Hirschsprung's disease: three decades' experience at a single institution

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Clinical Research

Background:

• Hirschsprung's disease (HSCR) is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
• Treatment involves surgical resection of the aganglionic segment and pull-through of healthy bowel.
• Long-term outcomes and evolving surgical techniques warrant ongoing investigation.

Purpose of the Study:

• To analyze trends in Hirschsprung's disease patient demographics, surgical approaches, and outcomes over three decades.
• To evaluate the incidence and risk factors for post-pull-through enterocolitis.
• To compare the efficacy and complication rates of different pull-through procedures.

Main Methods:

• Retrospective analysis of 250 patients treated for Hirschsprung's disease between the 1960s and 1980s.
• Data collection included patient demographics, disease extent, surgical technique, and postoperative complications.
• Statistical analysis to identify trends and correlations.

Main Results:

• Rectosigmoid disease was most common (58%), with long-segment and total colon involvement in 26% and 12% respectively.
• The male:female ratio was 3.3 overall, decreasing to 2.2 in total colon disease.
• Mean age at diagnosis and surgery significantly decreased over the decades.
• Endorectal pull-through became the predominant technique, replacing the Swenson procedure.
• Post-pull-through enterocolitis incidence increased over time, with higher rates in longer bowel segments and Duhamel procedures.
• Complication rates varied by procedure, with Swenson having the highest (20%) and Duhamel the lowest (13%).

Conclusions:

• Surgical management of Hirschsprung's disease has evolved, with earlier diagnosis and a shift towards endorectal pull-through.
• While overall mortality decreased, post-pull-through enterocolitis remains a significant concern, particularly with certain techniques and disease extent.
• Continued monitoring and refinement of surgical strategies are essential for improving patient outcomes.