Hirschsprung's disease: one surgeon's experience in one institution

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Genetics

Background:

• Hirschsprung's disease (HD) is a congenital disorder affecting the large intestine.
• It is characterized by the absence of ganglion cells in the distal bowel.
• HD predominantly affects males and can present in neonates.

Purpose of the Study:

• To analyze the clinical experience and surgical outcomes of pediatric patients with Hirschsprung's disease.
• To evaluate the efficacy and safety of the Duhamel operation for HD treatment.
• To identify factors influencing surgical outcomes and complications.

Main Methods:

• Retrospective analysis of 137 pediatric patients treated over 12 years.
• Surgical procedures included two-stage Duhamel operation, primary Duhamel operation, and anorectal myectomies.
• Patient data included demographics, clinical presentation, family history, surgical details, and postoperative outcomes.

Main Results:

• Neonatal HD occurred in 51.1% of cases, predominantly short-segment disease (83.2%).
• The Duhamel operation showed reduced postoperative enterocolitis (10.1%) compared to preoperative incidence (25.5%).
• Bowel function normalized within 3 months in 77.3% and within 1 year in 91.6% of patients, with no mortality.

Conclusions:

• The Duhamel operation, especially with a GIA stapler and preliminary colostomy, offers good functional results and acceptable morbidity in treating Hirschsprung's disease.
• Primary Duhamel operation can be safely performed on patients diagnosed after 90 days of life.
• Early diagnosis and appropriate surgical intervention are crucial for favorable outcomes in pediatric Hirschsprung's disease.