Internal sphincterotomy in post-pull-through Hirschsprung's disease

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 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 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.