Long-term outcome after Hirschsprung's disease: Patients' perspectives

Background: The results of treatment of Hirschsprung's disease (HD) are not uniformly successful, and the parents' and child's interpretation of the o utcome may be different from that of the surgeon. Methods: One hundred seven children treated for HD over 221/2 years were re viewed retrospectively. Adequate clinical data regarding functional outcome s were available in 78, of which, an additional questionnaire was completed by 69. Follow-ups were divided into type of pu I I-th rough (PT), age at t ime of PT (<4 months and >4 months) and age at last follow-up (<5 years, 5 to 15 years and >15 years). Degrees of constipation and incontinence were d etermined using standard scoring systems. Results: The median age at presentation was 9 days (range, 1 day to 9.4 yea rs), and 41% presented within the first 72 hours of life. Aganglionosis ext ended to the rectosigmoid region in 75%, proximal to the splenic flexure in 11%, and total colon in 6.5%. Treatments included a Soave procedure in 57, Duhamel in 31, Swenson in 10, and sphincterotomy-myectomyin two. No surgic al treatment or only a decompressing enterostomy was performed in two and t hree children, respectively, and two underwent a PT elsewhere. Complication s related to the enterostomy occurred in 39 patients. Postoperative enteroc olitis and bowel obstructions occurred in nine and 13 patients, respectivel y. Staple-line fusion, anastomotic stenosis, and anastomotic leak occurred in nine, 17, and two patients, respectively. Seven children died, only one directly related to the PT. Aside from more constipation associated with th e Duhamel procedure, functional outcomes were not significantly different a mong the types of PTs. No differences were found between patients who under went a Pi at less than 4 months of age and those at greater than 4 months o f age. Significantly, however, fecal continence was relatively poor in thos e less than 15 years of age (50% with only fair to poor continence), but im proved markedly once the child reached later adolescence (8%, P < .0002). C orrespondingly, the negative impact on the child's social life was much gre ater in the younger age groups compared with later adolescence (50% of thos e aged 5 to 15 years v 18% of those >15 years; P = .007). The effect on the families' lives mirrored this. Finally, only 64% of patients were interpre ted as having "normal" stooling habits, yet 90% of parents were moderately or very satisfied with their child's outcome, with no differences found amo ng the three sets of comparison groups. Conclusions.. The outcomes for HD are not always as good as surgeons may pe rceive; long term follow-up is important. With time, most children signific antly improve with respect to fecal continence, but this may not be until l ater adolescence. In the meantime, the impacts on their social and family l ives may be significant. Despite high complication rates, often poor contin ence, and relative infrequency of normal stooling habits, most parents are satisfied with their child's outcome and adapt to their functional abnormal ities along with them. J Pediatr Surg 34:1152-1160. Copyright (C) 1999 by W .B. Saunders Company.