Residual aganglionosis after pull-through operation for Hirschsprungs disease: a systematic review and meta-analysis

Florian Friedmacher , Prem Puri

Pediatr Surg Int (2011) 27:10531057

Introduction

Pull-through (PT) operations for Hirschsprungs disease(HD) generally have satisfactory outcome
constipation, enterocolitis and recurrent obstructive symptoms .

Some continue to have disturbances of bowel function after definitive operation :

Majority with residual bowel problems

managed by non-surgical treatment : laxatives, enemas or intrasphincteric botulinum toxin injection

Few patients with persistent abdominal distension, constipation or enterocolitis due to postoperative stricture or retained aganglionic segment

require a redo PT operation

Purpose of the study

meta-analysis designed to determine
incidence and outcome of residual aganglionosis(RA) in patients with HD following PT operation

Methods

PubMed and MEDLINE databases

All studies that reported cases of patients with HD who had undergone redo PT operations for RA or transition-zone bowel (TZB) Between 1985 and 2011.

Search terms

residual aganglionosis

transitionzone bowel

redo and repeat pullthrough

reoperation Hirschsprungs disease

Additional cases
The reference lists from retrieved articles were reviewed
All published studies and abstracts presented at various meetings evaluated.

Only publications containing all the relevant details were included in the literature review

Detailed information recorded

Type of study Gender Recurrent bowel symptoms Histological findings on repeat rectal biopsy Patients age at initial PT and redo PT operation Type of surgical procedures performed Postoperative follow-up with recurrent symptoms.

Publications not giving adequate clinical data of patients were excluded

Results

Between 1985 and 2011

29 published articles
24 articles (82.8%) from single centers 5 (17.2%) from multicenter studies

555 patients with HD underwent redo PT

193 (34.8%) demonstrated abnormal histological findings on repeat rectal biopsy.

144 (74.6%) revealed RA

49 (25.4%) TZB

Of these 193 patients

persistent abdominal distension/constipation n=135 recurrent episodes of enterocolitis n=45 Histological evidence of RA in the resected proximal margin of the pulled-through bowel n=13

Patients gender

reported in 135(69.9%) patients. A male-to-female ratio of 3.5:1 was observed

Initial PT procedure
Documented in 143 (74.0%) patients
Soave procedure Duhamel procedure Rehbein procedure Swenson procedure Transanal endorectal PT (TERPT) Posterior sagittal approach n=82 n=24 n=15 n=14 n= 7 n= 1

50 patients not reported.

Redo PT procedure
Documented in 143 (74.1%)
Duhamel procedure TERPT Soave procedure Swenson procedure Posterior sagittal approach n=57 n=40 n=35 n=10 n= 1

Type of redo procedure not reported in 50 pts

Age
Patients age at redo PT operation
Documented in 108 (56.0%) patients Mean of 4.4 years (range 4 months17 years).

Time between initial PT and redo PT operation

reported in 74 (38.3%) patients mean of 2.8 years (range 6 months8 years).

Follow-up
available in 134 (69.4%) patients mean follow-up time : 4.1 years (3 m 23 yrs) Of the 134 patients,
99 (73.9%) pts normal bowel habits after redo PT operation. 19 pts persistent/intermittent constipation with occasional soiling 16 patients had recurrent enterocolitis with or without perianal excoriation.

Most of the patients were fecally continent and had normal bowel movements except for occasional soiling . No significant difference in functional outcome between the various redo PT procedures

discussion

Several studies : no statistically significant difference in the functional outcome with respect to bowel function between the various PT procedures to treat HD

For a successful PT , it is essential that all aganglionic bowel is resected and bowel with normal innervation is anastomosed to the anus

193 (34.8%) demonstrated abnormal histological findings on repeat rectal biopsy.

144 (74.6%) revealed RA 49 (25.4%) TZB

Of these 193 patients

persistent abdominal distension/constipation
n=135

recurrent episodes of enterocolitis

n=45

Histological evidence of RA in the resected proximal margin of the pulled-through bowel

n=13

meta-analysis reveals
RA and TZB : underlying causes of persistent bowel symptoms in one-third of all patients requiring redo PT operation.

Redo PT operation for RA or TZB :

potentially preventable by accurate identification of the proximal margin of the aganglionic bowel and transitionzone by an experienced histopathologist

During frozen section analysis at the time of the initial PT operation:

the pathologist must confirm normal ganglion cells and absence of nerve trunks at the site of the planned anastomosis

Major problem with the intraoperative frozen section biopsies :

can indicate the presence of ganglion cells without differentiating between hypo- and dysganglionosis

Shayan K et al (2004): Reliability of intraoperative frozen sections in the management of Hirschsprungs disease, J Pediatr Surg
3% of 304 children who had intraoperative frozen section analysis during PT operation showed a discrepancy between the frozen section diagnosis and the final pathological diagnosis.

The use of rapid technique of acetylcholinesterase staining may help overcome this problem

To prevent pulling-through the transition-zone for anastomosis

resecting several cm above the proximal ganglionic bowel identified by the pathologist during frozen sections.

Recurrent bowel problems after PT

Constipation after PT operation in vast majority
non-operative methods :laxatives and enemas

Postoperative enterocolitis
rectal irrigation with or without metronidazole prophylaxis

persistent constipation, abdominal distension or recurrent episodes of enterocolitis

a full thickness rectal biopsy indicated to rule out RA or TZB

presence of RA or TZB
resection of this section of bowel may cure the patients of their recurrent symptoms. a redo PT is generally recommended for surgical management of RA

the choice of procedure far from obvious.

type of previous failed procedure level of anastomosis rectal blood supply and presence of fibrosis or inflammation in the perirectal pouches must be considered

In the present metaanalysis, most patients with HD had normal bowel function after redo PT operation

references

Repeated pull-through surgery for complicated Hirschsprung's disease--principles derived from clinical experience.
Schweizer P, Berger S, Schweizer M, Holschneider AM, Beck O

J Pediatr Surg. 2007 Mar;42(3):536-43

Methods
17 pts with HD aged 2 to 9 years Surgical revision indicated by incomplete resection of the transition zone in 16 patients, anastomotic strictures in 9 patients, and fistulas in 2 patients. All 17 patients Redo Duhamel PT Median follow-up 9 years (range, 1-23 years).

CONCLUSIONS
predominant cause for persistent or recurrent obstructive symptoms after initial pull-through procedure : incomplete resection of the transition zone.

Redo Duhamel pull-through procedure is able to provide the definitive solution to the problem.

Redo pull-through in Hirschsprung's [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel.
Lawal TA, Chatoorgoon K, Collins MH, Coe A, Pena A, Levitt MA.

J Pediatr Surg. 2011 Feb;46(2):342-7

METHODS

93 pts with HD with recurrent problems after PT

All required reoperations 25 had residual aganglionosis/transitionzone histology : the only indication for redo in 16 children.

RESULTS
Rectal biopsy: hypertrophic nerves (n = 16), absent ganglion cells (n = 6), and normal ganglion cells (n = 10). Original frozen-section biopsy only sampled the seromuscular layer in 3 children, leading to misdiagnosis. In all cases, obstructive symptoms were resolved, and no patient had recurrent enterocolitis.

CONCLUSIONS
Patients' post pull-through with recurrent obstructive symptoms may have residual aganglionosis or transitionzone bowel.
Reoperation can result in the resolution of these symptoms. A full-thickness biopsy at the time of the initial pullthrough to include the mucosa and submucosa may increase the possibility of identifying hypertrophic nerves

Reoperation for Hirschsprung disease: pathology of the resected problematic distal pull-through
Coe A, Collins MH, Lawal T, Louden E, Levitt MA, Pena A Pediatr Dev Pathol. 2012 Jan-Feb;15(1):30-8

histopathology of pull-through bowel segments resected because of poor postoperative outcome from 30 patients

MC indication for reoperation: constipation/obstruction

Transition zone (bowel with at least two nerves 40 m diameter per 400 high-power field, and ganglion cells) or aganglionic bowel (bowel with at least two nerves 40 m per high-power field diameter, but without ganglion cells) was found in 19/30 (63%) resections.

Thank you