Original Article

Long-Term Outcomes and Quality of Life in

Patients after Soave Pull-Through Operation for
Hirschsprung’s Disease: An Observational
Retrospective Study
Viet Quoc Tran1,2 Tania Mahler3 Martine Dassonville2 Dinh Quang Truong1 Annie Robert4
Philippe Goyens5 Henri Steyaert2

1 Department of Pediatric Surgery, City Children’s Hospital, Ho Chi Address for correspondence Viet Quoc Tran, MD, Department of
Minh City, Vietnam Pediatric Surgery, City Children’s Hospital, 15 Vo Tran Chi, Tan Kien,
2 Hôpital Universitaire des Enfants Reine Fabiola, Bruxelles, Belgique — Binh Chanh, Ho Chi Minh City, Vietnam
Université Libre de Bruxelles (ULB), Brussels, Belgium (e-mail: dr.tranquocviet@gmail.com).
3 Department of Gastroenterology, Hôpital Universitaire des Enfants
Reine Fabiola, Bruxelles, Belgique — Université Libre de Bruxelles
(ULB), Brussels, Belgium
4 Faculté de santé publique, Institut de Recherche Expérimentale et

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Clinique, Université catholique de Louvain (UCL), Brussels, Belgium
5 Laboratory of Pediatrics, Université Libre de Bruxelles (ULB),
Brussels, Belgium

Eur J Pediatr Surg

Abstract Introduction Patients after pull-through operation for Hirschsprung’s disease (HD)
are at high risk of defecation disorders. This study aimed at investigating their long-
term outcomes and quality of life (QoL) in comparison with controls.
Patients and Methods Patients older than 5 years operated on for HD were
interviewed to complete detailed questionnaires on bowel function. Patients without
neurologic impairment were enrolled in a QoL survey to compare with controls
matched for sex and age and selected randomly from the general population using
sampling set in a ratio of four controls to one case of HD.
Results In total, 53 operated patients were enrolled. Mean age of the patients was
16
8 years, with 68% boys. Rectosigmoid aganglionosis was the most seen form of
HD in 38 (72%) cases. Open Soave was performed in 40 (75.5%) cases, and minimally
invasive surgery Soave (MIS Soave) in 13 (24.5%) cases. At investigation, prevalence of
fecal incontinence and constipation were 22.6 and 13.2%, respectively. Regarding QoL
survey, 45 patients and 180 controls were enrolled, excluding 8 patients with
Keywords neurologic impairment. Thirty-seven (82.2%) patients were classified as having a
► Hirschsprung’s good QoL (score  9 points); whereas six had a fair QoL (5–8 points) and two had a
disease poor QoL (< 5 points). QoL score in the cases and the controls were 10.2
2.5 and
► fecal incontinence 11.9
0.4 points, respectively. Long aganglionosis form of HD was significantly
► constipation associated with a low QoL (score < 8 points), adjusted odds ratio ¼ 9, 95% confidence
► quality of life interval [1.3; 64.1] (p < 0.05). In subscales analyses, the prevalence of each dimension
► long-term outcomes including fecal continence, school absenteeism, unhappiness or anxiety, food

received © Georg Thieme Verlag KG DOI https://doi.org/

October 7, 2016 Stuttgart · New York 10.1055/s-0037-1604115.
accepted after revision ISSN 0939-7248.
May 28, 2017
Long-Term Outcomes and QoL in Patients after Soave Pull-Through Operation for HD Tran et al

restriction, and peer rejection was significantly higher in operated patients than in
controls (p <0.001).
Conclusions Although the QoL of patients operated on for HD in general was with
good outcomes, fecal incontinence and constipation still are problematic issues and
challenges in a high percentage of patients. Therefore, a long-term and multidisci-
plinary follow-up is essentially required for these patients.

Introduction study, NI patients were considered as having a chromosomal

Hirschsprung’s disease (HD) is a common congenital mal-
formation of the enteric nervous system characterized by an
absence of parasympathetic ganglia in the submucosal and
myenteric plexus of the distal large bowel.1 Regarding sur-
gical treatment, the distal aganglionic bowel segment is
resected, followed by an anastomosis of the normally in-
syndrome that significantly altered patient neurologic
status.
Regarding QoL, we performed a case–control analysis.
Controls were recruited among the general population.
Inclusion criteria of controls were having no prior surgical
intervention within the gastrointestinal tract. They were
matched to cases by birth year and sex using sampling sets

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nervated bowel to the anus.2
Clinically, it is well established that patients operated on
for HD are at high risk for postoperative defecation disor-
ders.3,4 Consequently, these disorders are reported to have a
negative impact on their emotional and social development,
resulting in a potential alteration of their QoL.4–12 Therefore,
long-term outcomes and quality of life (QoL) of these pa-
tients remain key points in postoperative management.4,13
However, assessment of overall QoL as well as bowel function
is difficult to objectify.14 Varying methods have been used in
the literature.4,5,12,13,15–17
So, this study aimed at investigating the long-term out-
comes and its impact on the QoL of patients operated on for
HD by the Soave technique and followed up at the “Hôpital
Universitaire des Enfants Reine Fabiola” (HUDERF) in
Brussels, Belgium. Given that defecation disorders are also
seen in general population, the controls matched by gender
and age were collected in an attempt to systematically
compare both bowel function and QoL with the cases in
this study.18
Materials and Method
An observational retrospective study was conducted at
HUDERF in Brussels, Belgium. This study was approved by
the research ethics committee of the hospital. All consecutive
charts of patients operated on for HD in our department
between 1987 and 2011 were retrieved. Demographic and
clinical data were collected on gender, date of birth, age at
surgery, type of operation, aganglionic colon segment, and
neurologic status. Patients over 5 years of age at the time of
the study received a letter detailing the planned study and
were invited to participate. Patients and their parents were
interviewed by the main investigator to complete two ques-
tionnaires: one about bowel function and one about QoL for
patients without neurologic impairment (non-NI patients).
During interview with the main investigator, a careful clin-
ical investigation was done to rule out the possibility
of distal anorectal stricture or other anomalies. In this
in a ratio of four controls to one HD.
Bowel function was expressed using variables related to
clinically important aspects of the patient’s stooling patterns
such as fecal incontinence, constipation, and enterocolitis, as
defined in the following paragraphs.
Fecal continence was based on the Wingspread classifica-
tion, which defines the following four levels of continence—
excellent, very good: clean (totally continent, toilet trained
with no medication); good: staining (rarely soiling, except
during stressful exercise); fair: intermittent fecal soiling,
urge incontinence; and poor: constant fecal soiling or smear-
ing.19 Patients classified as fair or poor were considered as
fecally incontinent; the other categories were considered as
fecally continent. Of note, we considered that fecal conti-
nence was acquired normally in children older than
4 years.20
Constipation: patients were interviewed for stooling fre-
quency, use of laxatives, and enema over the months pre-
ceding investigation. A patient was considered as suffering
from constipation if defecation was only possible with
laxatives or occurred less than three times per week accord-
ing to the Rome III criteria for functional constipation.21
Enterocolitis: history of enterocolitis was reviewed and
classified on a scale (none, single time, or several times) from
medical records as well as at interview for each patient.
QoL questionnaires: information about QoL of the patients
and the matched controls were collected according to the
scoring system proposed by Bai et al in patients with HD.12
This QoL scoring system includes fecal continence assess-
ment and psychosocial aspects (school absenteeism, unhap-
piness, food restriction, and peer rejection) (►Table 1). The
whole scale was subsequently stratified into three sub-
groups: good (9–12 points), fair (5–8 points), and poor (0–
4 points). A lower score represents a lower QoL and vice
versa. A score less than 9 points (including the two subgroups
fair and poor) was considered as a low QoL.
Operative procedures: initially, Soave pull-through with
abdominal approach (open Soave) was used in our institu-
tion. Recently, transanal endorectal pull through with or

European Journal of Pediatric Surgery

 Long-Term Outcomes and QoL in Patients after Soave Pull-Through Operation for HD Tran et al

Table 1 Quality-of-life scores with subscale analyses of the Statistical Analysis

cases versus the controls, as identified by main investigator at Data are reported as mean and standard deviation or as
the time of the study median and range for continuous variables according to the
normality of the distribution, and as number and proportion
Dimensions Cases Controls p-Value for discrete data. Chi-square or Fisher’s exact test were used
(points of score) (N ¼ 45) (N ¼ 180) to compare categorical data. Independent t-tests and analysis
n (%) n (%)
of variance were used to compare QoL scores among two and
Fecal continence (scale 0–4) more than two groups, respectively. Univariate and multi-
Absent soiling (4) 24 175 <0.001 variate logistic regression were used to identify the contri-
Accidental 15 3 bution of independent variables by calculation of odds ratios
soiling (3) (ORs) and 95% confidence intervals (CIs). All tests were two-
Frequent 3 1 sided and a p-value of <0.05 was considered as significant.
soiling (2) Cramer’s V was used to measure the association between
nominal variables. IBM SPSS Statistics version 20 (SPSS,
Accidental 1 1
incontinence (1) Chicago, Illinois, United States) was used for statistical
analysis.
Frequent 2 0
incontinence (0)
Mean
SD 3.2
1 3.9
0.2 <0.001a Results

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School absenteeism (scale 0–2) A total of 58 consecutive patients operated on for HD with
Never (2) 36 177 <0.001 histopathological proof of HD were retrieved from our
Accidental (1) 7 3 databases, of whom 53 patients (91%) agreed to participate
to the study. The five remaining patients have been lost to
Frequently (0) 2 0
follow-up.
Mean
SD 1.8
0.4 2
0.1 <0.001a
Unhappiness or anxiety (scale 0–2) Patients Characteristics
Never (2) 34 178 <0.001 Patient characteristics are summarized in ►Table 2. Mean
age of the patients at investigation was 16.1
7.6 years, with
Accidental (1) 10 1
68% male. Median age of the patients at surgery was 2.3 (0.3–
Frequently (0) 1 1
74.3) months. There were 8 (15%) out of 53 patients con-
Mean
SD 1.7
0.6 2
0.1 <0.001a sidered as NI patients, including seven cases of Down’s
Food restriction (scale 0–2) syndrome and one case of 13q chromosomal deletion syn-
No (2) 29 172 <0.001 drome. Of note, mean age of NI patients was 9
6.5 years,
significantly younger than 18.4
6.5 years of the non-NI
Somewhat (1) 13 6
patients (p < 0.001). Rectosigmoid aganglionosis was the
Much (0) 3 2 most seen form of HD, representing 38 (72%) cases, and 4
Mean
SD 1.6
0.7 2
0.1 <0.001a (7.5%) cases presented with total colon aganglionosis. Open
Peer rejection (scale 0–2) Soave was performed in 40 (75.5%) cases and MIS Soave in 13
(24.5%) cases.
Never (2) 36 178 <0.001
Accidental (1) 8 2 Bowel Function
Frequently (0) 1 0 Patient chart review and a thorough medical history taken at
Mean
SD 1.7
0.6 2
0.1 <0.001 a interview showed that 13 (24.5%) patients had been treated for
fecal incontinence and 17 (32.1%) others for constipation
Total score 10.2
2.5 11.9
0.4 <0.001
(scale 0–12) during their follow-up at our institution. A nonoperative
medical bowel management therapy was used in most of the
Abbreviation: SD, standard deviation. cases, consisting of two phases. First, fecal disimpaction was
Note: patients with neurologic impairment were excluded. The controls
initiated by oral enemas combined with retrograde colonic
were matched for sex and age at the time of the study and were included
based on having no prior surgical interventions within gastrointestinal washout to evacuate fecaloma if present. Second, a regular
tract. laxative regimen was administered and toilet habits to avoid
a
Independent t-test, otherwise Fisher’s exact test. recurrence of fecal retention were explained. Polyethylene
glycol was the recommended laxative in our hospital. In our
series, one boy aged 8 years with intractable constipation was
without laparoscopic assistance (MIS Soave) was used. In treated with three cures of botulinum injection.22 Another 19-
cases of severe enterocolitis or failure with nursing care year-old male patient underwent a sacral stimulating device
(washout) before pull-through operation, an enterostomy implementation to control severe fecal incontinence.23,24
was performed. For patients with total colon aganglionosis, At investigation, prevalence of fecal incontinence and
an ileoanal anastomosis without reservoir was performed. constipation was 22.6% (12 patients) and 13.2% (7 patients),

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Long-Term Outcomes and QoL in Patients after Soave Pull-Through Operation for HD Tran et al

Table 2 Patients characteristics and their association with fecal incontinence or constipation, as identified by main investigator at
last follow-up (N ¼ 53)

Variables All N Prevalence of fecal p-Value Prevalence of p-Value

incontinence (n/N, %) constipation (n/N, %)
Age (mean
SD), y 16.1
7.6 13.6
7.3 – 14.6
9.1 –
Age groups
5 to <10 y 12 25 0.41 25 0.48
10–16 y 13 30.7 7.7
> 16 y 28 17.9 10.7
Age at surgery (mo)
Median [range] 2.3 [0.3–74.3] – – – –
Gender
Male 36 27.8 0.47 13.9 >0.99
Female 17 11.7 11.7
Neurologic status

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NI patients 8 50 0.048 12.5 >0.99
Non-NI patients 45 17.8 13.3
Enterostomy
Yes 16 31.3 0.72 25 0.18
No 37 18.9 8.1
Resected segment
Rectosigmoid colon 38 23.7 0.93 7.9 0.13
Descending colon 8 12.5 25
Transversal colon 3 0 33.3
Total colon 4 50 25
Operative procedure
Open Soave 40 22.5 >0.99 12.5 >0.99
MIS Soavea 13 23.1 15.4
Enterocolitis
No 44 20.5 0.17 11.4 0.20
Single time 5 20 40
Several times 4 50 0
b
Postoperative complications
Anastomotic leak 3 2 0.25 0 >0.99
Bowel obstruction 3 0 1
Twisted colon 1 0 0
Residual aganglionosis 1 1 0
Total complications 8 3 1

Abbreviations: MIS, minimally invasive surgery; NI, neurologically impaired; SD, standard deviation.
a
Includes transanal endorectal pull-through with or without laparoscopic assistance.
b
Data for postoperative complications reported by numbers of encountered cases.
Note: Bold values (p < 0.05) were considered as statistically significant.

respectively. ►Table 2 shows patient characteristics and
their association with either fecal continence or constipation
at investigation. Prevalence of fecal incontinence was sig-
nificantly increased in NI patients, with OR ¼ 4.6, 95% CI [1;
22.5] (p < 0.05), as illustrated in ►Fig. 1. No other character-
istics of patients were significantly associated with fecal
incontinence, although it was higher in boys, and in cases
with postoperative complication, with enterostomy as well
as a history of enterocolitis. Regarding constipation, although
higher prevalence of constipation was noted in patients
younger than 16 years, in cases with enterostomy and with
long-form HD, none of the variables were significantly

European Journal of Pediatric Surgery

 Long-Term Outcomes and QoL in Patients after Soave Pull-Through Operation for HD
Fig. 1 Association of patient characteristics with either fecal incontinence or constipation, as identified by the main investigator at the time of
the study using univariate logistic regression.
associated (►Fig. 1). When considering the age groups, the
prevalence of both fecal incontinence and constipation were
higher in patients younger than 10 years, compared with the
older groups; however, the differences were not significant.
►Fig. 2 illustrates the prevalence of fecal continence using
the Wingspread classification. The prevalence was signifi-
cantly higher in patients than in controls; 7 (15.6%) out of the
45 non-NI patients had fecal incontinence identified as “fair”
and “poor,” compared with 3 (1.7%) out of the 180 controls.
►Fig. 3 shows stooling frequency in our 45 non-NI pa-
tients. It was significantly different when compared with the
180 controls; 13.3% (6) of the patients reported use of
laxatives or less than three defecations per week, compared
with 4.4% (8) of the controls who reported less than three
defecations per week.
Quality of Life
For the QoL survey, eight patients with NI were excluded. In
the remaining 45 patients, 37 (82.2%) reported a good QoL
(score  9 points), whereas 6 reported a fair QoL (5–8
points) and 2 reported a poor QoL (< 5 points), as illustrated
in ►Fig. 4. QoL scores according to patient characteristics are
illustrated in ►Table 3. No significant differences of QoL
scores were found across age groups and gender. Patients
Tran et al
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with total colon resection, with enterostomy, and with open
Soave reported significantly lower QoL scores as compared
with patients with shorter colon resection, without enter-
ostomy, and with MIS Soave.
►Table 4 shows the association of patient characteristics
with a risk of a lower QoL, defined as a score of <9 points.
There were 8 out of 45 (18%) patients with a low QoL. Risk of a
lower QoL was significantly increased in patients with en-
terostomy (OR ¼ 8.1, 95% CI [1.4; 46.9], p ¼ 0.017) and in
patients with long aganglionosis segment (OR ¼ 9.3, 95% CI
[1.6; 54.7], p ¼ 0.011), as demonstrated in ►Table 3. How-
ever, in multivariate regression analysis, only the long agan-
glionosis form of HD was significantly associated with a low
QoL after adjustment on age, sex, and enterocolitis (adjusted
OR [AOR] ¼ 9, 95% CI [1.3; 64.1]. Operative procedure was
not introduced in the models because no patient in the MIS
group had a low QoL. In addition, enterostomy was not
introduced into the multivariate regression because of its
high correlation with resected segment (Cramer’s V ¼ 0.56).
►Table 1 comprises the QoL scores in 45 nonneurologi-
cally impaired patients operated on for HD compared with
180 control patients that were matched to cases for age and
sex at the time of the study. QoL score in controls was
significantly higher than in operated patients (11.9
0.4
European Journal of Pediatric Surgery
Long-Term Outcomes and QoL in Patients after Soave Pull-Through Operation for HD Tran et al

FECAL CONTINENCE
Cases (n = 45) versus controls (n = 180), p < 0.001

93.9%

90%

70%

53.3%
50%

31.1%
30%

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8.9%
10% 6.7%
4.4%
1.1% 0.6%

Excellent Good Fair Poor

-10%

Cases Controls Cases Controls

Fig. 2 Prevalence of fecal continence in cases versus controls using the Wingspread classification, as identified by the main investigator at the
time of the study. Patients with neurologic impairment were excluded. The controls were matched for sex and age at the time of the study. Bar
charts are presented for percentages and curves for cumulative percentages.

STOOLING FREQUENCY
Cases (n = 45) versus controls (n = 180), p < 0.001
100%

90%

80%
69.4%
70%

60% 55.6%

50%

40%

30%
15.6% 21.1%
20%
13.3%
11.1%
10%
2.2% 5.0%
0.0% 4.4% 2.2% 0.0%
0%
Laxative < 3 times /week 1 time /1-2 days 1-2 times / day 3-5 times /day >6 times /day
-10%
Cases Controls Cases Controls

Fig. 3 Stooling frequency among cases versus controls, as identified by the main investigator at the time of the study. Patients with neurologic
impairment were excluded. The controls were matched for sex and age at the time of the study. Bar charts are presented for percentages and
curves for cumulative percentages.

European Journal of Pediatric Surgery

 Long-Term Outcomes and QoL in Patients after Soave Pull-Through Operation for HD
Fig. 4 Quality-of-life scores of patients at follow-up according to age group (n ¼ 45). Scores range from 0 to 12. Qualitative results were divided
into subgroups: good (9–12 points), fair (5 -8 points), and poor (0–4 points).
vs. 10.2
2.5; p < 0.001), also illustrated in ►Fig. 5. In
subscale analyses, prevalence of each dimension, including
fecal continence, school absenteeism, unhappiness or anxi-
ety, food restriction, and peer rejection, was significantly
higher in operated patients than in controls (p < 0.001).
Discussion
This observational retrospective study analyzes long-term
outcomes related to bowel function of patients with HD and
their QoL. Few series have studied quality of continence and
QoL in a homogenous group in terms of operative procedure
(Soave operation), on a long-term basis (more than half of the
patients were followed up for over 16 years), and comparing
with controls from the general population.13 We reported
that the prevalence of fecal incontinence as well as constipa-
tion was significantly higher in cases than in controls at the
time of the study (►Figs. 2 and 3). Remarkably, the rate of
constipation in our series improved over time (from 32.1%
during the time of follow-up down to 13.2% at investigation),
whereas fecal incontinence remained an issue (from 24.5%
down to 22.6%).25 In general, the history of constipation may
be short in duration and often improves with time, especially
with appropriate bowel management.26,27
At investigation, our findings show that prevalence of
fecal incontinence in patients with HD was 22.6%. This
compares favorably with the rates of up to 33% in the open
pull-through group, as well as with 25% in the MIS group that
have been reported by Gosemann et al in a systematic
review,3 and with the 35% fecal incontinence by Bai et al.12
Tran et al
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A very recent and similar study by Neuvonen et al in Finland
reported that 25% of patients presented socially with fecal
incontinence versus 2% in the controls (p < 0.001).3 This
result was comparable with our study. One reason for
persistent continence problems could be due to the technical
surgical issues that are still an ongoing debate in the litera-
ture.3,26,27 Of note, the operative technique was a Swenson
procedure in the study by Bai et al and a transanal endorectal
pull-through in that of Neuvonen et al. In our series, a Soave
technique was used, and we found no significant difference
between open and MIS Soave in terms of fecal incontinence
(►Fig. 1).
The prevalence of fecal incontinence was statistically
higher in patients with long-form (total colonic) HD and
with NI (►Fig. 1). Catto-Smith et al reported a very high
prevalence of fecal incontinence of 87% in children with
Down’s syndrome associated with HD.28 As described by
Powers et al, bowel continence in patients with Down’s
syndrome could appear at a later age than in normal
patients.29 In our series, NI patients were younger than the
non-NI ones.
The prevalence of constipation in our series was 13.2% at
investigation, which is lower than the 59% reported by
Gosemann et al.3 In contrast, according to Neuvonen et al,
the rate of constipation was 5% in patients versus 4% in
controls.13 In our series, as illustrated in ►Fig. 3, stooling
patterns were significantly different between cases and
controls. Discordance between the studies may be explained
by differences in criteria of defecation disorders as well as by
the exactitude of clinical diagnosis. On the basis of our
European Journal of Pediatric Surgery
Long-Term Outcomes and QoL in Patients after Soave Pull-Through Operation for HD Tran et al

Table 3 Quality-of-life scores of patients operated on for Table 4 Factors associated with LowQoL in patients operated
Hirschsprung’s disease, according to patient characteristics on for Hirschsprung’s disease using multivariate logistic
(N ¼ 45) regression (N ¼ 45)

Characteristics N Quality of life Characteristics n LowQoLa Univariate Multivariate

Mean
SD p-Value % p-Value OR Adjusted

[95%CI] OR [95%CI]
Age groups
Age groups
5 to <10 y 6 9.8
3.3 0.93 5–16 y 17 17.9 >0.99 1 1
10–16 y 11 10.2
2.3 > 16 y 28 17.6 1 0.5
[0.2; 4.8] [0.04; 5]
> 16 y 28 10.3
2.6
Gender
Gender
Male 32 18.8 0.58 1.3 1.9
Male 32 10.2
2.6 0.84 [0.2; 7.3] [0.2; 14.4]

Female 13 10.3
2.3 Female 13 15.4 1 1

Enterostomy Enterostomy
Yes 16 37.5 0.017 8.1 
Yes 16 9
3.2 0.013
[1.4; 46.9]
No 29 11
1.7 No 29 6.9 1 

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Resected segment Resected segment
Rectosigmoid colon 30 10.8
2 0.014 Rectosigmoid 30 6.7 0.011 1 1
segment
Descending colon 8 9.6
3.2
Long segmentb 15 40 9.3 9
Transverse colon 3 10.3
2.1 [1.6; 54.7] [1.3; 64.1]

Total colon 4 6.8
2.5 Operative procedure

Operative procedure Open Soave 37 17.8 0.18  

c
MIS Soave 8 0  
Open Soave 37 10.1
2.7 0.036
Enterocolitis
MIS Soave 8 11.3
0.9
Yes 8 37.5 0.14 3.8 2.6
Enterocolitis [0.7; 32.1] [0.3; 13]
No 37 10.5
2.3 0.09 No 37 13.5 1 1

Single time 4 10.5
3 Postoperative complication

Several times 4 7.5
2.9 Yes 6 33.3 0.29 2.75 4.7

[0.4; 18.5] [0.3; 62.3]
Postoperative complication
No 39 15.4 1 1
Yes 6 9
3.2 0.32
Abbreviations: CI, confidence interval; LowQoL, low quality of life; MIS,
No 39 10.5
2.3 minimally invasive surgery; OR, odds ratio.
Total 45 10.2
2.5 Note: Patients with neurologic impairment were excluded. Bold values
(p < 0.05) were considered as statistically significant.
a
Abbreviations: MIS, minimally invasive surgery; SD, standard deviation. LowQoL: QoL score < 9 points.
b
Note: patients with neurologic impairment were excluded. Bold values Long segment form: includes the cases with descending, transverse,
(p < 0.05) were considered as statistically significant. and total colon aganglionosis.
c
Includes transanal endorectal pull-through with or without laparo-
scopic assistance.

clinical experience, several cases with intractable constipa-
tion could result in fecal overflow incontinence. This issue
may be a consequence of either dysmotility of the proximal
pulled-through colon or loss of “neoanorectal” sensa-
tion.3,30,31 Anorectal manometry is perhaps a more objective
way to evaluate patients with persistent problems of con-
tinence, allowing for precise diagnosis, as suggested by
several authors.32
Regarding QoL, some authors stated that the QoL is
dramatically degraded in a nonnegligible number of patients
operated on for HD, especially those with severe fecal
incontinence.3,16,33 However, the magnitude of the problem
remains imprecise because authors used different defini-
tions of QoL. Moreover, until now, there are no standardized
protocols for evaluating QoL in such patients. In our study, we
used the QoL scoring system proposed by Bai et al for patients
with HD.12 This QoL score includes the assessments of fecal
incontinence and psychosocial aspects. The score is not too
complicated to understand. Simplicity was important to us
because of the heterogeneity of our population (multiple
languages, variety in ethnicities and in cultures, varying
socioeconomic levels, and so on). Therefore, we also con-
ducted a systematic case–control analysis using sampling
sets in a ratio of four controls to one HD in an attempt to
validate this score in a general population.
We reported that the QoL scores in the operated patients
and in the controls were quite good, with 10.2
2.5 and
11.9
0.4 points, respectively (►Table 1). Overall, 82.2% of

European Journal of Pediatric Surgery

 Long-Term Outcomes and QoL in Patients after Soave Pull-Through Operation for HD
QUALITY OF LIFE SCORE
Cases (n = 45) versus Controls (n = 180) , p < 0.001
100%
90%
80%
70%
60%
50%
40%
30%
20%
6.7%
10% 4.4% 1%
2.2% 0% 2.2% 0% 2.2% 0%
0%
0%
4 5 6 7
Cases Controls
Fig. 5 Quality-of-life scores of cases versus controls, as identified by main investigator at the time of the study. The higher curve illustrates a
lower score and a poorer quality of life among cases. Patients with neurologic impairment were excluded. The controls were matched for sex and
age at the time of the study. Bar charts are presented for percentages and curves for cumulative percentages.
the patients were classified as having a good QoL, with QoL
scores  9 points, as illustrated in ►Fig. 4, which is a better
result when comparing to Bai et al, with 7.7
2.9 for cases
versus 11.6
0.7 for controls.12 This difference could be
explained by the fact that the rate of fecal incontinence in our
study was 22.6%, lower than the 35% reported by Bai et al.12
In addition, according to Neuvonen et al, their QoL score was
similarly comparable between patients operated on for HD
and the controls, despite that a different QoL scoring system
was used in their study.13
Looking more precisely at the results, our report demon-
strated that a long-form HD was correlated with a higher risk
of low QoL, defined as a score of <9 points, compared with
the classical rectosigmoid form, with AOR ¼ 9, 95% CI [1.3;
64.1]. In fact, as shown in ►Table 2 as well, patients with
longer aganglionosis are at higher risk of fecal incontinence.
In the literature and in our series, this problem is described as
a main determinant of poor QoL.3–5,12,16 Subscale analyses of
the scores evidenced the multifactorial characteristics of QoL
(►Table 1).4,5 The incidences in terms of school absenteeism,
unhappiness, and worrying about soiling, as well as fecal
incontinence were all more commonly reported by patients
than by controls. Therefore, a long-term and multidisciplin-
ary follow-up is essential in these patients, including not
only the management of medical problems but also the
psychosocial development of our patients.33
Conclusions
Our report shows that the QoL of patients operated on for HD
was generally good. However, defecation disorders remain
Tran et al
93%
46.7%
20.0%
11.1%
5%
4.4% 0%
Downloaded by: MCP Hahnemann University. Copyrighted material.
1%
8 9 10 11 12
Cases Controls
problematic issues and are a challenge in a high percentage
of patients, even until adulthood that significantly altered
their QoL; this was especially the cases for fecal incontinence.
Therefore, a long-term follow-up by a multidisciplinary team
including pediatric surgeons, gastroenterologists, and dieti-
cians, as well as psychologists is essential and recommended.
In some instances, once the troubles persist into adolescent
or even into adulthood, a close cooperation with an adult
team specialized in fecal continence problems is recom-
mended. In our series, the patient who recently benefited
from a sacral nerve stimulation device implantation was
operated by our pediatric surgical team together with an
adult team before he was entrusted to the adult team.23,24
We believe this is an important transition for all cases now.
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