Bowel function and quality of life in adult patients with operated Hirschsprung's disease

Abstract

Fecal continence and quality of life were evaluated by a questionnaire in 100 patients (mean age 31 years, 88 males, 12 females) who had undergone surgery for Hirschsprung's disease (HD) during the years 1950–1975. The operative methods were Duhamel retrorectal pull-through or modified Duhamel operation in 71 patients, Swenson rectosigmoidectomy in 20, State-Rehbein anterior resection in 5, and Soave endorectal pull-through in 4. The postoperative follow-up period ranged from 15 to 39 years. Fecal continence was assessed by a score described by Holschneider. Eighty-one healthy people with a similar age and sex distribution as the patients were used as controls. All controls and 91 patients had good fecal continence scores. Of the 9 patients who had fair scores, 3 were mentally retarded and 3 had postoperative anastomotic complications. If these 6 patients with an obvious cause for deficient anal function are excluded, there was no statistically significant difference in fecal continence between the patients and the controls. Equally good continence outcomes were achieved with all the operative methods. In adulthood, only 1 patient had chronic constipation. However, during childhood constipation was the main problem in 40 patients who developed a recurrent rectocolonic septum following the Duhamel operation. Constipation subsided after recrushing of the septum. None of the patients had urinary incontinence. All the patients with a good continence outcome reported no limitations in their occupation, social life, or physical activities. We conclude that in adulthood the fecal continence and quality of life of most patients with operated HD do not differ from those of healthy adults.