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Article history: Aims: Hirschsprung disease (HD) is a chronic condition associated with long-term morbidity. We assessed the
Received 4 November 2016 short and long-term functional outcomes of operated patients in a single institution over a 12-year period.
Accepted 8 November 2016 Materials and methods: We conducted a retrospective review of all children operated for HD between 2002 and
Available online xxxx 2014. Postoperative functional outcomes were assessed using the Rintala Bowel Function Score (BFS, 0–20,
20 = best score). We assessed hospital admissions, complications including Hirschsprung associated enterocoli-
Key words:
tis (HAEC) and the need for further surgical procedures.
Hirschsprung disease
Duhamel operation
Results: 72 (52 male) patients were studied, of whom, 6 (8%) had a positive family history, 5 (7%) had Trisomy 21
Functional outcomes and 5 (7%) had total colonic HD. The median age at diagnosis was 6.5 days (2 days-6.7 years) and median follow-
up was 6 years (1–12 years). All patients except two underwent a Duhamel pull-through procedure. The median
age at surgery was 4 months (6 days–90 months). 37 (51%) procedures were performed single-stage and 7 (10%)
were laparoscopically assisted. Our early complication rate was 15%; 11 (15%) patients were treated for HAEC and
43 (60%) did not require any further surgery. 12 (17%) underwent injection of botulinum toxin, 7 (10%) needed
residual spur division and 4 (5%) required an unplanned, post pull-through enterostomy for obstructive defeca-
tion symptoms and HAEC. Two (3%) patients underwent an Antegrade Colonic Enema (ACE) stoma. The median
BFS was 17 (5–20). There were two deaths both out of hospital.
Conclusions: Long-term functional outcomes following Duhamel Pull-Through surgery are satisfactory although
40% of patients needed some form of further surgical intervention. The management of anal sphincter achalasia
has improved with the use of botulinum toxin and we advocate aggressive and early management of this condi-
tion for symptoms of obstructive defecation and HAEC.
Level of evidence: III
© 2016 Elsevier Inc. All rights reserved.
Hirschsprung disease (HD) is a chronic condition with long-term technique, aiming for a single-stage procedure provided satisfactory de-
effects on the physical and psychosocial health of a patient [1]. The compression could be attained preoperatively.
outcomes of surgery have improved over the last few decades We sought to assess the short and long-term outcomes of all operat-
and there is now an increased recognition in the literature focusing ed patients with HD in our institution over a 12-year period.
on the functional outcomes of pediatric patients as they mature into
adults. Long-term symptoms include constipation, bloating, soiling
and fecal incontinence. Whereas the majority of these symptoms can 1. Materials and methods
be managed either medically or through limited surgical intervention,
some patients have had an enterostomy fashioned to improve their A retrospective case note review was performed for all histopatho-
quality of life. Hirschsprung associated enterocolitis (HAEC) also has logically confirmed cases of HD undergoing pull-through surgery at
a well-recognized morbidity and mortality that can continue to affect our institution between January 2002 and January 2014 inclusive. Any
patients postoperatively. patients with a pull-through procedure performed external to our insti-
Several surgical pull-through procedures have been described with tution were excluded. Patient demographics, comorbidities, timing of
varying outcomes. Our preference has been to use the Duhamel diagnosis and surgery, need for a preoperative enterostomy and details
of the surgery were all collected.
Short-term outcomes included postoperative complications such as
⁎ Corresponding author. Tel.: +44 20 7188 7188; fax: + 44 20 71884612/020
bowel obstruction, sepsis or an anastomotic leak. HAEC was clinically
71884556. suspected on the basis of toxemia, offensive/bloody stools, abdominal
E-mail address: ali.keshtgar@gstt.nhs.uk (A.S. Keshtgar). distension and raised inflammatory markers.
http://dx.doi.org/10.1016/j.jpedsurg.2016.11.023
0022-3468/© 2016 Elsevier Inc. All rights reserved.
Please cite this article as: Thakkar HS, et al, Functional outcomes in Hirschsprung disease: A single institution's 12-year experience, J Pediatr Surg
(2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.11.023
2 H.S. Thakkar et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
Long-term functional outcomes were objectively assessed using the botulinum toxin Dysport® (Ipsen Biopharm Limited) into the internal
Rintala Bowel Function Score (BFS) [2]; a validated tool for use in pa- anal sphincter (IAS) and a rectal tube. One patient had misplacement
tients with anorectal malformations and HD. Seven domains focus on of an epidural catheter into the presacral space, recognized and re-
bowel control, symptoms of soiling and constipation as well as the social moved at the time of the pull-through. Two patients (one multistage,
impact of the disease. A maximum score of 20 can be attained (0–20). A one single-stage) developed postoperative sepsis with a pelvic collec-
single investigator contacted parents of all patients at least four years of tion, of whom one patient required a defunctioning ileostomy. Four pa-
age by telephone to attain the score. Ethical approval for this was tients required postoperative parenteral nutrition for high-enterostomy
attained from the Hospital (Approval Number #AN5019). output or loose stools until enteral feeding was established. There was
Patient records were also assessed for any surgical interventions in- one superficial wound dehiscence managed conservatively.
cluding the need for an examination under anesthesia, disimpaction of
stool from the rectum/washout, intrasphincteric botulinum toxin injec- 2.2. Longer-term outcomes
tion and rectal spur division. All data are presented as median (range).
Data are expressed as median (range). The Mann–Whitney U test was 2.2.1. Post-pull-through Hirschsprung associated enterocolitis (HAEC)
used to compare outcomes between groups with nonparametric data. There were 15 episodes of HAEC in 11 (15%) patients of whom 5 had
The Chi-Square test was used to compare outcomes between categorical multistage surgery. The stool specimens were positive for Rotavirus in
data. P b 0.05 was considered statistically significant. three cases. The median time at presentation was 18 months post-
pull-through (1–90 months). In addition to these cases, 23 (32%) pa-
tients also had at least one episode of simple gastroenteritis requiring
2. Results
hospital admission (including one Norovirus, four Rotavirus and one
Clostridium difficile).
86 patients with histopathologically confirmed HD were identified
during the study period. 14 patients were excluded; 10 patients
2.2.2. Further unplanned surgical interventions after pull-through
underwent pull-through surgery elsewhere, two sets of case notes
31 (43%) patients needed at least one unplanned surgical interven-
were missing, one patient was awaiting surgery and one infant with
tion; ranging from zero to six. Table 2 summarizes the most commonly
Smith–Lemli Opitz Syndrome died preoperatively from respiratory fail-
performed surgical interventions with their age at presentation. 12 pa-
ure. 72 data sets were thus included for analysis over the 12-year period.
tients had intrasphincteric injection of botulinum toxin Dysport®
Median follow-up for the series was for 6 (1–12) years.
(12 U per kg body weight up to maximum dose of 200 U) under
52 (72%) patients were male, 6 (8%) had a positive family history of
endosonographic guidance for obstructive defecation, recurrent ab-
HD, 5 (7%) had an associated history of Trisomy 21 and one patient had
dominal distension and HAEC symptoms. Of these 6 had injection into
MEN 2a syndrome who later underwent a prophylactic thyroidectomy
the internal anal sphincter (IAS) and 6 into the external anal sphincter
at 2 years of age. The median age at diagnosis was 6.5 days (range
(EAS) muscles depending on the preference of the surgeon. There was
2 days–6.7 years). Median patient follow-up was 6 (1–12) years with
significant improvement of abdominal distension following injection
data included up to December 2015.
of the toxin into the EAS versus the IAS (5/6 patients improved vs. 1/6
70 patients underwent a Duhamel pull-through with two undergo-
patients respectively, P b 0.05). 7 (60%) patients underwent only a sin-
ing a Soave (individual surgeon's preference). 7 (10%) cases were
gle course of botulinum toxin treatment. No patients in the EAS group
laparoscopically assisted including both cases of the Soave pull-
required formation of an enterostomy compared with two patients in
through. The median age at surgery was 4 months (6 days–90 months).
the IAS group.
Overall, 37 (51%) of all cases were performed single-stage. 18 (60%) of
cases were performed multistage between 2002 and 2007 with the fig-
2.2.3. Functional outcomes: Rintala Bowel Function Score (BFS)
ure dropping to 17 (41%) between 2008 and 2014. 4 (5%) patients had a
53 patients aged 4 years and above were eligible for the telephone
covering ileostomy performed at the time of the planned pull-through.
survey of whom 32 (60%) were contactable. The median BFS was 17
Table 1 summarizes the reasons for a pre-pull-through enterostomy
(5–20), Fig. 1. The median BFS in the multistage group was 15 compared
(n = 35).
with 18 for single-stage surgery (P = 0.08), which was not statistically
The level of the pull-through was at the sigmoid colon in 70% of
significant.
cases. 7% had total-colonic disease with an ileal pull-through. The histol-
20 (28%) of patients at their most recent follow up were still using
ogy of the pull-through was ganglionic in 99% of cases.
laxatives. Two (3%) patients have had an Antegrade Colonic Enema
(ACE) stoma procedure for chronic constipation and soiling and became
2.1. Early complications (within 30 days of pull-through) clean postoperatively. Four (5%) patients required an emergency or ur-
gent ileostomy for recurrent obstructive bowel symptoms and/or en-
11 (15%) patients had an early complication. One patient developed terocolitis of which only one patient has an enterostomy in situ to
adhesive bowel obstruction after multistage surgery requiring laparoto- date. Of these four patients, one patient also required excision of a
my. There were three cases of suspected obstruction; two necessitated megarectum at the time of his ileostomy.
an examination under anesthesia and one was managed on the ward
with a rectal tube. One had early problems with sphincter achalasia fol- Table 2
lowing primary pull-through and was managed with injection of Surgical interventions performed for symptomatic patients following initial surgery.
Please cite this article as: Thakkar HS, et al, Functional outcomes in Hirschsprung disease: A single institution's 12-year experience, J Pediatr Surg
(2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.11.023
H.S. Thakkar et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx 3
Please cite this article as: Thakkar HS, et al, Functional outcomes in Hirschsprung disease: A single institution's 12-year experience, J Pediatr Surg
(2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.11.023
4 H.S. Thakkar et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
Table 3
Further surgical interventions post pull-through for Hirschsprung disease (UK comparison).
Laparotomy for bowel obstruction 1/72 (1.4%) 7/91 (7.7%) 1/76 (1.3%)
Anal stretch/myectomy/Botulinum toxina 12/72 (17%) 19/91 (21%) 15/76 (20%)
Rectal spur division 7/72 (10%) 9/91 (10%) 11/76 (14%)
Enterostomy formation for obstructive defecation or soiling 4/72 (5%) 6/91 (6.5%) 6/76 (8%)
Antegrade Colonic Enema (ACE) stoma 2/72 (3%) 1/91 (1%) –
Major revisional surgery 0/72 (0%) 2/91 (2%) 3/76 (4%)
Total 31 (43%) 44 (48%) 36 (47%)
a
Botulinum toxin is the preferred treatment of choice for anal sphincter achalasia in our institution.
We have observed significant improvement of abdominal distension bowel function score is good but you have a large group
following intrasphincteric injection of botulinum toxin in this series. who may have dropped out.
This was more effective if the toxin was injected into the EAS muscles A: Hemanshoo Thakkar One of the patients who died we did have a
with no patients requiring an enterostomy in this group. This is in agree- post mortem but we couldn't find a cause. The other patient
ment with our experience of botulinum toxin treatment of children probably had enterocolitis but we can collate this if we get
with chronic functional constipation [12,13]. round to publishing this (!).
Heij et al. reported the long-term outcomes of their patients follow- Q: Gregor Walker (Glasgow) Were you tempted to categorize your pa-
ing pull-through surgery for HD [14]. At a median age of 6.6 years, 20% of tients for length of aganglionosis, as you had one slide on your
patients aged more than 4 years were continent without constipation, total colonics but you also had quite a disparate group of dif-
45% had soiling and/or constipation and 35% were incontinent. More re- ferent lengths of aganglionosis within the colon itself. Did
cently, in a series of 49 adult patients who had undergone a Duhamel they have different bowel scores?
pull-through in childhood, approximately half the patients were A: Hemanshoo Thakkar The only groups which we compared were sin-
deemed to have a satisfactory result [15]. The authors defined “satisfac- gle and multistaged and in that we didn't find difference in
tory” to be a BFS of ≥ 17 with the lack of an enterostomy or major scores but something we can certainly do is look at the length
revisional surgery [4]. In this latter series, similar satisfactory results of aganglionosis because in the paper published by Prof.
were attained in 38/89 (42%) of patients with 6/91 (6.5%) needing an Rintata from the Simpson Smith symposium, they certainly
enterostomy in later life for intractable symptoms of either constipation did look at these things and we want to as well.
or soiling. Our results are comparable to above reports with a BFS of ≥17
attained in 45% of our cases and 4/72 (5.5%) needing an enterostomy of
which all but one have been reversed. The main difference in our series
was the lack of major revisional surgery. We feel that this is largely References
owing to the good outcomes from intrasphincteric injection of botuli-
num toxin for residual distal obstructive symptoms that can help [1] Rintala RJ, Pakarinen MP. Long-term outcomes of Hirschsprung's disease. Semin
Pediatr Surg 2012;21:336–43.
avoiding the revisional surgery. [2] Rintala RJ, Lindahl H. Is normal bowel function possible after repair of intermediate
In a recently published series of adult patients having undergone and high anorectal malformations? J Pediatr Surg 1995;30:491–4.
transanal endorectal pull-through in childhood, bowel functional out- [3] Langer JC. Hirschsprung disease. In: Coran AG, editor. Pediatric surgery. 7th ed. Phil-
adelphia: Elsevier, Saunders; 2012. p. 1265–78.
comes seemed to improve with age. This is in agreement with our obser- [4] Nah SA, de Coppi P, Kiely EM, et al. Duhamel pull-through for hirschsprung
vation and reports of other investigators [9,16]. Botulinum toxin can disease: a comparison of open and laparoscopic techniques. J Pediatr Surg 2012;
provide effective temporary relief from anal sphincter achalasia symp- 47:308–12.
[5] Huang EY, Tolley EA, Blakely ML, et al. Changes in hospital utilization and manage-
toms that is more acceptable to the child and family while awaiting im- ment of Hirschsprung disease: analysis using the kids' inpatient database. Ann
provement in bowel function as the child grows up. Rectal washouts or Surg 2013;257:371–5.
formation of an ACE may also be necessary in these children. [6] Sulkowski JP, Cooper JN, Congeni A, et al. Single-stage versus multi-stage pull-
through for Hirschsprung's disease: practice trends and outcomes in infants. J
In conclusion, our experience with the Duhamel pull-through proce-
Pediatr Surg 2014;49:1619–25.
dure for managing patients with Hirschsprung disease has been favor- [7] Demehri FR, Halaweish IF, Coran AG, et al. Hirschsprung-associated enterocolitis:
able and the functional outcome and complications have been pathogenesis, treatment and prevention. Pediatr Surg Int 2013;29:873–81.
comparable to other reports. This is particularly true as children grow [8] Teitelbaum DH, Qualman SJ, Caniano DA. Hirschsprung's disease. Identification of
risk factors for enterocolitis. Ann Surg 1988;207:240–4.
into adolescence. In the last two decades, the approach to performing [9] Baillie CT, Kenny SE, Rintala RJ, et al. Long-term outcome and colonic motility
pull-through surgery has evolved with single-stage surgery and laparo- after the Duhamel procedure for Hirschsprung's disease. J Pediatr Surg 1999;34:
scopic assistance becoming increasingly popular. The management of 325–9.
[10] Langer JC, Birnbaum E. Preliminary experience with intrasphincteric botulinum
anal sphincter achalasia and obstructive bowel symptoms has also im- toxin for persistent constipation after pull-through for Hirschsprung's disease. J
proved with the use of botulinum toxin treatment. We advocate early Pediatr Surg 1997;32:1059–61.
and aggressive management of this condition to help achieve better [11] Koivusalo AI, Pakarinen MP, Rintala RJ. Botox injection treatment for anal outlet ob-
struction in patients with internal anal sphincter achalasia and Hirschsprung's dis-
long-term functional outcomes and avoid complications of HAEC and ease. Pediatr Surg Int 2009;25:873–6.
unplanned surgical interventions. We also support the notion of devel- [12] Keshtgar AS, Ward HC, Sanei A, et al. Botulinum toxin, a new treatment modality for
oping core outcome data acquisition in the future to help standardize chronic idiopathic constipation in children: long-term follow-up of a double-blind
randomized trial. J Pediatr Surg 2007;42:672–80.
and compare outcomes.
[13] Keshtgar AS, Ward HC, Clayden GS. Transcutaneous needle-free injection of botuli-
num toxin: a novel treatment of childhood constipation and anal fissure. J Pediatr
Appendix A. Discussions Surg 2009;44:1791–8.
[14] Heij HA, de Vries X, Bremer I, et al. Long-term anorectal function after Duhamel op-
eration for Hirschsprung's disease. J Pediatr Surg 1995;30:430–2.
Hemanshoo Thakkar [15] Conway SJ, Craigie RJ, Cooper LH, et al. Early adult outcome of the Duhamel proce-
dure for left-sided hirschsprung disease—a prospective serial assessment study. J
Q: Unknown You have 75 patients 2 died and 4 had stoma. So this gives Pediatr Surg 2007;42:1429–32.
[16] Neuvonen MI, Kyrklund K, Rintala RJ, et al. Bowel function and quality of life after
me mourned 10% of the cases don't have a very good out- transanal endorectal pull-through for hirschsprung disease: controlled outcomes
come. So you may have to consider how you look at it up to adulthood. Ann Surg 2016 [Epub ahead of print].
Please cite this article as: Thakkar HS, et al, Functional outcomes in Hirschsprung disease: A single institution's 12-year experience, J Pediatr Surg
(2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.11.023