Myectomy for Hirschsprung Disease

Mike Chen, MD, Andrew Zigman, MD, CM, Ankush Gosain, MD, PhD
Myectomy for Hirschsprung Disease is a topic covered in the Pediatric Surgery NaT.

To view the entire topic, please or purchase a subscription.

APSA Pediatric Surgery Library combines Pediatric Surgery Not a Textbook (NaT) with APSA ExPERT, a powerful platform for earning MOC CME credits -- all powered by Unbound Medicine. Explore these free sample topics:

Pediatric Surgery Library

-- The first section of this topic is shown below --

Steps of the Procedure

How is a myectomy performed?

Lynn initially described this operation via a transanal approach. A transverse incision is made approximately one cm from the dentate line in the posterior rectal wall [1]. A mucosal flap is raised proximally and a 0.5 to 1 cm strip of muscle is excised. The length of the myectomy corresponds with the pathologic process and is usually several cm long. The mucosal flap is closed at the completion of the myectomy. Thomas et al advocated approaching the posterior rectum, in a fashion that would later be popularized by Pena for imperforate anus, via an incision from the coccyx to the posterior rectum. Once exposed, the overlying rectal serosa is divided in a longitudinal fashion and the myectomy is performed. Care is taken not to enter the mucosa in a similar fashion to a pyloromyotomy [2][3]. When a myectomy is performed for short segment Hirschsprung disease, the operation is both diagnostic and potentially therapeutic. In the case where the aganglionosis extends beyond the proximal margin a pull through operation is required.

-- To view the remaining sections of this topic, please or purchase a subscription --

Last updated: May 6, 2016