Defecation disorders in children after surgery for HD

ORIGINAL ARTICLE: GASTROENTEROLOGY

Defecation Disorders in Children After Surgery for
Hirschsprung Disease
Bruno P. Chumpitazi and Samuel Nurko
ABSTRACT
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Background and Objective: The majority of children with Hirschsprung
disease (HD) after corrective surgery (CS) develop protracted defecation
disorders (DDs) such as constipation, fecal incontinence, and/or enterocolitis. The aim of this investigation was to determine the diagnoses, therapies,
and long-term clinical outcomes using a systematic algorithm to address
protracted DD in children with HD after CS.
Methods: Retrospective review of children with HD after CS cared for using
a systematic algorithm at a tertiary care center. Potential anatomic etiologies
were evaluated for first. Clinical outcome was categorized into 4 groups
based on symptom severity, time interval from last enterocolitis episode,
laxative usage, and/or rectal therapies at the time of last follow-up.
Results: Fifty-seven children were identified, of whom 51 (89.5%) had
obstructive symptoms and/or enterocolitis and 6 (10.5%) had nonretentive
fecal incontinence. Nonintractable constipation responsive to laxatives was
identified in 10 (17.5%), colonic dysmotility in 4 (7.0%), nonrelaxing anal
sphincter as a primary etiology in 22 (38.6%), bacterial overgrowth in 2
(3.5%), food intolerance in 2 (3.5%), and rapid transit in 2 (3.5%). Further
surgical intervention was undertaken in 22 (38.6%), including 9 (15.8%) for
residual aganglionosis. Mean follow-up was 41.4 Æ 4.5 months. Clinical
outcomes were excellent in 16 (28.1%), good in 22 (38.6%), fair in 1 (1.8%),
and poor in 18 (31.6%). Children with enterocolitis were more likely to have
an excellent or good clinical outcome.
Conclusions: The majority of children with HD and protracted DD after CS
have a favorable long-term clinical outcome when following a systematic

algorithm.

been evolving, particularly with the introduction of laparoscopic
and transanal techniques (4). Irrespective of the surgical technique
used, up to 60% of children with HD experience a protracted
defecation disorder (DD) and/or enterocolitis after initial HD
corrective surgery (CS) (5–7). These DDs may include fecal
incontinence (5), constipation (6), at times associated with episodes
of abdominal distention and emesis (8), and/or enterocolitis (9).
Postoperative enterocolitis may lead to increased morbidity and
hospitalizations as well as increased mortality (9). Full bowel
continence and resolution of symptoms are often achieved by late
adolescence, but not in all cases (10), and there is a significant
negative effect on the child and the child’s family when dealing
with these postoperative DDs (11,12).
Given the effect and prevalence of DDs and/or enterocolitis
after HD CS in children, various diagnostic and management
strategies have been suggested to evaluate specific (eg, fecal
incontinence alone) postoperative DDs in this population (13–
15). These strategies include various radiologic, manometric,
dietary, pharmaceutical, and surgical interventions. Evaluation of
a comprehensive systematic algorithm combining strategies and
determining the ability to affect long-term clinical outcome in all
children with all postoperative DDs has not been completed,
however.
The purpose of the present study was to determine the
diagnoses, management, and long-term clinical outcomes following
a well-defined systemic algorithm incorporating a variety of diagnostic and management strategies to address DDs in children with
HD after CS presenting to a tertiary care pediatric gastroenterology
clinic.

(JPGN 2011;53: 75–79)

METHODS

H

irschsprung disease (HD), a neural crest disorder (1) characterized by aganglionosis beginning in the rectum and extending to various lengths proximally, occurs in approximately 1 in
every 5000 to 10,000 live births (2,3). Surgical management has
Received November 7, 2010; accepted January 17, 2011.
From the Center for Motility and Functional Gastrointestinal Disorders,
Children’s Hospital Boston, Boston, MA.
Address correspondence and reprint requests to Samuel Nurko, MD, MPH,
Children’s Hospital, 300 Longwood Ave, Boston, MA 02115 (e-mail:
).
(B.C. present affiliation: Texas Children’s Hospital Neurogastroenterology
and Motility Program, Baylor College of Medicine, Houston.)
The present study was supported in part by NIH grant K24-DK082792A (Dr
Nurko), the Children’s Digestive Health and Nutrition Foundation/Nestle
Nutrition Research Young Investigator Award (Dr Chumpitazi), and NIH
grant P30-DK056338 (Dr Chumpitazi).
The authors report no conflicts of interest.
Copyright # 2011 by European Society for Pediatric Gastroenterology,
Hepatology, and Nutrition and North American Society for Pediatric
Gastroenterology, Hepatology, and Nutrition
DOI: 10.1097/MPG.0b013e318212eb53

JPGN



Volume 53, Number 1, July 2011

Following institutional review board approval, medical
records of all children status post-CS for HD presenting to a
tertiary care pediatric gastroenterology clinic at a tertiary medical
center between 1998 and 2006 were reviewed. Children with a
physician’s diagnosis of constipation, fecal incontinence, and/or
enterocolitis and documented follow-up after initial evaluation
were included.
A diagnostic algorithm (Fig. 1) that first excludes potential
anatomic etiologies via physical examination and/or barium enema
was systematically followed in all children as part of the routine
clinical practice. Although a barium enema was preferred, at times
given the child’s anxiety, an examination under anesthesia was
undertaken. All of the children were tested for the presence of
Clostridium difficile toxin in the stool and treated appropriately. The
senior author (S.N.) was involved in the evaluation and management
of all of the patients. Children with anal stenosis, strictures, or a
transition zone were referred for further surgical evaluation.
Following initial evaluation, children were classified into 1
of 2 groups: obstructive symptoms (OS) (eg, constipation, straining
with defecation, abdominal distention) and/or enterocolitis, or
nonretentive fecal incontinence based on radiologic results and

75

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Chumpitazi and Nurko

JPGN

Child with
Hirschsprung’s disease
s/p corrective surgery

Stricture or
Transition zone

Surgical
evaluation

History and physical
examination
C difficile testing

Anal stenosis

Anal dilatation
and surgical
evaluation

Barium enema

No anatomic etiology

Nonretentive
Fecal Incontinence

Obstructive symptoms
and/or enterocolitis

Expand differential
diagnosis
Begin fiber and antidiarrheals

Begin laxative
regimen

FIGURE 1. Initial diagnostic and treatment algorithm for a
child with Hirschsprung disease after corrective surgery presenting with a defecation disorder and/or enterocolitis.



Volume 53, Number 1, July 2011

and were started on oral agents (Imodium, fiber supplementation) to
slow colonic transit. Children with abdominal distention and/or
recurrent enterocolitis were started on a 5-day course of antibiotics
(metronidazole or amoxicillin with clavulanic acid) monthly for the
duration of the symptom.
If initial management was unsuccessful per physician assessment, then patients underwent further anorectal function evaluation
(Fig. 2), which included anorectal manometry, flexible sigmoidoscopy, and rectal suction biopsy. Clostridium botulinum toxin was
injected after completion of a flexible sigmoidoscopy in children
with anal sphincter pressure above 50 mmHg, who continued with
OS and/or enterocolitis (16). Anorectal manometry was completed
before all C botulinum toxin injections. Surgical referral was
completed if residual aganglionosis or an anatomic etiology (eg,

stricture) was identified. Children with intractable or recurring
symptoms despite aggressive medical therapy were referred for
surgical consultation after discussion between the primary pediatric
gastroenterologist and the family.
Anorectal manometry was performed as previously
described using a continuously perfused catheter using a lowcompliance pneumo-hydraulic system (Model ARM2; Arndorfer
Medical Specialties, Greendale, WI) (16). Colonic manometry was
performed in children with continued symptoms despite completing
an anorectal evaluation with directed therapy (Fig. 2) (16). Injection
of Botox (C botulinum toxin A, Allergan, Irvine, CA) with a 1-mL
syringe and a 25- to 30-gauge needle, 6 U/kg (up to 100 U total), was
divided and injected equally into 4 quadrants at the level of the
dentate line, as previously described (8).

Management
clinical impression. Children classified as having OS and/or enterocolitis were started on an aggressive bowel regimen. Children with
nonretentive fecal incontinence received full medical evaluations

Management was based on the diagnostic findings and
clinical course (Figs. 1 and 2). Laxative bowel regimens included
oral combinations of senna, polyethylene glycol 3350, magnesium

Child with Hirschsprung Disease after corrective
surgery unresponsive to initial management

Begin rectal therapies

Residual
aganglionosis

Surgical evaluation
for repeat pullthrough

Flexible sigmoidoscopy
Anorectal manometry
Rectal suction biopsy
If obstructive symptoms and/or enterocolitis:
Clostridium botulinum injection

Adjust bowel regimen based on
results

Improvement

Close follow-up
Reassess as needed

No improvement

Expand differential diagnosis
Colonic Manometry

Consider directed surgical
procedure

FIGURE 2. Diagnostic and therapeutic algorithm for a child with Hirschsprung disease and defecation disorders and/or
enterocolitis unresponsive to initial management.

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Volume 53, Number 1, July 2011

hydroxide, and bisacodyl. In patients with fewer than 2 bowel
movements per week and/or abdominal distention causing discomfort despite the use of an aggressive oral laxative regimen,
rectal therapies were recommended. These included either normal
saline irrigations or bisacodyl suppositories or sodium phosphate
enemas.

Final Diagnoses Responsible for the
Symptoms
Diagnoses responsible for the symptoms were determined
based on the result of diagnostic testing and management response.
Children with OS that improved significantly (eg, no longer having
constipation or fecal incontinence episodes related to stool retention) following an initial bowel regimen were classified as having
nonintractable constipation. Children who improved following anal
sphincter C botulinum injection were classified as having nonrelaxing anal sphincter as the reason for the symptoms, if another cause
(eg, colonic dysmotility, persistent aganglionosis) was not subsequently identified. Children with abnormalities on colonic manometry were classified as having colonic dysmotility. Children
undergoing a surgical procedure received a final diagnosis based
on the indication for the procedure (eg, residual aganglionosis).
Children with nonretentive fecal incontinence received a final
diagnosis reflecting the etiology of the incontinence (eg, food

intolerance).

Clinical Outcome
Clinical outcome was determined based on symptom
severity and management needed at the time of last follow-up
as compared to baseline. The 4 categories were defined as
follows:
1. Poor: No improvement or worsening in the number of bowel
movements and/or fecal accidents, or an enterocolitis episode
within 3 months of the last documented follow-up.
2. Fair: Improvement in the number of bowel movements with
need for rectal therapies (eg, enema, suppository, irrigation), or
fecal incontinence episodes more than once per week, or an
enterocolitis episode between 3 to 6 months of the last
documented follow-up.
3. Good: Improvement in the number of bowel movements with
continued usage of oral laxatives, or fecal incontinence
episodes less than once per week, or an enterocolitis episode
between 6 to 12 months of the last documented follow-up.
4. Excellent: Improvement in the number of bowel movements or
other OS without need for medications, or elimination of all
fecal incontinence episodes, or last enterocolitis episode more
than 1 year from the last documented follow-up.

Postoperative Hirschsprung Disease Problems

RESULTS
Baseline Characteristics
Fifty-seven children were identified. The mean age was
5.1 Æ 0.6 years. Forty-six (81%) were boys and 11 (19%) girls.

Seven (12%) had Down syndrome, 5 (9%) had developmental delay
of unknown etiology, and 1 (2%) had Bardet-Biedl syndrome. Of
the 57 children, 30 (53%) had OS alone (eg, constipation, straining
with defecation, abdominal distention), 14 (25%) had both OS and
postoperative enterocolitis episodes, 7 (12%) had postoperative
enterocolitis alone, and 6 (11%) had nonretentive (nonoverflow)
fecal incontinence.
The original extent of aganglionosis was short segment (rectosigmoid) in 40 (70%), long segment (through to ascending colon) in
8 (14%), and total colonic with or without small bowel involvement in
9 (16%). Surgical pull-through procedures included Soave in 36
(63%), Duhamel in 11 (19.4%), Swenson in 7 (12.3%), and unknown
(documentation from outside hospital unavailable) in 3 (5.3%).

Diagnostic Studies
Diagnostic studies completed within this population are found
in Table 1. The findings for children undergoing a barium enema were
notable for 5 identifying a stricture, 6 identifying a transition zone,
and 10 identifying colonic dilatation. All of the children undergoing
an anorectal manometry (n ¼ 45) did not have a recto-anal inhibitory
reflex. The mean sphincter pressure in those undergoing an anorectal
manometry was 90.2 Æ 3.3 mmHg (range 40–140). Six (10.5%)
children underwent colonic manometry evaluation. Of these, 2 were
normal; 3 had high-amplitude propagating contractions only in the
proximal colon, and 1 demonstrated complete colonic inertia.

Final Diagnoses
Ten (17.5%) children with OS responded well to an aggressive laxative regimen and were classified as having nonintractable
constipation. Eight (14%) children were found to have a mechanical
obstruction (eg, stricture), and 7 (12%) were found to have residual
aganglionosis. Twenty-two (38.6%) children were classified as

having a nonrelaxing internal anal sphincter (IAS) as the primary
reason for their OS. Four (6.8%) were identified as having
colonic dysmotility.
Of the 6 children with nonretentive fecal incontinence, 2 had
food intolerances that were identified; 2 had bacterial overgrowth as
a contributing factor as identified via lactulose breath testing and
response to antibiotic therapy, and the remaining 2 had abnormally
rapid colonic transit.

Surgical Interventions
Thirty-seven children (69.1%) received anal sphincter C
botulinum (Botox) injections. The mean number of injections in

Statistical Analyses
TABLE 1. Diagnostic studies completed
Results are presented as mean Æ standard error. Percentages
are rounded to the nearest decimal point. x2 analysis was used to
analyze differences in categorical variables between groups. Excellent and good long-term outcomes were classified as being favorable. Binomial logistic forward stepwise regression analysis with
favorable outcome as the dependent factor incorporating sex, age at
presentation, symptom type (eg, enterocolitis), Down syndrome,
extent of original aganglionosis, type of original pull-through (eg,
Duhamel), and final diagnosis was performed. SPSS was the
statistical software used (SPSS Inc, Chicago, IL).
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Diagnostic study
Barium enema
Anorectal manometry
Rectal suction biopsy
Flexible sigmoidoscopy

Full-thickness biopsy
Colonic manometry

n, %
47,
45,
39,
38,
13,
6,

83
79
69
67
23
11

77

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TABLE 2. Surgical procedures undertaken in children with HD
after the initial CS
Surgical procedure

Repeat pull-through
Anal dilatation and/or stricturoplasty
Anal sphincter myectomy
Ileostomy/colostomy
Antegrade cecostomy enema
Sigmoid stricturoplasty

n, %
9,
8,
6,
4,
2,
2,

16
14.0
10.5
7.0
3.5
3.5

Some children underwent more than 1 procedure. CS ¼ corrective surgery;
HD ¼ Hirschsprung disease.

those undergoing at least 1 Botox injection was 2.8 Æ 0.3. Of the 37
children undergoing Botox injections, 33 (89.2%) demonstrated an
initial short-term improvement.
Twenty-two (38.6%) children with HD after CS underwent at
least 1 further surgical procedure, with 10 (17.5%) undergoing

multiple procedures. A listing of surgical procedures performed can
be found in Table 2. All of the children with residual aganglionosis
underwent repeat pull-through. A repeat pull-through was also
performed in other select cases (eg, failed previous stricturoplasty).
The original type of surgical procedure did not predict the need for a
repeat surgical procedure (P ¼ 0.47). Four (7.0%) failed all therapy
and required diverting ileostomies or colostomies.

Long-term Clinical Outcomes
The mean follow-up period in this cohort was 41.4 Æ 4.5
months. Thirty-eight (66.7%) children had a favorable (excellent or
good) long-term outcome. Children who underwent a subsequent
surgical procedure (14/22) were as likely to have a favorable
outcome as those following medical therapy (24/35) alone
(P ¼ 0.78). Children with any form of developmental delay including Down syndrome or Bardet-Biedl syndrome (6/13) were as likely
as those without developmental delay (32/44) to have a favorable
outcome (P ¼ 0.09) (Fig. 3).
Multivariate logistic regression analysis determined that
children with enterocolitis as a presenting symptom were more
likely to have a favorable outcome (18/21) than those without
enterocolitis (20/36) (P < 0.05). Other factors, including sex, age
at presentation, Down syndrome, any form of developmental delay,
extent of original aganglionosis, type of original pull-through (eg,

FIGURE 3. Distribution of clinical outcomes by percentage
into ex, good, fair, poor categories in all 57 patients, and by
those having and not having enterocolitis. Ex ¼ excellent.

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Duhamel), and final diagnosis, were not found to predict long-term
clinical outcome.
Two of the 6 (33%) children with nonretentive fecal incontinence had a favorable outcome at the time of the last follow-up.
The 1 child with nonretentive fecal incontinence with identified low
anorectal sphincter pressure had a poor long-term outcome.

DISCUSSION
To our knowledge, this is the first study to evaluate long-term
clinical outcomes as well as diagnoses and management strategies
when following a comprehensive systematic algorithm addressing
all protracted DDs in children with HD after CS. A variety of
diagnoses were made, including identification of residual aganglionosis and other anatomic problems that required surgical correction.
Management using the algorithm was dependent on the underlying
etiology of the symptoms, and approximately two-thirds of the
cohort had had a good or excellent long-term clinical outcome.
Previous authors have reported that the need for reoperation
following initial pull-through CS for HD may be relatively high,
with estimates ranging between 26% and 34% (17,18). The rate of
need for reoperation in our population is slightly higher (38%) than
we had initially expected, given that all of the children evaluated in
our gastroenterology clinic were past the initial perioperative
period. More than 25% of children required further surgical management for either a mechanical obstruction (eg, stricture) or
residual aganglionosis. This may have been partly the result of
the nature of the referral population to a tertiary care center because
these patients were likely to have failed previous more conservative
measures and/or their symptoms may have been more pronounced.

Nonetheless, communication with a pediatric surgeon is clearly
needed in the routine care of these children.
The cause for a relatively high number of children presenting
with residual aganglionosis is unknown; it may be the result of
surgical technique (eg, transition zone pull-through), chronic unidentified enterocolitis, or loss of ganglion cells after pull-through
(19,20). Efforts directed toward minimizing postoperative aganglionosis are already being investigated through modification of
surgical techniques (10). In the future, progenitor cell transplantation to repopulate areas of aganglionosis after CS with functional
ganglion cells may be possible, and any such advances would
change the algorithm accordingly (21).
Patients with postoperative HD may be at greater risk for
DDs given a postsurgical neorectum with likely decreased sensation
and accommodative capacity, continued nonrelaxation of the IAS
(16), and higher propensity for colonic dysmotility (22,23). Despite
these apparent disadvantages, certain children with OS and/or
enterocolitis responded well to an aggressive oral laxative regimen
alone. This supports the view that children with postoperative HD
may develop a pattern of defecation avoidance because of trauma or
previous discomfort similar to children with functional constipation
(6). We support an initial approach of using oral laxative regimens
with common behavioral approaches (eg, toilet sitting) in this
population of children in an attempt to avoid further unnecessary
interventions.
In theory, all children with HD have a nonrelaxing IAS,
which may cause a functional obstruction. Attribution of symptoms
to this functional obstruction following improvement with local
Botox injection therapy has been supported by us and others
(16,20,24). Given Botox anal sphincter injection is a local therapy,
and children who responded had not previously done so with more
conservative measures, we believe that attribution of symptoms to a
nonrelaxing IAS is appropriate. We recently reported our long-term

experience of using Botox in children with nonrelaxing IAS (16)
and believe that it may be a useful therapy within an overall
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therapeutic algorithm. Other centers may have more experience
with anal sphincter myectomy (25), although we prefer to avoid
myectomy when possible given higher long-term fecal incontinence
rates, and the natural tendency of the anal sphincter to become
hypotensive in adults over time with HD after CS (26). Future
incorporation of topical therapies (eg, glycerin trinitrate) to relax
the anal sphincter may prove to be beneficial (27).
Nonretentive fecal incontinence in our population was less
common. In the 6 patients within this cohort, the differential
diagnosis was broadened and encompassed small bowel bacterial
overgrowth, food intolerance, and rapid colonic transit. These
diagnoses suggest that children with HD with nonretentive fecal
incontinence may benefit from full medical evaluations beyond the
use of agents to slow intestinal transit alone.
The only baseline predictive factor for a favorable outcome
using this algorithm was enterocolitis as a presenting symptom,
suggesting children with this presentation are most likely to benefit

from the management within the algorithm. The vast majority of
children with enterocolitis had a favorable outcome in the present
study. This may in part be due to the ability of many of the
interventions to improve colonic transit and prevent obstruction.
Moreover, we postulate that enterocolitis is less likely to have a
functional/behavioral component and as such may be more directly
amenable to the rendered therapies.
The primary weakness of the present study is that it is
retrospective. As such, factors such as follow-up intervals and
compliance with medication regimens prescribed were not
accounted for. Symptom improvement was subjective and based
on history as recorded by the primary gastroenterologist. However,
some of the nonstandardized factors may have been ameliorated by
the fact that 1 of the investigators (S.N.) was involved in the care of
all of the patients, that all of the patients with postoperative HD with
DDs at our institution are referred to the pediatric gastroenterology
program, and that a systematic algorithm was followed in all of the
patients. Another limitation of the present study is that it incorporates numerous diagnostic techniques and interventions. As such,
in theory, an effective intervention may be buried within other less
effective or even harmful interventions. It is for this reason,
however, that we chose to evaluate the systematic algorithm as a
whole. Given that the algorithm was followed in the entire studied
cohort in a manner consistent with daily clinical care, we hope that
this model will serve as an early step toward further work in
comparative effectiveness in the care of these children. In the
future, changes in the algorithm with new clinical and/or technical
advances may be made and outcomes compared accordingly.
We propose that the stepwise algorithm is generalizable and
can be followed in the majority of medical centers that treat children
with HD. Evaluation of the child’s anatomy followed by appropriate

categorization into obstructive or nonretentive symptoms will lead
to appropriate management. Referral to another institution will vary
depending on the availability of therapies such as anal sphincter
Botox, or the need for a more advanced colonic manometry
evaluation. In our cohort, only a minority of patients underwent
colonic manometry, and these patients did not have a superior longterm outcome. As such, only a small number of patients would need
to be referred for this evaluation using the algorithm.
In conclusion, the results of the present study suggest that
following a comprehensive systematic algorithm for protracted
DDs in children with postoperative HD may identify diagnoses
that require specific therapies and leads to favorable long-term
outcomes for the majority of children evaluated.

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