Korean J Neurogastroenterol Motil.
2006 Dec;12(2):170-176.
A Case of Zonal Adult Hirschsprung's Disease
- Affiliations
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- 1Institute for Digestive Research, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea. drjslee@dreamwiz.com
- 2Department of Pathology, Soonchunhyang University College of Medicine, Seoul, Korea.
- 3Department of General Surgery, Soonchunhyang University College of Medicine, Seoul, Korea.
Abstract
- Hirschsprung's disease is associated with congenital megacolon of the colon proximal to a segment that lacks ganglion cells in the myenteric plexus and submucosal plexus. Hirschsprung's disease is usually considered as a pediatric disease entity. In the typical cases, the aganglionosis arises from the anal verge and this extends proximally for varying distances; the lesion mainly involves the rectum or rectosigmoid colon. Zonal segmental involvement is very rare even in children. We experienced a 45-year-old male who suffered from acute intestinal obstruction with a collapsed luminal portion in the distal descending colon and proximal sigmoid colon. The patient underwent surgical resection for segmental constriction. We present here a case of zonal adult Hirschsprung's disease along with a review of the literature.
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