A male newborn with colonic atresia and total colonic aganglionosis

A case of colonic atresia (CA) and aganglionosis is presented, which is pro bably the tenth in the English literature. The boy presented on day 3 of li fe with delayed passage of meconium, milk intolerance, and progressive abdo minal distension. A barium enema study was complicated by barium peritoniti s. Emergency peritoneal lavage was done and the intestinal obstruction was relieved by a proximal defunctioning ileostomy; type III CA (Grosfeld class ification) was identified. The proximal atretic end was the blind-ending ca ecum and the distal atretic end commenced in the splenic area as a microcol on extending to the pelvis and replacing the normal colon. Total colonic ag anglionosis(TCA) was confirmed by biopsies. This is the first case in the l iterature of TCA with CA documented before any attempts at colonic resectio n and anastomosis were made. This association highlights the recommendation for biopsy of the micro-colon and rectum when Type III CA (Grosfeld classi fication) is encountered in a newborn. The infant underwent a cholecystecto my and Duhamel-Martin operation at 14 months that was complicated by abdomi nal wound dehiscence; the ileostomy was closed at 22 months of age.