Surgical management of short-segment Hirschsprung disease

Abstract

Hirschsprung disease (HSCR) is the most common congenital motility disorder of the intestine, characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, leading to functional bowel obstruction. Short-segment Hirschsprung disease (SS-HSCR) accounts for the majority of cases, with surgical resection being the cornerstone of treatment. Despite advances in surgical techniques, considerable variability exists in practice regarding the timing of surgery, the choice of technique, and the length of aganglionic rectal cuff to resect. This review synthesizes the current evidence surrounding surgical management of SS-HSCR, discussing technique-specific outcomes and areas for future research, with a focus on optimizing patient care and functional outcomes.

Keywords: Colorectal Surgery; Evidence-Based Medicine; History Of Medicine.

Figure 1. Illustration of various techniques for pull through procedure in the treatment of Hirschsprung disease. Ganglia are depicted as yellow dots. (A) The Swenson procedure achieves end-to-end anastomosis by resecting the aganglionic bowel down to the sphincters. (B) The Duhamel procedure involves creating a retrorectal reservoir to connect ganglionic bowel to the rectum while preserving part of the aganglionic rectum. (C) The Yancey-Soave procedure creates a submucosal tunnel for the pull through of the ganglionic bowel while sparing the aganglionic rectal muscular cuff.

Figure 2. Intraoperative image of laparoscopic mobilization of the rectum and dissection in the pelvis for short-segment Hirschsprung disease.

Figure 3. Depiction of transanal endorectal pull through (TEPT) for short-segment Hirschsprung disease. (A) Use of Lone Star retractor to aid in identification of the appropriate dissection plane. (B) Pulled through colon prior to excision and reconstructive anastomosis.