Appendiceal perforation: a potentially lethal initial mode of presentation of Hirschsprung's disease.

In Hacettepe University Children's Hospital, between 1976 and 1993 two patients among 302 with Hirschsprung's disease were diagnosed with appendiceal perforation (AP) at initial admission. Both patients were less than 2 months of age. One of them was a boy with total colonic aganglionosis and the latter a girl with long-segment disease. In both cases the site of AP was the base, and periappendicitis without mucosal involvement was detected. The present cases and review of the literature suggest that longer aganglionic segment carries a higher risk of AP.