Twenty-five years' experience with Hirschsprung's disease.

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Medical Genetics

Background:

• Hirschsprung's disease is a congenital condition affecting the large intestine.
• Surgical intervention, specifically pull-through procedures, is the standard treatment.
• Optimizing surgical timing and technique is crucial for favorable outcomes.

Purpose of the Study:

• To analyze surgical outcomes and complications in Hirschsprung's disease patients.
• To identify factors influencing anastomotic strictures, encopresis, and enterocolitis.
• To provide evidence-based recommendations for surgical management.

Main Methods:

• Retrospective review of 63 patients with biopsy-proven Hirschsprung's disease.
• Analysis of surgical procedures, complications, and long-term follow-up (average 8 years).
• Correlation of outcomes with level of aganglionosis, patient age at surgery, and diversionary procedures.

Main Results:

• Anastomotic strictures were more common with sigmoid colon aganglionosis.
• Postoperative encopresis was higher in endorectal pull-throughs performed before 10 months.
• Selective use of colostomies/enterostomies before pull-through resulted in low enterocolitis rates (0% mortality).

Conclusions:

• Careful assessment of mesenteric vasculature is vital for preventing strictures in sigmoid colon cases.
• Delaying endorectal pull-through procedures until after 10 months may reduce encopresis.
• Prior fecal diversion is recommended for most infants to minimize enterocolitis risk.