Adult Hirschsprung's disease

Two cases of adult Hirschsprung's disease are reported along with a review of 47 cases reported in the literature. Chronic constipation is present in almost all cases. The barium enema examination may suggest the diagnosis if an area of smooth narrowing with proximal dilatation is seen (83%), but biopsy proof of absence of ganglion cells is necessary. Fourteen per cent of patients had a dilatated colon without a narrowed segment. Anal manometry is helpful if it demonstrates absence of normal anorectal inhibitory reflex. Total colonic aganglionosis in the adult has been reported, but is exceptionally unusual. The diagnosis of adult Hirschsprung's disease should be suspected in patients with a history of chronic constipation and appropriate radiographic findings.