Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis

Anna Löf Granström, Willemijn Irvine, Anders Telle Hoel, Merit Tabbers, Kristiina Kyrklund, Francesco Fascetti Leon, Fabio Fusaro, Nikhil Thapar, Anne Dariel, Cornelius E.J. Sloots, Marc Miserez, Annette Lemli, Sabine Alexander, Cecile Lambe, Célia Crétolle, Niels Qvist, Nagoud Schukfeh, Martin Lacher, Duccio Cavalieri, Ernst van HeurnRony Sfeir, Mikko P. Pakarinen, Kristin Bjørnland, Tomas Wester

Research output: Contribution to journalArticleScientificpeer-review

Abstract

Background: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis. Methods: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements. Results: The consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus. Conclusions: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center. Type of Study: Clinical consensus statement. Level of Evidence: 3a.

Original languageEnglish
Article number161565
JournalJournal of Pediatric Surgery
Volume59
Issue number10
Number of pages11
ISSN0022-3468
DOIs
Publication statusPublished - 2024
MoE publication typeA1 Journal article-refereed

Bibliographical note

Publisher Copyright:
© 2024 The Authors

Fields of Science

  • Hirschsprung's disease
  • Long-term management
  • Poor bowel function
  • Surgical treatment
  • Total colonic aganglionosis
  • 3123 Gynaecology and paediatrics
  • 3126 Surgery, anesthesiology, intensive care, radiology

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