Original article

Long-term functional outcomes and quality of life in patients

with Hirschsprung’s disease
R. J. Meinds1 , A. F. W. van der Steeg3,4 , C. E. J. Sloots5 , M. J. Witvliet6 , I. de Blaauw7 ,
W. G. van Gemert8 , M. Trzpis2 and P. M. A. Broens1,2
1
Division of Paediatric Surgery and 2 Anorectal Physiology Laboratory, Department of Surgery, University of Groningen, University Medical Centre
Groningen, Groningen, 3 Department of Paediatric Surgery, Emma Children’s Hospital, Academic Medical Centre and VU University Medical Centre,
Amsterdam, 4 Centre of Research on Psychology in Somatic Diseases, Tilburg University, Tilburg, 5 Department of Paediatric Surgery, Erasmus Medical
Centre, Sophia Children’s Hospital, Rotterdam, 6 Department of Paediatric Surgery, Wilhelmina Children’s Hospital, University Medical Centre

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Utrecht, Utrecht, 7 Division of Paediatric Surgery, Department of Surgery, Radboudumc–Amalia Children’s Hospital, Nijmegen, and 8 Department of
Paediatric Surgery, University Medical Centre Maastricht, University of Maastricht, Maastricht, the Netherlands
Correspondence to: Mr R. J. Meinds, Division of Paediatric Surgery, Department of Surgery, University of Groningen, University Medical Centre
Groningen, Hanzeplein 1, PO Box 30 001, 9700 RB Groningen, the Netherlands (e-mail: [email protected])

Background: It is unclear whether functional outcomes improve or deteriorate with age following
surgery for Hirschsprung’s disease. The aim of this cross-sectional study was to determine the long-term
functional outcomes and quality of life (QoL) in patients with Hirschsprung’s disease.
Methods: Patients with pathologically proven Hirschsprung’s disease older than 7 years were included.
Patients with a permanent stoma or intellectual disability were excluded. Functional outcomes were
assessed according to the Rome IV criteria using the Defaecation and Faecal Continence questionnaire.
QoL was assessed by means of the Child Health Questionnaire Child Form 87 or World Health
Organization Quality of Life questionnaire 100. Reference data from healthy controls were available
for comparison.
Results: Of 619 patients invited, 346 (55⋅9 per cent) responded, with a median age of 18 (range 8–45)
years. The prevalence of constipation was comparable in paediatric and adult patients (both 22⋅0 per cent),
and in patients and controls. Compared with controls, adults with Hirschsprung’s disease significantly
more often experienced straining (50⋅3 versus 36⋅1 per cent; P = 0⋅011) and incomplete evacuation (47⋅4
versus 27⋅2 per cent; P < 0⋅001). The prevalence of faecal incontinence, most commonly soiling, was lower
in adults than children with Hirschsprung’s disease (16⋅8 versus 37⋅6 per cent; P < 0⋅001), but remained
higher than in controls (16⋅8 versus 6⋅1 per cent; P = 0⋅003). Patients with poor functional outcomes scored
significantly lower in several QoL domains.
Conclusion: This study has shown that functional outcomes are better in adults than children, but
symptoms of constipation and soiling persist in a substantial group of adults with Hirschsprung’s disease.
The persistence of defaecation problems is an indication that continuous care is necessary in this specific
group of patients.
Presented to the 19th European Paediatric Surgeons’ Association Annual Congress, Paris, France, June 2018

Paper accepted 28 October 2018

Published online 17 January 2019 in Wiley Online Library (www.bjs.co.uk). DOI: 10.1002/bjs.11059

Introduction surgery, defaecation disorders, such as constipation or

faecal incontinence, can persist1 – 11 .
Hirschsprung’s disease (HD) is a congenital absence of gan- It has been postulated that functional outcomes
glion cells of the distal bowel that in most instances presents improve as patients grow older, especially after reach-
with severe functional obstruction shortly after birth. Fol- ing adolescence5 – 7 . Other studies, however, have drawn
lowing diagnosis, resection is usually performed to remove attention to the fact that long-term outcomes of HD in
the aganglionic bowel and to restore continuity. Although adulthood are far from satisfactory2 – 4 . Indeed, one study3
many patients may attain normal bowel function following found that defaecation problems actually deteriorated

© 2019 The Authors. BJS published by John Wiley & Sons Ltd on behalf of BJS Society Ltd. BJS 2019; 106: 499–507
This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in
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500 R. J. Meinds, A. F. W. van der Steeg, C. E. J. Sloots, M. J. Witvliet, I. de Blaauw et al.

after the patients reached adulthood. Unfortunately, a lack children, these were the Paediatric Defaecation and Fae-
of data on healthy controls hinders interpretation of the cal Continence (P-DeFeC) questionnaire16 and the Child
majority of these studies. Health Questionnaire Child Form 87 (CHQ-CF87)17 .
Persistent defaecation disorders, such as constipation Adults received the Defaecation and Faecal Continence
and faecal incontinence, can potentially have a negative (DeFeC) questionnaire16 and the WHO Quality of Life
influence on quality of life (QoL)12,13 . A distinction is 100 (WHOQOL-100) questionnaire18 .
often made between generic QoL and health-related QoL,
the latter focusing primarily on aspects of life that are
influenced directly by an individual’s health. In patients Assessment of functional outcomes
with HD, the relationship between functional complaints Functional outcomes were assessed using patients’ answers

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and QoL has been studied previously, but these studies to the P-DeFeC and DeFeC questionnaires, which allowed
were often performed with health-related QoL question- the authors to score the Rome IV criteria, and assess the use
naires and only rarely were generic QoL questionnaires of therapies for constipation and faecal incontinence.
used14 . Moreover, it remains unclear how functional out- Constipation was defined by the Rome IV criteria for
comes continue to influence QoL after the transition into functional constipation19 . To meet these criteria, patients
adulthood14 . should have at least two of the following symptoms: strain-
The primary aim of this study was to investigate the ing, hard or lumpy stools, incomplete evacuation, anorectal
long-term functional outcomes in different age groups and obstruction, use of manual manoeuvres to facilitate defae-
to compare them with data from matched controls. Sec- cation, or fewer than three bowel movements a week. Add-
ondary aims were to identify factors associated with poor itionally, loose stools should rarely be present without the
outcomes and to evaluate the influence of poor functional use of laxatives. The individual symptoms incorporated in
outcomes on QoL using generic QoL questionnaires. the Rome IV criteria were also assessed for functional con-
stipation, which had to occur at least several times a month.
Faecal incontinence was defined by the Rome IV criteria
Methods
for faecal incontinence as recurrent uncontrolled passage
This study had the approval code METc 2013/226 and was of faecal material, including soiling, at least several times a
performed in compliance with the requirements of the local month20 . Several subtypes of faecal incontinence were also
medical ethics review board. Written informed consent was assessed, such as soiling (loss of small amounts of faeces),
obtained from each participant. urge incontinence (inability to reach the toilet in time),
The medical records of all known patients diagnosed with incontinence to solid stool (loss of large amounts of solid
HD in all six paediatric surgical centres in the Nether- faeces without having felt urge) and incontinence to liquid
lands were reviewed. Inclusion criteria were pathologically stool (loss of watery stools or diarrhoea).
proven HD and a minimum age of 8 years. The following By means of the questionnaire, an evaluation was under-
variables were collected from the records: co-morbidities, taken of the use of laxatives and bowel management at least
length of aganglionosis, surgical treatment, episodes of several times a month as therapy for constipation or faecal
enterocolitis, surgical complications and additional surgical incontinence.
interventions. Enterocolitis was defined as the presence of Reference data for the P-DeFeC and DeFeC question-
symptoms such as abdominal distension, diarrhoea, bloody naires were available from studies that had been performed
stools and/or fever with the intention to treat as such15 . previously in the general Dutch population. This produced
Surgical complications were defined as complications that 1103 healthy children and adults who did not have a history
occurred within 30 days and were the direct result of the of bowel surgery or somatic diseases that could influence
initial surgical intervention (such as anastomotic leakage, their bowels21,22 .
wound infection and adhesions).
After the exclusion of patients who were ineligible to par-
Assessment of quality of life
ticipate (deceased, living abroad, had a permanent stoma
or an intellectual disability), the remaining patients were The CHQ-CF8717 was used to assess QoL in children aged
invited to participate in the study. In the case of chil- 8–17 years. This is a generic QoL questionnaire with 87
dren aged between 8 and 17 years, parents or caregivers items that are scored on a four- to six-point Likert scale.
were asked to participate together with the patients, or on Following completion, ten multi-item domains and two
their behalf. On agreeing to participate, patients received single-item questions were calculated and converted to a
questionnaires on anorectal functioning and QoL. For 0–100-point continuum, where a higher score indicates

© 2019 The Authors. BJS published by John Wiley & Sons Ltd www.bjs.co.uk BJS 2019; 106: 499–507
on behalf of BJS Society Ltd.
Functional outcomes and quality of life in patients with Hirschsprung’s disease 501

Table 1 Patient characteristics and dropout analysis

Non-responders (n = 273) Responders (n = 346) P†

Age (years)* 22 (8–50) 18 (8–45) 0⋅004‡

Sex ratio (M : F) 224 : 49 274 : 72 0⋅373
Co-morbidities 26 (9⋅5) 33 (9⋅5) 1⋅000
Length of aganglionosis 0⋅804
Ultrashort 5 (1⋅8) 10 (2⋅9)
Rectosigmoid 222 (81⋅3) 282 (81⋅5)
Long segment 23 (8⋅4) 29 (8⋅4)
Total colonic 23 (8⋅4) 25 (7⋅2)
Preoperative enterocolitis 30 (11⋅0) 46 (13⋅3) 0⋅395

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Primary surgical treatment 0⋅443
Surgical reconstruction 265 (97⋅1) 337 (97⋅4)
Sphincterectomy 3 (1⋅1) 4 (1⋅2)
Other 2 (0⋅7) 0 (0⋅0)
None/conservative 3 (1⋅1) 5 (1⋅4)
Surgical reconstruction 0⋅166
Duhamel 149 (56⋅2) 210 (62⋅3)
Soave 1 (0⋅4) 1 (0⋅3)
Rehbein 80 (30⋅2) 73 (21⋅7)
Swenson 0 (0) 1 (0⋅3)
Transanal endorectal pull-through 35 (13⋅2) 52 (15⋅4)
Postoperative complication 26 of 270 (9⋅6) 36 of 341 (10⋅6) 0⋅706
Postoperative enterocolitis 24 of 270 (8⋅9) 47 of 341 (13⋅8) 0⋅061
Redo pull-through 15 of 270 (5⋅6) 23 of 341 (6⋅7) 0⋅546

Values in parentheses are percentages unless indicated otherwise; *values are median (range). †χ2 test, except ‡Mann–Whitney U test.

better QoL. The following domains were assessed for this tended towards significance (P < 0⋅100) in the univariable
study: behaviour, mental health, self-esteem and general analyses. Two-sided P < 0⋅050 was considered statistically
health. significant. Data were analysed with SPSS® version 23.0
The WHOQOL-10018 was used to assess QoL in adults. for Windows® (IBM, Armonk, New York, USA).
The WHOQOL-100 consists of 100 items covering six
domains and a general evaluative facet (overall QoL and Results
general health). The items are scored on a five-point Likert
scale. Calculated domain scores range between 4 and 20 Based on the inclusion criteria, 830 patients were identified
points; a higher domain score indicates better QoL. The as eligible for the study, of whom 211 were subsequently
following domains were analysed in the present study: excluded: patients who had died (43), who lived abroad (47),
overall QoL, physical health, psychological health and whose addresses were not available (10) or who were unable
social relationships. to complete one of the questionnaires because of a perm-
Reference data for the healthy Dutch population were anent stoma (25) or intellectual disability (such as Down
available for both the CHQ-CF8723 and WHOQOL-100 syndrome, 86). The most common reasons for a perma-
questionnaires (courtesy of J. de Vries, University of nent stoma were postoperative complications (7), persistent
Tilburg)24 . constipation (5) and severe intellectual disability (3). A total
of 619 patients received an invitation to participate in the
study (Fig. S1, supporting information).
Statistical analysis
Following invitation, 346 patients and their parents
Proportions are reported as prevalence percentages with or caregivers (55⋅9 per cent) agreed to participate and
95 per cent confidence intervals. Quantitative variables are completed the questionnaires (Table 1). There were 173
expressed as mean(s.d.) or median (range). Statistical tests children aged 8–17 years and 173 adults with HD.
used were χ2 , Mann–Whitney U and t tests. Univariable Additional patient characteristics are shown in Table 1.
and multivariable logistic regression analyses were used to A dropout analysis showed that the only significant dif-
test the association between potential risk factors and the ference between non-responders and responders was in
likelihood of faecal incontinence, with results reported as median age: 22 (range 8–50) versus 18 (8–45) years respec-
odds ratios (ORs) with 95 per cent confidence intervals. tively (P = 0⋅004). The 346 patients who responded were
The multivariable analysis was built using variables that randomly matched 1 : 1 with controls on the basis of sex

© 2019 The Authors. BJS published by John Wiley & Sons Ltd www.bjs.co.uk BJS 2019; 106: 499–507
on behalf of BJS Society Ltd.
502 R. J. Meinds, A. F. W. van der Steeg, C. E. J. Sloots, M. J. Witvliet, I. de Blaauw et al.

Table 2 Functional outcomes in children and adults

Children (8–17 years) Adults (≥ 18 years)

Patients Controls Patients Controls P (patients:

(n = 173) (n = 147) P† (n = 173) (n = 147) P† children versus adults)†

Constipation
Prevalence (Rome IV) 38 (22⋅0) 21 (14⋅3) 0⋅077 38 (22⋅0) 28 (19⋅0) 0⋅520 1⋅000
Symptoms
Straining 64 (37⋅0) 32 (21⋅8) 0⋅003 87 (50⋅3) 53 (36⋅1) 0⋅011 0⋅013
Lumpy or hard stools 5 (2⋅9) 14 (9⋅5) 0⋅012 11 (6⋅4) 9 (6⋅1) 0⋅931 0⋅125

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Incomplete evacuation 68 (39⋅3) 13 (8⋅8) < 0⋅001 82 (47⋅4) 40 (27⋅2) < 0⋅001 0⋅129
Anorectal obstruction 39 (22⋅5) 17 (11⋅6) 0⋅010 44 (25⋅4) 25 (17⋅0) 0⋅068 0⋅529
Manual manoeuvres 0 (0) 3 (2⋅0) 0⋅059 10 (5⋅8) 7 (4⋅8) 0⋅686 0⋅001
< 3 bowel movements per week 12 (6⋅9) 12 (8⋅2) 0⋅678 18 (10⋅4) 19 (12⋅9) 0⋅482 0⋅252
Laxative use 53 (30⋅6) 6 (4⋅1) < 0⋅001 9 (5⋅2) 6 (4⋅1) 0⋅636 < 0⋅001
Bowel management for constipation 31 (17⋅9) 1 (0⋅7) < 0⋅001 14 (8⋅1) 1 (0⋅7) 0⋅002 0⋅007
Faecal incontinence
Prevalence (Rome IV) 65 (37⋅6) 9 (6⋅1) < 0⋅001 29 (16⋅8) 9 (6⋅1) 0⋅003 < 0⋅001
Subtypes*
Soiling 60 (34⋅7) 6 (4⋅1) < 0⋅001 29 (16⋅8) 6 (4⋅1) < 0⋅001 < 0⋅001
Urge incontinence 7 (4⋅0) 2 (1⋅4) 0⋅148 2 (1⋅2) 3 (2⋅0) 0⋅525 0⋅091
Incontinence to solid stool 12 (6⋅9) 3 (2⋅0) 0⋅039 2 (1⋅2) 3 (2⋅0) 0⋅525 0⋅006
Incontinence to liquid stool 15 (8⋅7) 2 (1⋅4) 0⋅004 5 (2⋅9) 5 (3⋅4) 0⋅793 0⋅021
Bowel management for faecal incontinence 19 (11⋅0) 1 (0⋅7) < 0⋅001 3 (1⋅7) 0 (0⋅0) 0⋅109 0⋅017

Values in parentheses are percentages. *Respondents often had various types of faecal incontinence. †χ2 test.

Table 3 Prevalence and likelihood of faecal incontinence

Likelihood of faecal incontinence‡

Prevalence of Univariable analysis Multivariable analysis

Total no. faecal
of patients* incontinence (%) P† Odds ratio P Odds ratio P

Overall 346 27⋅2 (22⋅5, 31⋅9)

Sex 0⋅175
Men 274 (79⋅2) 28⋅8 (23⋅4, 34⋅2) 1⋅00 (reference)
Women 72 (20⋅8) 21 (11, 30) 0⋅65 (0⋅35, 1⋅21) 0⋅177
Patient group < 0⋅001
Children (8–17 years) 173 (50⋅0) 37⋅6 (30⋅3, 44⋅9) 1⋅00 (reference) 1⋅00 (reference)
Adults (≥ 18 years) 173 (50⋅0) 16⋅8 (11⋅1, 22⋅4) 0⋅33 (0⋅20, 0⋅55) < 0⋅001 0⋅35 (0⋅21, 0⋅58) < 0⋅001
Length of aganglionosis 0⋅482
Ultrashort 10 (2⋅9) 20 (–10, 50) 0⋅70 (0⋅15, 3⋅38) 0⋅660
Rectosigmoid 282 (81⋅5) 26⋅2 (21⋅1, 31⋅4) 1⋅00 (reference)
Long segment 29 (8⋅4) 28 (10, 45) 1⋅07 (0⋅45, 2⋅52) 0⋅876
Total colonic 25 (7⋅2) 40 (19, 61) 1⋅87 (0⋅81, 4⋅35) 0⋅144
Postoperative complication 0⋅777
No 305 (89⋅4) 27⋅2 (22⋅2, 32⋅2) 1⋅00 (reference)
Yes 36 (10⋅6) 25 (10, 40) 0⋅89 (0⋅40, 1⋅97) 0⋅769
Redo pull-through 0⋅001
No 323 (93⋅4) 25⋅1 (20⋅3, 29⋅8) 1⋅00 (reference) 1⋅00 (reference)
Yes 23 (6⋅6) 57 (35, 78) 3⋅88 (1⋅64, 9⋅20) 0⋅002 3⋅54 (1⋅46, 8⋅62) 0⋅005

Values in parentheses are 95 per cent confidence intervals unless indicated otherwise; *values in parentheses are percentages. †χ2 test. ‡Logistic regression
analysis; variables with P < 0⋅100 in univariable analysis were subsequently included in the multivariable analysis.

© 2019 The Authors. BJS published by John Wiley & Sons Ltd www.bjs.co.uk BJS 2019; 106: 499–507
on behalf of BJS Society Ltd.
Functional outcomes and quality of life in patients with Hirschsprung’s disease 503

Hirschsprung’s disease (n = 150) Functional outcomes in children

Reference data (n = 1041)
The prevalence of constipation was comparable between
100 † † children with HD and their controls (22⋅0 versus 14⋅3 per
*
cent respectively). However, the patients had symptoms
80 such as straining, incomplete evacuation and anorectal
obstruction significantly more often (Table 2). The patients
Mean score

60 also used laxatives (30⋅6 versus 4⋅1 per cent; P < 0⋅001) and
bowel management (17⋅9 versus 0⋅7 per cent; P < 0⋅001)
40 to treat constipation significantly more frequently than
controls.

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20 The overall prevalence of faecal incontinence was sig-
nificantly higher in children with HD than their controls
0 (37⋅6 versus 6⋅1 per cent; P < 0⋅001). The most common
r

th

th
ou

subtype of faecal incontinence among the patients was

al

al
vi

te
he

he
ha

es

soiling (34⋅7 per cent), followed by incontinence to liquid

l

al
ta
Be

lf-

er
en

Se

en
M

(8⋅7 per cent) and solid (6⋅9 per cent) stool, all of which
G

a Children (8–17 years) were significantly more prevalent than in controls (Table 2).
Bowel management to treat faecal incontinence was used
Hirschsprung’s disease (n = 160) in 11⋅0 per cent of patients, but only 0⋅7 per cent of
Reference data (n = 198) controls (P < 0⋅001).
20
* † *
*

Functional outcomes in adults

15
The prevalence of constipation was comparable between
Mean score

adult patients and adult controls (22⋅0 versus 19⋅0 per

10
cent; P = 0⋅520). The adults with HD had symptoms such
as straining and incomplete evacuation significantly more
5 often than their controls (Table 2). Using laxatives was not
more common among patients compared with their control
group, whereas they more often used bowel management
0 to treat constipation (8⋅1 versus 0⋅7 per cent; P = 0⋅002).
oL

th

lth

ps
al

The overall prevalence of faecal incontinence was

ea

hi
Q

he

ns
ll

lh
ra

tio
al

ca

higher in adult patients compared with their controls (16⋅8

ve

ic

la
gi
ys
O

re
lo
Ph

ho

versus 6⋅1 per cent; P = 0⋅003). This was mainly the result
al
ci
yc

So
Ps

of a significantly higher prevalence of soiling (16⋅8 versus

b Adults (≥ 18 years) 4⋅1 per cent; P < 0⋅001), which was the only subtype of
faecal incontinence that showed a significant difference
Fig. 1Quality-of-life scores of children and adults with
(Table 2).
Hirschsprung’s disease compared with reference data from
healthy controls. a Child Health Questionnaire Child Form
(CHQ-CF87) scores in children; b WHO Quality of Life
Comparison of functional outcomes in children
(WHOQOL-100) score in adults. Values are mean with 95 per
cent confidence interval. QoL, quality of life. *P < 0⋅050, and adult patients
†P < 0⋅001 (t test) The prevalence of constipation was the same in children
and adults with HD (both 22⋅0 per cent). Nevertheless,
adult patients reported straining and manual manoeuvres
and age. Because of the high prevalence of male patients, when defaecating more often than children (Table 2). For
it was not possible to match 52 patients with appropriate the treatment of constipation, children used laxatives (30⋅6
controls. This did not result in significant differences in versus 5⋅2 per cent; P < 0⋅001) and bowel management (17⋅9
sex or age between the group of 346 patients with HD and versus 8⋅1 per cent; P = 0⋅007) significantly more often than
the group of 294 controls. adult patients.

© 2019 The Authors. BJS published by John Wiley & Sons Ltd www.bjs.co.uk BJS 2019; 106: 499–507
on behalf of BJS Society Ltd.
504 R. J. Meinds, A. F. W. van der Steeg, C. E. J. Sloots, M. J. Witvliet, I. de Blaauw et al.

Table 4 Comparison of functional outcomes and quality of life in patients with Hirschsprung’s disease

Constipation (Rome IV criteria) Faecal incontinence (Rome IV criteria)

No Yes P* No Yes P*

CHQ-CF87 score (n = 150)

Behaviour 82 (21–99) 76 (46–97) 0⋅010 83 (61–99) 77 (21–98) 0⋅010
Mental health 81 (50–100) 76 (42–97) 0⋅021 81 (42–100) 77 (50–100) 0⋅056
Self-esteem 77 (30–100) 73 (41–100) 0⋅013 75 (41–100) 77 (30–100) 0⋅877
General health 80 (21–100) 69 (19–99) 0⋅004 81 (19–100) 74 (26–100) 0⋅017
WHOQOL-100 score (n = 160)
Overall QoL 16 (10–20) 16 (7–20) 0⋅055 16 (9–20) 16 (7–20) 0⋅319
Physical health 16 (8–20) 15 (7–20) 0⋅077 16 (10–20) 15 (7–20) 0⋅018

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Psychological health 16 (10–20) 15 (10–19) 0⋅025 16 (10–20) 15 (10–20) 0⋅204
Social relationships 16 (9–20) 15 (9–19) 0⋅079 16 (9–20) 16 (9–20) 0⋅141

Values are median (range). CHQ-CF87, Child Health Questionnaire Child Form; WHOQOL-100, WHO Quality of Life questionnaire. QoL, quality
of life. *Mann–Whitney U test.

Among patients with HD, the overall prevalence of fae- (mean(s.d.) 80(13) versus 85(11); P < 0⋅001), self-esteem
cal incontinence was lower in adults than children (16⋅8 (76(13) versus 79(13); P = 0⋅008) and general health (73(20)
versus 37⋅6 per cent; P < 0⋅001). The subtypes of faecal versus 82(14); P < 0⋅001) (Fig. 1a). Among children with
incontinence, such as soiling, incontinence to solid stool HD, the constipated group had significantly lower median
and incontinence to liquid stool, were all significantly scores on all four domains tested than the group without
less prevalent in adults (Table 2). Only 1⋅7 per cent of constipation (Table 4). Children with faecal incontinence
the adult patients required bowel management for fae- alone had significantly lower median scores for the domains
cal incontinence, compared with 11⋅0 per cent of children behaviour (83 (range 61–99) versus 77 (21–98); P = 0⋅010)
(P = 0⋅017). and general health (81 (19–100) versus 74 (26–100;
P = 0⋅017) than those without faecal incontinence.
In an analysis of mean QoL domain scores in adult
Factors associated with faecal incontinence
patients compared with their reference data set, adult
In univariable analyses, sex, length of aganglionosis patients had significantly higher scores for overall QoL
and postoperative complication were not significantly (mean(s.d.) 16(3) versus 15(3); P = 0⋅001), physical health
associated with faecal incontinence, whereas age group (16(3) versus 15(3); P = 0⋅002), psychological health (16(2)
and redo pull-through procedures were (Table 3). The versus 15(2); P < 0⋅001) and social relationships (16(3) versus
multivariable analysis showed that adult patients were 15(3); P = 0⋅002) (Fig. 1b). The only significant difference
significantly less likely to report faecal incontinence than between constipated and non-constipated adult patients
children with HD (OR 0⋅35, 95 per cent c.i. 0⋅21 to was in the domain psychological health (median 16 (range
0⋅58). Patients who required a redo pull-through were 10–20) versus 15 (10–19); P = 0⋅025). The only significant
significantly more likely to have faecal incontinence difference between faecally continent and incontinent adult
than patients who had only undergone one procedure patients was in the domain of physical health (16 (10–20)
(OR 3⋅54, 1⋅46 to 8⋅62). There was no significant inter- versus 15 (7–20); P = 0⋅018).
action between the variables used in the multivariable
analysis. Discussion

This nationwide study showed that functional outcomes

Comparison of functional outcomes and quality
were better in adults than children with HD, but that defae-
of life
cation disorders persisted in a substantial group of adults.
The QoL questionnaires were completed by 150 children Patients who required a redo pull-through procedure were
and 160 adults with HD; 36 patients omitted the QoL ques- more likely to have faecal incontinence. In the present
tionnaire after completing the questionnaire on anorectal cohort, defaecation disorders, especially constipation,
functioning. negatively influenced QoL domains, with a more promi-
Comparing mean QoL domain scores of children with nent effect in children than adults.
HD with reference data from the general population, Interestingly, the prevalence of constipation in both chil-
the patients had significantly lower scores for behaviour dren and adults with HD was comparable to that in their

© 2019 The Authors. BJS published by John Wiley & Sons Ltd www.bjs.co.uk BJS 2019; 106: 499–507
on behalf of BJS Society Ltd.
Functional outcomes and quality of life in patients with Hirschsprung’s disease 505

respective control groups. These findings warrant reflec- pull-through procedures were complicated by a relatively
tion. The true prevalence of constipation in patients with high rate of soiling and faecal incontinence30 . Important
HD may be masked by the more frequent use of laxatives to note, however, is the finding that other functional symp-
and bowel management than in controls. If true, this could toms, such as constipation and abdominal pain, improved
also mean that the prevalence of constipation may decrease following the redo procedure30 . It remains unclear to what
as the patients grow older, because the use of laxatives and extent the redo procedure itself contributed to the impaired
rectal irrigation was significantly lower in adults than in faecal continence, because it may already have been worse
children. As indicated by the increased frequency of symp- in these patients before the redo procedure. The authors
toms found in this study, it may be that both children and merely conclude that a redo procedure may ultimately
adults with HD experienced more severe forms of consti- be necessary in some patients, but that one should be

Downloaded from https://academic.oup.com/bjs/article/106/4/499/6121040 by guest on 13 September 2022

pation than controls. cautious about promising favourable functional outcomes,
In contrast to constipation, the prevalence of faecal because the prevalence of faecal incontinence remains high
incontinence was significantly higher in patients with HD after redo procedures. Given the heterogeneity of surgical
than controls. It is important to note, however, that chil- techniques used, more comprehensive studies are required
dren more often had severe subtypes of faecal incontinence, to analyse accurately the possible association between
such as incontinence to solid and liquid stool, whereas long-term outcomes and different types of pull-through
adult patients often reported soiling only. This means that procedure.
both the prevalence and severity of faecal incontinence may In terms of QoL, the present results showed that children
decrease with age, although adults with HD did retain a with HD had significantly lower QoL domain scores com-
significantly higher prevalence of soiling than controls. In pared with the reference data. These differences may be
contrast to the present results, a recent study6 concluded
explained partially by poor functional outcomes, because
that faecal incontinence would eventually diminish to a
constipation and faecal incontinence negatively influenced
prevalence not significantly different from that of healthy
several QoL domains. In contrast to children, adult patients
controls, even though soiling persisted in well over 40
scored better on all four domains tested compared with
per cent in their adult subgroup. It therefore seems that
their respective reference data sets. This could be the result
faecal continence may improve with age, but that prob-
of improved functional outcomes in adult patients com-
lems persist well into adulthood. There are various reasons
pared with children. A more plausible explanation might
for this. Faecal incontinence in these patients may result
be that adults develop better coping strategies to deal with
from damage to the anal sphincter during reconstructive
their complaints31,32 . By way of illustration, adults may
surgery25 , depending on the type and quality of the initial
have more options to adapt their lives to accommodate
surgery. With regard to the type of surgery, there is cur-
rently little evidence that substantiates the decision to opt for any defaecation disorders, whereas children are often
for one technique over another26 – 28 . The authors believe bound by fixed schedules, such as school and after-school
that further analyses, such as anorectal manometry or anal activities.
sphincter electromyography, should be performed to assess The strength of this study was the large number of par-
the differences between the techniques, especially between ticipants, from all six paediatric surgery centres in the
the transanal endorectal pull-through and Duhamel oper- Netherlands, and the relatively high response rate of 55⋅9
ations, which currently are the two most commonly per- per cent. One limitation was the significant age differ-
formed procedures. Such investigations might also give ence between included patients and non-respondents, even
an objective prognosis regarding the functional outcomes though the remaining variables all proved to be stat-
in individual patients. In addition, impaired continence istically non-significant. The difference in age between
may result from more severe constipation in patients with respondents and non-respondents was most likely the
HD21 , which could be the result of a persistently absent result of the high response rate among children with HD,
rectoanal inhibitory reflex, stenosis of the anal sphincter supported by their parents, who were found to be more
following surgery or lack of pelvic floor coordination29 . motivated to participate than adult patients. An attempt
The absence of a rectal reservoir following surgery and sub- was made to overcome this possible inclusion bias by per-
sequent increased defaecation frequency may further con- forming separate analyses in adults and children, and mak-
tribute to impaired faecal continence. In the present study, ing age- and sex-matched comparisons. Another limitation
patients who required a redo pull-through procedure were may be the cross-sectional design of this study. A longi-
significantly more likely have faecal incontinence. A recent tudinal design would have been preferable to analyse the
study30 indeed showed that short-term outcomes after redo influence of ageing on functional outcomes.

© 2019 The Authors. BJS published by John Wiley & Sons Ltd www.bjs.co.uk BJS 2019; 106: 499–507
on behalf of BJS Society Ltd.
506 R. J. Meinds, A. F. W. van der Steeg, C. E. J. Sloots, M. J. Witvliet, I. de Blaauw et al.

The results of this nationwide study showed that func- 7 Catto-Smith AG, Trajanovska M, Taylor RG. Long-term
tional outcomes were better in adults than children with continence after surgery for Hirschsprung’s disease.
HD, although symptoms of constipation and soiling per- J Gastroenterol Hepatol 2007; 22: 2273–2282.
sisted in a substantial group of adults. One factor associated 8 Bjørnland K, Pakarinen MP, Stenstrøm P, Stensrud KJ,
Neuvonen M, Granström AL et al.; Nordic Pediatric
with poor functional outcomes was a redo pull-through
Surgery Study Consortium. A Nordic multicenter survey of
procedure, following which patients were significantly long-term bowel function after transanal endorectal
more likely have faecal incontinence. Poor functional out- pull-through in 200 patients with rectosigmoid
comes negatively influenced QoL in children, whereas this Hirschsprung disease. J Pediatr Surg 2017; 52: 1458–1464.
influence diminished partially upon reaching adulthood, 9 Kim AC, Langer JC, Pastor AC, Zhang L, Sloots CE,
indicating better coping strategies in adults. Persistent Hamilton NA et al. Endorectal pull-through for

Downloaded from https://academic.oup.com/bjs/article/106/4/499/6121040 by guest on 13 September 2022

symptoms of constipation and soiling indicate the need Hirschsprung’s disease – a multicenter, long-term
for counselling and transitional care in a specific group of comparison of results: transanal vs transabdominal approach.
patients. J Pediatr Surg 2010; 45: 1213–1220.
10 Rintala RJ, Pakarinen MP. Long-term outcomes of Hirsch-
sprung’s disease. Semin Pediatr Surg 2012; 21: 336–343.
11 Mills JL, Konkin DE, Milner R, Penner JG, Langer M,
Acknowledgements
Webber EM. Long-term bowel function and quality of life
The authors thank the employees of RoQua, particularly in children with Hirschsprung’s disease. J Pediatr Surg 2008;
43: 899–905.
I. A. M. ten Vaarwerk and E. Visser, for their help in pro-
12 Bartlett L, Nowak M, Ho YH. Impact of fecal incontinence
cessing the data from the digital questionnaire and prepar-
on quality of life. World J Gastroenterol 2009; 15:
ing the database; T. van Wulfften Palthe for correcting the 3276–3282.
English manuscript; and J. de Vries for supplying 13 Belsey J, Greenfield S, Candy D, Geraint M. Systematic
the appropriate reference data for the WHOQOL-100 review: impact of constipation on quality of life in adults and
questionnaire. children. Aliment Pharmacol Ther 2010; 31: 938–949.
14 Hartman EE, Oort FJ, Aronson DC, Sprangers MA. Quality
of life and disease-specific functioning of patients with
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Supporting information
Additional supporting information can be found online in the Supporting Information section at the end of the
article.

© 2019 The Authors. BJS published by John Wiley & Sons Ltd www.bjs.co.uk BJS 2019; 106: 499–507
on behalf of BJS Society Ltd.
 European Colorectal Congress
LORECTAL
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2022

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28 November – 1 December 2022, St.Gallen, Switzerland
28

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Monday, 28 November 2022 Tuesday, 29 November 2022 Wednesday, 30 November 2022

09.50 9.00 9.00

Opening and welcome CONSULTANT‘S CORNER Advanced risk stratification in colorectal
Jochen Lange, St.Gallen, CH Michel Adamina, Winterthur, CH cancer – choosing wisely surgery and
adjuvant therapy
10.00 10.30 Philip Quirke, Leeds, UK
It is leaking! Approaches to salvaging an COFFEE BREAK
anastomosis 09.30
Willem Bemelman, Amsterdam, NL 11.00 Predictors for Postoperative Complications
SATELLITE SYMPOSIUM and Mortality
10.30 Ronan O‘Connell, Dublin, IE
Predictive and diagnostic markers
of anastomotic leak 10.00
Andre D‘Hoore, Leuven, BE 11.45 Segmental colectomy versus extended
Trends in colorectal oncology and colectomy for complex cancer
11.00 clinical insights for the near future Quentin Denost, Bordeaux, FR
SATELLITE SYMPOSIUM Rob Glynne-Jones, London, UK
10.30
12.15 COFFEE BREAK
LUNCH
11.45 11.00
Of microbes and men – the unspoken 13.45 Incidental cancer in polyp - completion
story of anastomotic leakage VIDEO SESSION surgery or endoscopy treatment alone?
James Kinross, London, UK Laura Beyer-Berjot, Marseille, FR
14.15
12.15 SATELLITE SYMPOSIUM 11.30
LUNCH SATELLITE SYMPOSIUM
13.45
Operative techniques to reduce 15.00
anastomotic recurrence in Crohn’s disease COFFEE BREAK 12.00
Laura Hancock, Manchester, UK Less is more – pushing the boundaries
15.30 of full-thickness rectal resection
14.15 The unsolved issue of TME: Xavier Serra-Aracil, Barcelona, ES
Innovative approaches in the treatment open, robotic, transanal, or laparoscopic –
of complex Crohn Diseases perianal fistula shining light on evidence and practice 12.30
Christianne Buskens, Amsterdam, NL Des Winter, Dublin, IE LUNCH
Jim Khan, London, UK
14.45 Brendan Moran, Basingstoke, UK 14.00
To divert or not to divert in Crohn surgery – Management of intestinal
technical aspects and patient factors 16.30 neuroendocrine neoplasia
Pär Myrelid, Linköping, SE SATELLITE SYMPOSIUM Frédéric Ris, Geneva, CH
15.15 14.30
COFFEE BREAK Poster Presentation & Best Poster Award
Michel Adamina, Winterthur, CH
15.45 17.15
Appendiceal neoplasia – when to opt for a Lars Pahlman lecture 15.00
minimal approach, when and how to go for Søren Laurberg, Aarhus, DK SATELLITE SYMPOSIUM
a maximal treatment
Tom Cecil, Basingstoke, Hampshire, UK

16.15 15.45
SATELLITE SYMPOSIUM COFFEE BREAK

16.15
Reoperative pelvic floor surgery –
17.00 er 2022 dealing with perineal hernia, reoperations,
Outcomes of modern induction therapies ecemb
ay, 1 D
and complex reconstructions
and Wait and Watch strategies, Hope or Hype h u rs d urgery
rectal S
T Guillaume Meurette, Nantes, FR
Antonino Spinelli, Milano, IT C o lo
class in y
Master logy Da 16.45
17.30 Procto Salvage strategies for rectal neoplasia
EAES Presidential Lecture - Use of ICG in Roel Hompes, Amsterdam, NL
colorectal surgery: beyond bowel perfusion
Salvador Morales-Conde, Sevilla, ES 17.15
Beyond TME – technique and results
of pelvic exenteration and sacrectomy
Paris Tekkis, London, UK
18.00
Get-Together with your colleagues 19.30
Industrial Exhibition FESTIVE EVENING

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