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Hirschsprung's disease in young adults
Insights
Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung's disease (HSCR) is a congenital condition characterized by the absence of ganglion cells in the distal bowel.
- HSCR is rare in young adults, presenting unique management challenges due to proximal colonic dilatation and hypertrophy.
Observation:
- Diagnosis involves barium enema, rectal biopsy (suction or full-thickness), and anal manometry.
- Eight young adult patients with HSCR were managed using a two-stage surgical approach.
Findings:
- The initial stage involved a sigmoid colostomy with a defunctionalized stoma, allowing distal colon cleansing and proximal colon decompression.
- The proximal colon typically normalized in caliber within 2 to 6 months.
- Reconstruction using Duhamel or Soave procedures yielded good results, with Duhamel preferred for significant rectal segment discrepancies.
Implications:
- A two-stage surgical approach with preliminary colostomy is effective for managing Hirschsprung's disease in young adults.
- This management strategy facilitates subsequent pull-through procedures and improves patient outcomes.
- Early diagnosis and appropriate surgical intervention are crucial for long-term success in HSCR management.
Abstract:
Hirschsprung's disease is rarely seen in the young adult, and presents unique problems in management because of the massive dilatation and hypertrophy that occur proximal to the aganglionic rectum or the rectosigmoid colon. The diagnosis, which may be suspected by barium enema, is confirmed by suction or full-thickness biopsy of the rectum that may be complemented by anal manometry. Based on our experience with eight patients, a two-stage surgical reconstruction is recommended, with a preliminary sigmoid colostomy through the normally innervated colon and an associated defunctionalized stoma constituting the initial operation. The distal colonic stoma permits cleansing of the caudal colon while the normally innervated proximal colon reverts to near normal caliber, usually within 2 to 6 months. This approach is in accord with the recommendation of Fairgrieve. Reconstruction using a Duhamel or Soave procedure has given good results. The Duhamel procedure seems preferable when a considerable discrepancy remains between the ganglionic and aganglionic segments of rectum.