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Internal sphincterotomy in post-pull-through Hirschsprung's disease
G K Blair1, J J Murphy, G C Fraser
1Division of Paediatric General Surgery, British Columbia Children's Hospital, Vancouver, Canada.
Insights
Posterior internal sphincterotomy effectively treated severe constipation after pull-through surgery for Hirschsprung's disease in 90% of patients. This intervention resolved megarectum and megacolon, improving bowel evacuation.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Colorectal Surgery
Background:
- Pull-through operations for Hirschsprung's disease can result in persistent bowel evacuation difficulties.
- Severe constipation, obstipation, and fecal retention are common post-operative complications.
- Previous conservative management with laxatives and enemas often proves unsuccessful.
Purpose of the Study:
- To evaluate the efficacy of full posterior internal sphincterotomy in patients with Hirschsprung's disease who experienced persistent bowel evacuation issues after pull-through surgery.
- To assess the resolution of megarectum and megacolon following sphincterotomy.
Main Methods:
- Ten patients with Hirschsprung's disease and post-pull-through constipation underwent full posterior internal sphincterotomy.
- Procedures included Soave, Duhamel, and Kimura-Soave techniques.
- Radiographic and biopsy assessments were performed to confirm megarectum/megacolon and presence of ganglia.
Main Results:
- Nine out of ten patients (90%) achieved good or excellent outcomes after sphincterotomy.
- Sphincterotomy led to the resolution of megarectum and megacolon in these patients.
- Three patients required minimal, decreasing doses of senna compound post-operatively.
Conclusions:
- Full posterior internal sphincterotomy is a highly effective treatment for refractory constipation and megarectum/megacolon following pull-through surgery for Hirschsprung's disease.
- This surgical intervention significantly improves bowel evacuation in the majority of affected pediatric patients.
- One patient with Down's syndrome and a Duhamel pull-through required a stoma, indicating potential specific challenges.
Abstract:
Despite proper technique, pull-through operations for Hirschsprung's disease sometimes fail to deliver normal or effective bowel evacuation. Ten patients, described herein, had had a pull-through procedures. The procedures had been performed by various pediatric surgeons. Five cases had been diagnosed in the newborn period and had undergone colostomies. The remainder had been diagnosed later (at 2 months to 2 years of age). They too had undergone colostomy initially, and all had an elective pull-through procedure. The techniques varied; two had Soave procedures, seven had Duhamel procedures, and one had a Kimura-Soave procedure (the only case of total colonic Hirschsprung's disease in the series). All the patients had manifested difficulty in passing stools after the pull-throughs. The problem was described as "severe constipation," "obstipation," or "fecal retention." Four patients had been treated with many laxatives, suppositories, enema routines, and diet regimens for years, with no success. All had been examined radiographically to detect megarectum or megacolon. All had additional biopsies to confirm the presence of ganglia in the pulled-through segments. At 21 months to 12 years of age, these patients underwent full posterior internal sphincterotomies. Nine of the 10 had a good or excellent outcome, with resolution of the megarectum or megacolon. Three patients still require small doses of senna compound, which are being decreased continuously. Therapy failed for a patient with Down's syndrome and a, Duhamel pull-through, and a stoma was required.