Long-term continence after surgery for Hirschsprung's disease

Aim: Our aim was to examine the long-term bowel dysfunction that followed surgery for Hirschsprung's disease. Methods: Of 414 patients diagnosed with Hirschsprung's disease between 1974 and 2002, 98 were interviewed using a structured questionnaire to provide an assessment of bowel function, medication, diet, physical and social limitations. Forty-two completed a prospective 4-week toileting diary and 16 underwent anorectal manometry. Results: Four of the 98 patients had permanent stomas and 10 had Down's syndrome. Of the remaining 84 patients (mean age 12 +/- 8 years, range 1.9-41.9 years), 13% (11/84) had heavy soiling by day and 17% (14/84) by night. Fifty percent reported episodic urgency, but 36% also reported episodic constipation. Stool consistency was looser in patients with a history of long segment disease. Some aspects of bowel function improved with age. Enuresis was much more frequent than expected. Sixty-four percent reported adverse reactions to foods, particularly to fruit, vegetables, fats and diary products, and 15% limited their social activities because of fecal incontinence. There were no significant differences in manometric parameters between those patients who soiled and those who did not. Conclusions: Fecal incontinence is common after surgery for Hirschsprung's disease and has a significant impact on social activities. Some aspects of bowel function did improve with age. Adverse reactions to food were unexpectedly frequent and need to be further studied.