Trans Anal Rectal Myomectomy Following Martin Left Colon Patch Procedure — A Proven Therapeutic Surgical Technique with Literature Review

Abstract Hirschsprung disease (HD) is a congenital disorder of the enteric nervous system, wherein the intestine lacks ganglion cells owing to inadequate caudal migration of neural crest cells. Total colonic aganglionosis (TCA) and small-intestinal HD (SIHD) tend to present more diagnostic challenges, and identification of these cases calls for a high level of awareness. The Martin left colon patch procedure is the most common technique among the various surgical methods followed. Based on a review of the literature on the surgical management of TCA and SIHD, we report a case of SIHD in a 2-year-old girl who was treated by following a staged approach involving the Martin’s left colon patch procedure and adjunctive trans-anal rectal myomectomy after ileostomy closure. Trans-anal rectal myomectomy can be beneficial for reaching a histological diagnosis and treating patients whose definitive treatment has failed. There is no case of HD managed using a similar method in the literature.