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Journal of Pediatric Surgery 52 (2017) 449–453

Contents lists available at ScienceDirect

Journal of Pediatric Surgery


journal homepage: www.elsevier.com/locate/jpedsurg

Long-term results of the Duhamel technique are superior to those of the


transanal pullthrough
A study of fecal continence and quality of life
Ana Cristina Aoun Tannuri, Mariana Aparecida Elisei Ferreira, Arthur Loguetti Mathias, Uenis Tannuri ⁎
Division of Pediatric Surgery and Liver Transplantation Unit, University of Sao Paulo Medical School, Sao Paulo, Brazil

a r t i c l e i n f o a b s t r a c t

Article history: Background/purpose: The Duhamel and transanal pull-through (TAPT) techniques have been commonly used for
Received 21 April 2016 the treatment of children with Hirschsprung disease (HD). However, despite adequate treatment, some patients
Received in revised form 26 August 2016 present with fecal incontinence that severely affects the quality of life (QoL) and lead to psychiatric disorders. The
Accepted 11 October 2016 objectives of the present study were to evaluate, through previously adapted questionnaires, the incidence of
fecal incontinence and the quality of life (QoL) of children with HD who underwent Duhamel or TAPT techniques.
Key words:
In addition, we compared the incidence of fecal incontinence and QoL indices in these patients with those in
Fecal continence
Quality of life
healthy children.
Validation of questionnaire Materials/methods: The Fecal Continence Index (FCI) questionnaire and the questionnaire for the Assessment of Qual-
Hirschsprung disease ity of Life in Children and Adolescents with Fecal Incontinence (AQLCAFI) were used in this study. A series of 41 pa-
Transanal pull-through tients with HD were divided into 2 groups according to the utilized surgical technique: the Duhamel group (20
Aganglionosis patients) and the TAPT group (21 patients). The patients were compared with a control group of 59 healthy children.
Results: In the control group, 25 (42.4%) children had good continence and 34 (57.6%) had normal continence. In con-
trast, among patients with HD, 4 (9.8%) had poor fecal continence, 11 (26.8%) had fair continence, 18 (43.9%) had good
continence, and 8 (19.5%) had normal continence. The QoL of children with HD was globally impaired in all domains
of the AQLCAFI as well as in the FCI, when compared with the QoL of healthy children (P = 0.001). The comparison
between children who underwent surgery with the Duhamel technique and those who underwent surgery with
TAPT technique showed similar outcomes according to the FCI. However, the results were inferior in patients who
underwent TAPT technique according to the AQLCAFI questionnaire (P = 0.003), lifestyle (P = 0.006), behavior
(P = 0.01), depression (P = 0.01), and embarrassment (P = 0.003).
Conclusion: The QoL and the FCI were impaired in patients who underwent surgery for correction of HD compared
with healthy children; however, the impairment in QoL was greater in patients who underwent TAPT technique.
Level of evidence: 2B.
Published by Elsevier Inc.

Although several techniques for the correction of Hirschsprung's dis- preserves the muscle layer and the serosa. In addition, by avoiding a lap-
ease (HD) are available, the common goal of all of these techniques is arotomy, this technique eliminates the risks of intraabdominal contam-
the resection of the aganglionic colon with reconstruction of the intesti- ination, the formation of intestinal adhesions, and damage to pelvic
nal transit through anastomosis of the innervated colon to the anus, structures [4].
with preservation of the internal sphincter function [1,2]. Among the Although adequately treated, some patients present with fecal in-
utilized techniques for correction of HD, the Duhamel technique and continence and constipation. Fecal incontinence is the inability to con-
the transanal pull-through (TAPT) technique proposed by De la Torre- trol the release of flatus and/or feces. This condition may become an
Mondragon have been the most frequently used. The greatest advan- occupational, social, affective, athletic, and sexual obstacle for the pa-
tage of the first technique is a restricted dissection of the pelvic rectum tient and may lead to psychiatric disorders and even loss of indepen-
[3], while for the latter, the greatest advantage is the perineal access dence [5]. Therefore, fecal incontinence is intimately associated with
with resection of the mucosal–submucosal aganglionic cuff, which quality of life (QoL), which, according to the World Health Organization,
is defined as “individual's perception of his/her position in life in the
context of the culture and value systems in which he/she lives and in re-
⁎ Corresponding author at: Faculdade de Medicina da Universidade de São Paulo,
Avenida Dr. Arnaldo 455, 4° andar, sala 4106, São Paulo, – SP CEP: 01246-903, Brazil.
lation to his/her goals, expectations, standards and concerns” [5]. Thus,
Tel.: +55 11 30812943; fax: +55 11 32556285. in the last decade, QoL has become an important measure for outcomes
E-mail address: [email protected] (U. Tannuri). in medicine, and it has also been important in the follow-up of patients

http://dx.doi.org/10.1016/j.jpedsurg.2016.10.007
0022-3468/Published by Elsevier Inc.
450 A.C.A. Tannuri et al. / Journal of Pediatric Surgery 52 (2017) 449–453

with HD, which justifies the use of questionnaires for the assessment of satisfaction with his or her own health and intestinal function. Each
QoL in the setting of HD. question is assigned a score from 1 to 4, with 1 being the worst scenario.
In a review article, the majority of the studies analyzed by Hartman The final score, which ranges from 4 to 16, is obtained by a summation
et al. [6] showed that patients with impaired intestinal function also had of the average scores obtained in each domain.
impaired QoL, with a correlation that varied from small, moderate, to
strong. Additional studies showed that children are more affected by 1.3. Patients
fecal incontinence in comparison with adolescents; in contrast, the
QoL among adolescents is more impaired than that in children [7]. From 1973 to 1999, in the Pediatric Surgery Division of the Universi-
Therefore, questionnaires that allow an objective assessment and a ty of São Paulo Medical School, all HD patients were treated with the
longitudinal patient follow-up have been increasingly utilized. Many Duhamel procedure. In the year 2000, the TAPT procedure was intro-
questionnaires associate QoL with fecal incontinence, while others are duced and indicated mainly in children with less than 3 years of age.
disease-specific. Some examples are as follows: “Fecal Incontinence The current investigation was based on questionnaires that were applied
Quality of Life (FIQL)” [8] scale, the “Hirschsprung disease/Anorectal to 41 patients with HD whose age range varied from 7 to 19 years and
malformation Quality of Life questionnaire (HAQL)” [9] and the “Clinical who are at least 6 months has lapsed from the operation. The patients
Evaluation of Continence (Holschneider criteria)” [10]. These question- were randomly selected, but we excluded those with conditions that
naires have been adopted and validated in several countries, such as might affect sphincter control, such as neurological disorders and impair-
France, Spain, and Sweden. For use in Portuguese-speaking populations, ment of neuropsychomotor development. Presently, we accumulate an ex-
our research group validated and published the Fecal Continence Index perience of 70 TAPT procedures and more than 200 Duhamel operations.
(FCI) questionnaire and the Assessment of Quality of Life in Children All patients had a clinical history and physical examination compat-
and Adolescents with Fecal Incontinence (AQLCAFI) questionnaire [11]. ible with HD. The HD diagnosis was confirmed by rectal biopsy, which
Nowadays, the long-term outcomes of the Duhamel technique and assessed the presence of ganglion cells in hematoxylin–eosin-stained
the TAPT technique vary across published studies, and no consensus sections, and by immunohistochemistry, which evaluated acetylcholin-
has been reached regarding the best technique for HD correction esterase activity. We selected the patients in whom an opaque enema
when functional outcomes, fecal continence, and QoL are considered. showed a transition zone in the rectum-sigmoid. These cases were ran-
Thus, the objectives of the present study were to perform a long-term domly divided into 2 groups according to the type of surgery performed
assessment of the fecal continence and QoL of children in a series of pa- and were matched by gender and age, as follows: Duhamel group (n =
tients who underwent surgery for the correction of HD through ques- 20 patients); and TAPT group (n = 21 patients).
tionnaires specifically designed for the study of our pediatric All the patients were operated on by one of the three surgeons
population. Additionally, we compared the fecal continence index and (ACAT, ALM or UT). Duhamel procedure was performed according to a
QoL in children with HD with the fecal continence index and QoL in a previously used protocol that involved a preoperative colostomy in
group of healthy children. the transition zone of the colon and indicating the pull through opera-
tion after the sixth month of life. Two days before the operation, the pa-
1. Materials and methods tients received a mechanical colon preparation consisting of stoma and
rectal irrigations, and antibiotics were initiated (parenteral gentamicin
The tools utilized to evaluate QoL and fecal continence were the and oral metronidazole). A classical pull-through of the colostomy
AQLCAFI and the FCI, respectively. These instruments were based on stoma was performed according to the Duhamel technique.
well-established questionnaires that have been subjected to a rigorous The patients who underwent TAPT were started on preoperative in-
validation process, as described in a previous study performed by the tramuscular gentamicin and oral metronidazole and only one or two
same group [11]. The questionnaires were mailed to patients and were colon irrigations were performed. In the operating room, after general
also provided through telephone calls or during outpatient visits. The and regional sacral anesthesia, all patients were submitted to a labori-
study protocol was fully approved by the ethical committee of our institu- ous irrigation of feces using warm saline solution, prior to beginning
tion and an informed consent was obtained from all patients' parents. the operation. The patients were operated on in a prone jack-knife posi-
tion and no catheter was introduced through the urethra. As a first step,
1.1. Fecal Continence Index for the transanal rectal mucosal dissection, no circumferential stay su-
tures were performed around the anal verge and no anal retractors
The FCI was based on the Clinical Evaluation of Fecal Continence were used. Instead, in all cases a 4–0 prolene purse-string suture was
(Holschneider Criteria) (Table 1). The FCI questionnaire consists of 8 performed 1 cm above the dentate line and tied in order to occlude
questions of easy comprehension regarding daily activities (questions the rectal lumen and to avoid any contamination during the mucosal
1 to 5); it also contains questions about diarrhea, constipation, and the dissection; this traction suture was used to pull the mucosa. Electrocau-
use of supportive treatments (questions 6 to 8) according to the tery was only utilized for hemostasis of the main vessels and was not
Holschneider criteria. The final score ranges from 0 to 16, with a value used for this delicate dissection, in an effort to prevent injury to the
of 0–2 points for each question. The 0 to 5 range indicates poor conti- thin pelvic nerves. The prolapsed rectal cuff was cut 10 to 12 cm
nence, 6 to 10 indicates fair continence, 11 to 15 indicates good conti- above the starting point of the submucosal dissection and the peritoneal
nence, and the maximum score of 16 indicates normal fecal continence. cavity was reached. The sigmoid colon was dissected to above the tran-
sition zone and if possible, above the dilated zone, at a site with normal
1.2. Assessment of Quality of Life in Children and Adolescents with Fecal In- caliber. The muscular rectal cuff was divided and the normal colon was
continence (AQLCAFI) sutured to the anal canal. After surgery, the antibiotics were adminis-
tered for an additional 48 h.
The AQLCAFI was based on the validated Fecal Incontinence Quality A control group of 59 healthy volunteers who had no complaints re-
of Life (FIQL) questionnaire once it was translated into Portuguese garding bowel function and who were demographically similar to the
(Table 2). The questionnaire contains 24 questions and contains the study patients also answered to the questionnaires. These volunteers
same domains of the original questionnaire: lifestyle (questions 2, 4, 7, were recruited at an outpatient pediatric surgery clinic and had been di-
10, 16, 19, 20; total of 7 items), behavior (questions 1, 6, 11, 12, 15, agnosed with common surgical conditions such as inguinal hernia or
17, 21; total of 7 items), depression (questions 3, 8, 14, 18, 22, 23, 24; phimosis. As with the study groups, these volunteers received the ques-
total of 7 items), and embarrassment (questions 5, 9, 13; total of 3 tionnaires by mail or they responded to the questionnaires through
items). Questions 22 and 23 are related to the patient's opinion and phone calls or during clinic visits.
A.C.A. Tannuri et al. / Journal of Pediatric Surgery 52 (2017) 449–453 451

1.4. Statistical analysis (continued)

The data of children with HD were compared to those of control chil-


dren. In addition, the data from children with HD who underwent the
Duhamel technique were compared with the data from children who
underwent the TAPT technique.
Histograms showed that the data do not follow a normal distribu-
tion. Therefore, we employed the Mann–Whitney test in all statistical
analyses. The confidence interval was set at 95% and an alpha of 0.05.

2. Results

Among the 41 patients, 11 (26.8%) were female and 30 (73.2%) were


male, and the mean age was 10.4 ± 3.8 years. After the surgery, all pa-
tients were regularly followed in our service. The follow-up periods
from surgery until the present study for both groups were similar: me-
dian 30 months – range 6 to 60 months; median 26 months – range 6 to
55 months, for Duhamel and TAPT groups, respectively (P = 0.5).

2.1. Comparison of patients with HD and control children

It was observed that the QoL in children with HD was impaired in all
domains in the AQLCAFI as well as in the FCI (median values: AQLCAFI
12.2; lifestyle [LS] 3.7; behavior [BEH] 3.6; depression [DEP] 2.7; embar-
rassment [EMB] 2.3; and FCI 12.0) when compared with the QoL of
healthy children (median values: 15.6, 4.0, 4.0, 3.7, 4.0 and 16.0, for

Table 1
Fecal Continence Index (FCI) questionnaire.

AQLCAFI, lifestyle, behavior, depression, embarrassment and FCI, re-


spectively). The comparisons between HD children and controls
showed significant values (P = 0.001).
Among the controls, 30 children (50.8%) were female and 29 (49.2%)
were male (age – 10.9 ± 2.9 years). Twenty-five children (42.4%)
showed good continence and 34 (57.6%) showed normal continence.
In contrast, among patients with HD, 4 (9.8%) showed poor fecal conti-
nence, 11 (26.8%) fair, 18 (43.9%) good, and 8 (19.5%) showed normal
continence.
452 A.C.A. Tannuri et al. / Journal of Pediatric Surgery 52 (2017) 449–453

Table 2 Table 3
Assessment of Quality of Life in Children and Adolescents with Fecal Incontinence FCI, AQLCAFI, and domains of patients with HD who underwent Duhamel or TAPT
(AQLCAFI). technique.

A. Individual and social questions Median Percentile 25 Percentile 75 P

Below, we will ask other questions to determine if your bowel function bothers FCI Duhamel 13.0 10.0 15.0 0.17
you and how often it bothers you. If this complaint exists but it is not because of TAPT 9.6 6.2 14.1
poor bowel function, please leave the question blank. LS Duhamel 4.0 3.7 4.0 0.006
TAPT 2.8 2.3 3.8
Almost Sometimes Rarely Never
BEH Duhamel 3.8 3.3 4.0 0.01
always
TAPT 3.0 2.2 3.6
1 When I am away from home, I try to
DEP Duhamel 3.4 2.4 3.8 0.01
stay near the bathroom
TAPT 2.3 1.8 3.4
2 I avoid visiting my friends
EMB Duhamel 3.7 2.3 4.0 0.003
3 I am not often invited to parties or to
TAPT 1.5 1.0 3.0
go on trips
AQLCAFI Duhamel 15.1 12.0 15.8 0.003
4 I avoid spending the night away from
TAPT 9.9 7.7 13.8
home
5 I fear that people may smell feces or FCI (Fecal Continence Index); LS (Life style); BEH (Behavior), DEP (Depression); EMB (Em-
flatus barrassment); AQLCAFI (Assessment of QoL in Children and Adolescents with Fecal
6 I would rather stay home than go out Incontinence);
7 I avoid eating out
8 I am unable to participate in activities
with my friends
9 I avoid talking about the problem with
questionnaires, which are essential for research studies, as they allow
others useful assessments in clinical practice.
10 I need to plan my activities according Many of the traditional questionnaires, such as the “Fecal Inconti-
to my bowel function nence Questionnaire” [13], “Fecal Incontinence Severity Index” [14],
11 It impairs my school performance
“Fecal Incontinence QoL (FIQL),” “Hirschsprung's disease Anorectal mal-
12 It affects my professional work
13 I release stool without noticing formation QoL (HAQL),” “Medical Outcomes Study 36-item short –
14 I prefer for people not to be aware of Form Health Survey,” [15] and “Clinical Evaluation of Continence
my problem (Holschneider Criteria),” have been translated into different versions.
15 It hinders the practice of sports Minguez et al. validated the FIQL questionnaire in Spanish and applied
16 I avoid traveling
17 I have a difficult time making friends
it to patients with fecal incontinence because of several etiologies
18 I worry about accidents with feces [16]; Rullier et al. validated the FIQL in French [17], while Wigander
19 Leaving home worries me et al. translated the HAQL questionnaire into Swedish and adapted it
20 I stop doing the things I like to Swedish culture [18]. In Brazil, Yusuf et al. validated the FIQL in Por-
21 I feel that I cannot control my bowel
tuguese in an adult population with an average age of 52.8 years, but
movements
they did not adapt this questionnaire to children and adolescents [19].
Poor Fair Good Excellent Following this trend, our group created and validated the AQLCAFI
22 In general, you think your health is:
and FCI questionnaires because our group of patients has specific char-
23 How would you rate your bowel
function? acteristics that are not incorporated into other questionnaires [11]. The
24 In your case, if it was indicated, AQLCAFI and the FCI are both in Portuguese and address relevant issues
would you accept other forms of of children and adolescents; these questionnaires use accessible lan-
treatment or surgeries to improve guage and avoid overly technical terms and choices that are complex
your bowel function?
and difficult to understand. These questionnaires can be applied
through interviews or may be completed by the patient or parents with-
out the need for an interviewer. Questions considered inadequate for
children, such as those that involve sexuality or situations of severe de-
2.2. Comparison of Duhamel and TAPT groups pression, were excluded. Translation, cultural adaptation, reproducibil-
ity over time, construct validity, and discriminative validity were
The median age of children in the Duhamel group was 8 years (range rigorously performed and assessed. Once finalized, these instruments
7 to 12 years). The median age of operation in this group was were applied to a larger number of patients, and the present study re-
41.0 months (range 6 months–9 years and 2 months). Five children ports the data on these patients. Finally, it is important to stress that
were female and 15 were male. Two (10%), 4 (20%), 9 (45%), and 5 in the process of questionnaires validation we have shown a strong cor-
(25%) children presented with poor, fair, good and normal continence, relation between FCI and AQLCAFI in all its domains although no age ef-
respectively. In the TAPT group, the median age was 8 years (range 7 fect had been studied [11].
to 11 years). The median age of operation was 10.0 months (range Although our study may have some limitations because of the sam-
10 days–6 years). Sixteen were male and 5 were female. Four (19.0%), ple size of Duhamel and TAPT groups, the number of groups is similar to
7 (33.4%), 6 (28.6%) and 4 (19.0%) children presented with poor, fair, those from other studies [20,21] and we could take to important and
good, and normal continence, respectively. Both groups performed sim- significant conclusions. First of all, the inclusion of a control group
ilarly on the FCI, but the differences in the AQLCAFI values and domains allowed us to conclude that the QoL in children with HD was impaired
were statistically significant, and lower scores were observed in patients in all domains in the AQLCAFI as well as in the FCI. As previously
who underwent TAPT technique (Table 3). shown, the disease itself may be associated with other anomalies like
Down syndrome or genetic mutations [22,23]. In addition, surgical pro-
3. Discussion cedures in this region, mainly TAPT procedure, may damage microstruc-
tures such as nerves and muscle fibers. These critical structures are
Fecal incontinence may occur to some degree of intensity in approx- responsible for differentiation between gas, solid and liquid stools, and
imately 30% to 50% of patients with HD [12], which is similar to what have a very important role in continence mechanisms. Consequently,
was observed in the present study. The importance of fecal incontinence a decrease in QoL was identified in these patients. Among the AQLCAFI
and its relationship with QoL have been widely investigated through domains, the most affected were embarrassment (median of 4.0 for
A.C.A. Tannuri et al. / Journal of Pediatric Surgery 52 (2017) 449–453 453

control healthy children and 2.3 for the HD group) and depression (me- overcoming the psychological and social difficulties caused by changes
dian of 3.7 for controls and 2.7 for HD children). Fifteen patients (36.6%) in fecal continence. A better understanding and acceptance of limita-
were considered incontinent (with poor or fair continence), whereas no tions will lead to self-knowledge and the practice of more appropriate
control child presented with such a problem. Although the average age nutritional recommendations. This will result in improved fecal conti-
of the study patients suggests that most of them have not yet reached nence and QoL in children with these disorders.
adolescence age, we observed that even in childhood, problems related
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