A Case of Adult Hirschsprung's Disease with Obstructive Colitis

A case of adult Hirschsprung's disease with obstructive colitis is reported. A 28-year-old woman hed been suffering from severe constipation and abdominal distension since childhood. Although constipation tendency continued, she was able to have a bowel movement without cathartics after several days. Recently constipation gradually worsened, and she was admitted to our hospital complaining of abdominal distension and vomiting. Barium enema examination revealed a typical change in caliber and dilatation of the sigmoid colon with partial stenosis. Colonscopy revealed a narrowed rectum, a dilatated sigmoid colon with segmental stesosis and a map-like ulcer, and normal mucosa lying between the narrow rectum and the ulcer. Manometry revealed loss of anorectal reflex. Microscopic findings of the map-like ulcer showed a nonspecific inflammation and ganglion cells were present in the dilatated sigmoid colon, but absent in the rectum. In addition, acetylcholine esterase staining revealed the presence of hypertrophied nerve fibers in the rectal biopsy specimen.