Appendiceal perforation, pneumoperitoneum, and Hirschsprung's disease.

In this report, we describe an infant with appendiceal perforation due to total colonic Hirschsprung's disease, to emphasize this rare but important presentation of Hirschsprung's disease. Based on our own experience and a survey of similar cases, we suggest that an underlying etiology, namely long-segment Hirschsprung's disease, should be suspected in infants with a perforated appendix, especially when a radiographic pneumoperitoneum is present. Prompt diagnosis and appropriate therapy is essential in this situation to prevent morbidity and mortality.