Severe colitis in late-onset Hirschsprung's disease.

Most patients with Hirschsprung's disease have symptoms of constipation and abdominal distension in the first several months of life. The development of enterocolitis in these patients, characterized by diarrhea, abdominal distension, and vomiting, is most common in the first 3 months of life and has been associated with a 30-50% mortality. We report a child with previously normal daily bowel movements who first developed constipation at 16 months of age; only when severe colitis and chronic lower gastrointestinal bleeding appeared at 3.5 years of age was the diagnosis of Hirschsprung's disease made.