N L Yanchar1, P Soucy. 1. Division of Paediatric General Surgery, Children's Hospital of Eastern Ontario, Ottawa, Canada.
Abstract
BACKGROUND: The results of treatment of Hirschsprung's disease (HD) are not uniformly successful, and the parents' and child's interpretation of the outcome may be different from that of the surgeon. METHODS: One hundred seven children treated for HD over 22 1/2 years were reviewed retrospectively. Adequate clinical data regarding functional outcomes were available in 78, of which, an additional questionnaire was completed by 69. Follow-ups were divided into type of pull-through (PT), age at time 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 determined using standard scoring systems. RESULTS: The median age at presentation was 9 days (range, 1 day to 9.4 years), and 41% presented within the first 72 hours of life. Aganglionosis extended 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-myectomy in two. No surgical treatment or only a decompressing enterostomy was performed in two and three children, respectively, and two underwent a PT elsewhere. Complications related to the enterostomy occurred in 39 patients. Postoperative enterocolitis and bowel obstructions occurred in nine and 13 patients, respectively. 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 the Duhamel procedure, functional outcomes were not significantly different among the types of PTs. No differences were found between patients who underwent a PT at less than 4 months of age and those at greater than 4 months of age. Significantly, however, fecal continence was relatively poor in those less than 15 years of age (50% with only fair to poor continence), but improved markedly once the child reached later adolescence (8%, P < .0002). Correspondingly, the negative impact on the child's social life was much greater in the younger age groups compared with later adolescence (50% of those 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 interpreted as having "normal" stooling habits, yet 90% of parents were moderately or very satisfied with their child's outcome, with no differences found among the three sets of comparison groups. CONCLUSIONS: The outcomes for HD are not always as good as surgeons may perceive; long term follow-up is important. With time, most children significantly improve with respect to fecal continence, but this may not be until later adolescence. In the meantime, the impacts on their social and family lives may be significant. Despite high complication rates, often poor continence, and relative infrequency of normal stooling habits, most parents are satisfied with their child's outcome and adapt to their functional abnormalities along with them.
BACKGROUND: The results of treatment of Hirschsprung's disease (HD) are not uniformly successful, and the parents' and child's interpretation of the outcome may be different from that of the surgeon. METHODS: One hundred seven children treated for HD over 22 1/2 years were reviewed retrospectively. Adequate clinical data regarding functional outcomes were available in 78, of which, an additional questionnaire was completed by 69. Follow-ups were divided into type of pull-through (PT), age at time 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 determined using standard scoring systems. RESULTS: The median age at presentation was 9 days (range, 1 day to 9.4 years), and 41% presented within the first 72 hours of life. Aganglionosis extended 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-myectomy in two. No surgical treatment or only a decompressing enterostomy was performed in two and three children, respectively, and two underwent a PT elsewhere. Complications related to the enterostomy occurred in 39 patients. Postoperative enterocolitis and bowel obstructions occurred in nine and 13 patients, respectively. 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 the Duhamel procedure, functional outcomes were not significantly different among the types of PTs. No differences were found between patients who underwent a PT at less than 4 months of age and those at greater than 4 months of age. Significantly, however, fecal continence was relatively poor in those less than 15 years of age (50% with only fair to poor continence), but improved markedly once the child reached later adolescence (8%, P < .0002). Correspondingly, the negative impact on the child's social life was much greater in the younger age groups compared with later adolescence (50% of those 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 interpreted as having "normal" stooling habits, yet 90% of parents were moderately or very satisfied with their child's outcome, with no differences found among the three sets of comparison groups. CONCLUSIONS: The outcomes for HD are not always as good as surgeons may perceive; long term follow-up is important. With time, most children significantly improve with respect to fecal continence, but this may not be until later adolescence. In the meantime, the impacts on their social and family lives may be significant. Despite high complication rates, often poor continence, and relative infrequency of normal stooling habits, most parents are satisfied with their child's outcome and adapt to their functional abnormalities along with them.