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Hirschsprung's disease in children with Down syndrome: a comparative study
D Travassos1, M van Herwaarden-Lindeboom, D C van der Zee
1University Medical Centre Utrecht, Pediatric Surgery, Utrecht, Netherlands. d.vieira-travassos@umcutrecht.nl
Summary
Children with Down syndrome (DS) undergoing surgery for Hirschsprung's disease experience more complications and longer hospital stays. Long-term, they face higher rates of severe constipation and enterocolitis compared to those without DS.
Area of Science:
- Pediatric Surgery
- Clinical Outcomes
- Genetics
Background:
- Hirschsprung's disease (HSCR) is a congenital disorder affecting the large intestine.
- Outcomes following surgical correction of HSCR in children with Down syndrome (DS) remain controversial.
- This study compares surgical outcomes in children with and without DS.
Purpose of the Study:
- To compare the surgical outcomes of Hirschsprung's disease in children with Down syndrome (DS) versus those without Down syndrome (NDS).
- To analyze the impact of laparoscopic versus open Duhamel procedures in DS patients.
- To evaluate postoperative complications, hospital stay, and long-term sequelae.
Main Methods:
- Retrospective analysis of 149 children operated on for Hirschsprung's disease between 1987 and 2008.
- Comparison of 20 children with DS to 129 children without DS (NDS).
- Evaluation of open or laparoscopic Duhamel procedures, focusing on postoperative complications, hospital stay, enterocolitis, constipation, and incontinence.
Main Results:
- Children with DS had significantly higher rates of postoperative leakage (25% vs 0.7%) and related abscesses (15% vs 0%).
- Postoperative hospital stay was significantly longer in the DS group (p<0.05).
- DS patients experienced higher incidences of severe constipation (55% vs 22.3%) and enterocolitis (45% vs 31%) during long-term follow-up.
Conclusions:
- Children with Down syndrome undergoing surgery for Hirschsprung's disease face increased postoperative complications and extended hospital stays.
- Long-term follow-up reveals a higher prevalence of severe constipation and enterocolitis in DS patients.
- Laparoscopic and open Duhamel procedures showed no significant difference in major complications within the DS group.
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