Long‐term continence after surgery for Hirschsprung's disease

Abstract

Our aim was to examine the long-term bowel dysfunction that followed surgery for Hirschsprung's disease. 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. 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. 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.