Unexpected death from enterocolitis after surgery for Hirschsprung's disease.

Unanticipated death from enterocolitis occurred in five children 3 weeks to 20 months after uncomplicated reconstruction for Hirschsprung's disease. In each case the presenting symptoms of enterocolitis were mild and were misinterpreted by examining physicians. Within 2 to 12 days of onset of symptoms, unexpected death occurred. Although fatal enterocolitis is a well-known complication of Hirschsprung's disease, emphasis is usually placed on preoperative enterocolitis. Fatal postoperative enterocolitis is not a new entity associated with Hirschsprung's disease, but physician awareness of this possibility is obviously deficient. We strongly recommend extensive parent education and better postoperative communication between the surgeon and the referring physician.