Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 81 patients

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Quality of Life Research

Background:

• Hirschsprung's disease (HD) understanding has advanced, yet long-term outcomes and patient/parent perspectives require clarification.
• Treatment success in HD can be subjective, highlighting the need for standardized outcome assessments.
• The pediatric incontinence/constipation scoring system (PICSS) offers a validated tool for evaluating functional outcomes in children with HD.

Purpose of the Study:

• To analyze quality of life and functional outcomes in children who underwent surgery for Hirschsprung's disease.
• To assess the effectiveness of the PICSS in evaluating continence and constipation in pediatric HD patients.
• To compare surgeon and patient/parent perceptions of treatment outcomes in HD.

Main Methods:

• Retrospective questionnaire-based study of children operated for HD between 1995 and 2017.
• Utilized the PICSS to categorize patients based on continence and constipation status.
• Collected data on surgical procedures, including Soave procedure and colostomy rates.

Main Results:

• 66.6% of 81 participating patients achieved normal bowel function post-surgery.
• 4.9% experienced incomplete continence, and 16% had persistent constipation.
• The Soave procedure was the primary surgical approach in 96% of cases.

Conclusions:

• Surgical outcomes for Hirschsprung's disease may not always align with surgeon expectations; long-term follow-up is essential.
• Most school-aged children show improvement in fecal continence over time, with potential for late adolescent development.
• Standardized scoring systems like PICSS are vital for objective assessment of long-term functional outcomes in pediatric HD patients.