Surgical management of short-segment Hirschsprung disease

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 ca...

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Bibliographic Details
Main Authors: Haley Etskovitz, Rosa S Kim, Sarah Ziqi Wang, Prathima Nandivada
Format: Article
Language:English
Published: BMJ Publishing Group 2024-12-01
Series:World Journal of Pediatric Surgery
Online Access:https://wjps.bmj.com/content/7/4/e000916.full
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Summary: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.
ISSN:2516-5410