results
after late and inadequate treatment. J Pediatr Orthop. 1983;
ing procedure requiring intensive postoperative physiotherapy, 3:461–6.
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follow-up results of such intra-articular osteotomies are cur- study. Clin Orthop. 1978;132:115–28.
rently unavailable. 9. Shapiro F. Pediatric orthopedic deformities. Basic science, diag-
nosis, and treatment. London: Academic Press; 2001.
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Iatrogenic Deformities
32
Austin T. Fragomen and S. Robert Rozbruch
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
e-mail: [email protected]
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
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°.
606 A.T. Fragomen and S.R. Rozbruch
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
32 Iatrogenic Deformities 607
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 correc- tion [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 near- ing, 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
608 A.T. Fragomen and S.R. Rozbruch
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
32 Iatrogenic Deformities 609
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
610 A.T. Fragomen and S.R. Rozbruch
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,
32 Iatrogenic Deformities 611
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.
612 A.T. Fragomen and S.R. Rozbruch
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
32 Iatrogenic Deformities 613
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.
614 A.T. Fragomen and S.R. Rozbruch
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
32 Iatrogenic Deformities 615
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 dis- placed 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
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.
32 Iatrogenic Deformities 617
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
618 A.T. Fragomen and S.R. Rozbruch
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Index
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
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