Role of anorectal myectomy after failed endorectal pull-through in Hirschsprung's disease

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Colorectal Surgery

Background:

• Hirschsprung's disease (HD) is a congenital condition affecting the large intestine.
• Endorectal pull-through (ERPT) is a common surgical treatment for HD.
• Some patients experience persistent symptoms post-ERPT, necessitating further intervention.

Purpose of the Study:

• To evaluate the efficacy of Anorectal Myectomy (ARM) in patients with Hirschsprung's disease who have residual symptoms after ERPT.
• To determine if ARM can alleviate persistent high anal canal pressure and associated symptoms.

Main Methods:

• Retrospective analysis of 37 patients who underwent ERPT for HD.
• Six patients with persistent symptoms (constipation, distension, enterocolitis) unresponsive to conservative therapy were identified.
• Anorectal manometry was performed, revealing high anal canal pressure in these patients.
• ARM, including posterior rectal myectomy and partial internal sphincterotomy, was performed 6-55 months post-ERPT.

Main Results:

• Five of the six patients experienced marked improvement in symptoms after ARM.
• One patient showed a partial response to ARM.
• All six patients demonstrated a significant reduction in anal canal pressure post-ARM.
• No patients required a secondary pull-through operation.

Conclusions:

• Anorectal myectomy is a recommended and effective surgical option for managing persistent symptoms and high anal canal pressure following ERPT in Hirschsprung's disease patients.
• ARM should be considered before pursuing more extensive secondary pull-through procedures.
• Early intervention with ARM can improve outcomes and quality of life for select HD patients.