Bowel perforation in Hirschsprung's disease.

A review of the medical records of 45 infants less than 1 year old with Hirschsprung's disease identified two (4.4%) who presented with bowel perforation. A literature survey was done to evaluate the relationship between bowel perforation of the bowel early in the course of Hirschsprung's disease indicates that: the infant under 4 months old is at the greatest risk; the majority of cases (62%) were associated with long-segment or total colonic Hirschsprung's disease; the most common sites of perforation were the proximal colon (68%) and appendix (17%); in cases with a short or intermediate length of aganglionic bowel, the perforation was proximal to or at the site of transition, but in 84% of infants with total colonic aganglionosis the perforation was situated in aganglionic bowel. Hirschsprung's disease should be a prime differential consideration in a young infant with penumoperitoneum caused by distal bowel perforation.