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Long-term anorectal function after Duhamel operation for Hirschsprung's disease
H A Heij1, X de Vries, I Bremer
1Paediatric Surgical Centre Amsterdam, Academic Medical Centre/Free University Hospital, The Netherlands.
Insights
Long-term outcomes for children undergoing Duhamel
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Colorectal Surgery
Background:
- Hirschsprung's disease is a congenital disorder affecting the large intestine.
- The Duhamel procedure is a surgical technique used to treat Hirschsprung's disease.
- Long-term anorectal function after surgery is a critical outcome measure.
Purpose of the Study:
- To assess long-term anorectal function in children treated with the Duhamel technique for Hirschsprung's disease.
- To identify factors influencing functional outcomes.
- To evaluate the effectiveness of a detailed questionnaire in assessing patient status.
Main Methods:
- Retrospective review of 75 patients operated on between 1977 and 1991.
- Analysis of detailed questionnaires completed by 63 patients over 2 years of age.
- Categorization of patients based on the extent of aganglionosis (rectosigmoid, long segment, total colon).
Main Results:
- No significant correlation found between age or sex and anorectal function.
- Long segment aganglionosis did not result in worse function than rectosigmoid aganglionosis.
- Only 1 of 7 patients with total colonic aganglionosis achieved continence; symptoms are often underreported due to patient adaptation.
Conclusions:
- The Duhamel technique's long-term functional outcomes vary, with total colonic disease posing significant challenges.
- Detailed questionnaires are reliable tools for assessing actual anorectal function in pediatric Hirschsprung's disease patients.
- Systematic follow-up is crucial for early detection and management of complications.
Abstract:
Long-term anorectal function was assessed in children operated on using Duhamel's technique for Hirschsprung's disease. The files of 75 patients (16 girls and 59 boys) operated on between 1977 and 1991 were reviewed. Questionnaires were analysed on 63 (12 girls and 51 boys) over 2 years of age. The median age at completing the questionnaire was 6.6 years. Forty-one of these 63 patients had aganglionosis of the rectosigmoid, 15 of a long colonic segment, and 7 of the total colon. Of 14 children age 4 or less, 6 had severe constipation, whereas 8 had regular spontaneous defaecation. Ten of the 49 over 4 years of age were continent without constipation, 22 had soiling and/or constipation, and 17 were incontinent, one of whom had a permanent colostomy. There was no correlation between age or sex and anorectal function. Anorectal function in children with long segment was not worse than in those with rectosigmoid aganglionosis, but only 1 of 7 with total colonic aganglionosis was continent. Because many adapt themselves to the handicap, symptoms are often underreported. A detailed questionnaire appears to be a reliable tool for elucidating the real situation. A systematic follow-up of patients with Hirschsprung's disease is proposed to anticipate the complications and to institute proper measures at an early stage.