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Hirschsprung's disease: a 20-year experience
R Reding1, J de Ville de Goyet, S Gosseye
1Department of Paediatric Surgery, St-Luc University Clinics, University of Louvain Medical School, Brussels, Belgium.
Journal of Pediatric Surgery
|August 1, 1997
Summary
Surgical treatment for Hirschsprung's disease (HD) in children shows functional improvement over time, particularly for short-segment cases. However, long-term outcomes remain poorer compared to healthy peers, with enterocolitis being a significant concern.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Outcomes Research
Background:
- Hirschsprung's disease (HD) is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- Surgical intervention is the primary treatment for HD, aiming to remove the aganglionic segment.
- Long-term functional outcomes and associated morbidities require continued investigation.
Purpose of the Study:
- To evaluate the long-term functional outcomes of surgical treatment for Hirschsprung's disease in children.
- To assess the incidence of enterocolitis as a complication and cause of mortality.
- To compare functional results with a healthy control group.
Main Methods:
- Retrospective analysis of 59 children treated for biopsy-proven Hirschsprung's disease between 1972 and 1992.
- Categorization of patients based on the extent of aganglionosis (short, long, ultra-short segments).
- Functional outcomes assessed using a novel semiquantitative scoring system evaluating stool evacuation, distention, soiling, and incontinence, compared to healthy controls.
Main Results:
- The majority of patients (75%) had short-segment HD. Enterocolitis was the most common cause of death (5/6) and a major source of morbidity (27% in short-segment, 43% in long-segment).
- Functional outcomes showed progressive improvement in short-segment patients during childhood and adolescence (P = .04).
- Despite improvements, functional outcomes remained significantly poorer than healthy controls across all age groups (P < .01 to P < .05).
Conclusions:
- Surgical management of Hirschsprung's disease leads to functional improvement over time, especially in short-segment disease.
- Enterocolitis remains a critical complication associated with significant mortality and morbidity in HD patients.
- Long-term functional results after surgery for HD are suboptimal when compared to healthy individuals, highlighting the need for ongoing management and research.