Photo Credit: Jian Fan
The following is a summary of “Ernica Clinical Consensus Statements On Total Colonic And Intestinal Aganglionosis,” published in the April 2024 issue of Pediatrics by Granström et al.
Hirschsprung disease, characterized by the absence of enteric ganglion cells, presents life-threatening challenges in its total colonic and intestinal aganglionosis forms, affecting a small fraction of patients. This project, led by ERNICA, the European Reference Network for Rare Congenital Digestive Diseases, aimed to establish clinical consensus statements on four critical topics: surgical interventions for total colonic and intestinal aganglionosis, management of poor bowel function, and long-term care. A multidisciplinary panel from ERNICA centers collaborated, conducting literature searches and utilizing a modified Delphi process to formulate and refine statements.
The consensus process involved 24 experts from 10 European countries, agreeing on 45 statements after three Delphi rounds. However, the scarcity of high-quality clinical evidence necessitated reliance on expert opinion for most statements, highlighting the challenges in addressing these rare variants of Hirschsprung disease. While 25 statements did not achieve consensus, the consensus statement underscores the importance of multidisciplinary and personalized care in expert centers for patients with total colonic and intestinal aganglionosis. Despite the limitations, this effort provides valuable guidance on surgical interventions, bowel function management, and long-term care for these complex cases, emphasizing the need for tailored approaches in their management.
Source: sciencedirect.com/science/article/pii/S0022346824002951
