Hirschsprung's disease complicating colon atresia.

An infant with the rare association of colon atresia, total colonic Hirschsprung's disease, and tetralogy of Fallot is presented. While Hirschsprung's disease associated with atresia of the colon has been reported five times previously, this is the first case with associated congenital cardiac disease. As in previous reports, initial operations to relieve obstruction due to the colon atresia failed because of the unrecognized aganglionosis. This infant initially had an ileostomy performed as a newborn and then two failed attempts at closure of the ileostomy. In this case, as in four previously reported cases, histological examinations of resected colon from the initial operations were first reported as normal and only under the pressure of increasing clinical evidence was the pathology reviewed and the absence of ganglion cells appreciated. We stress the importance of suspecting the coexistence of Hirschsprung's disease and colon atresia when a functional obstruction is present after repair of the atresia.