Natural history of adult Hirschsprung's disease.

The natural history of adult Hirschsprung's disease was reviewed in 22 patients (four from our hospital and 18 from the literature). The diagnosis in nearly half the patients was made before age 30, and the oldest patients were 69 years of age. Male to female ratio was 4:1. Almost all patients had severe constipation since birth. Barium enema showed rectal narrowing in 77%, and colonic dilation in 100%. Anorectal manometry was performed in only four patients; three showed no relaxation with rectal distention. In all patients, rectal biopsy showed no ganglion cells. Appropriate operation for Hirschsprung's disease improved symptoms in over 80% of patients. Hirschsprung's disease should be considered in all adult patients who have had severe constipation since birth, especially if they are male.