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Introduction
Pull-through (PT) operations for Hirschsprungs disease(HD) generally have satisfactory outcome
constipation, enterocolitis and recurrent obstructive symptoms .
Few patients with persistent abdominal distension, constipation or enterocolitis due to postoperative stricture or retained aganglionic segment
Methods
Search terms
residual aganglionosis
transitionzone bowel
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
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.
Results
29 published articles
24 articles (82.8%) from single centers 5 (17.2%) from multicenter studies
49 (25.4%) TZB
Patients gender
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
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
Age
Patients age at redo PT operation
Documented in 108 (56.0%) patients Mean of 4.4 years (range 4 months17 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
meta-analysis reveals
RA and TZB : underlying causes of persistent bowel symptoms in one-third of all patients requiring redo PT operation.
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
Postoperative enterocolitis
rectal irrigation with or without metronidazole prophylaxis
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
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
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.
METHODS
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
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.
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