Adult Hirschsprung's disease presenting as sigmoid volvulus: a case report and review of literature.
Sigmoid volvulus is a rare presentation of Hirschsprung's disease. A 38-year-old male presented with acute intestinal obstruction and a history of chronic constipation since childhood. Abdominal radiographs showed megarectum and megacolon with dissipated feces. Sigmoidoscopy revealed gangrenous bowel mucosa affecting the sigmoid colon. Emergency laparotomy revealed a grossly dilated bowel with concurrent gangrenous sigmoid volvulus. He was treated successfully with proctocolectomy with J-pouch-anal anastomosis and a defunctioning ileostomy. Histological analysis was consistent with short segment Hirschsprung's disease. Although uncommon, adult Hirschsprung's disease is a cause of chronic constipation and can present acutely with a sigmoid volvulus. Mortality in cases with sigmoid volvulus is greater than in cases without (15.4% vs. 0%). A better awareness of this condition will facilitate management.
Tan
F L S
FL
Department of General Surgery, Singapore General Hospital, Singapore. feliciates@hotmail.com
Tan
Y-M
YM
Heah
S M
SM
Seow-Choen
F
F
D000328:Adult; D006627:Hirschsprung Disease / Q000150:complications* / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D045822:Intestinal Volvulus / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D008297:Male; D011859:Radiography; D012810:Sigmoid Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery