Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung Disease

Area of Science:

• Pediatric surgery
• Gastroenterology
• Quality of life research

Background:

• Hirschsprung disease (HSCR) is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
• Long-term outcomes and functional differences based on the extent of aganglionosis in HSCR patients are not fully understood.
• Understanding these differences is crucial for optimizing patient management and predicting long-term health trajectories.

Purpose of the Study:

• To compare long-term bowel function and generic quality of life in patients with Hirschsprung disease based on the length of aganglionic segments.
• To investigate the association between aganglionosis length (rectosigmoid, long-segment, total colonic) and specific bowel issues like fecal incontinence and constipation.
• To assess the impact of aganglionosis extent on the overall quality of life in adult HSCR patients.

Main Methods:

• A nationwide, cross-sectional study involving 334 patients with proven Hirschsprung disease.
• Participants completed validated questionnaires assessing defecation, fecal continence, and generic quality of life (Child Health Questionnaire Child Form-87 or WHO Quality of Life-100).
• Exclusion criteria included deceased patients, those younger than 8 years, living abroad, permanent enterostomy, or intellectual impairment.

Main Results:

• Fecal incontinence prevalence was similar across rectosigmoid, long-segment, and total colonic aganglionosis groups.
• Liquid fecal incontinence was significantly associated with total colonic aganglionosis (OR = 6.00).
• Constipation was less frequent in total colonic or long-segment aganglionosis compared to rectosigmoid aganglionosis (ORs < 0.91).
• Overall quality of life was comparable, but physical scores were lower in the total colonic aganglionosis group.

Conclusions:

• Hirschsprung disease patients with long-segment or total colonic aganglionosis do not experience worse general fecal incontinence long-term compared to rectosigmoid cases.
• Differences in stool consistency may explain the higher rates of liquid fecal incontinence in total colonic aganglionosis and constipation in rectosigmoid aganglionosis.
• Despite functional variations, generic quality of life in adulthood is comparable across different aganglionosis lengths in Hirschsprung disease patients.