TY - JOUR
T1 - Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis
AU - Granström, Anna Löf
AU - Irvine, Willemijn
AU - Hoel, Anders Telle
AU - Tabbers, Merit
AU - Kyrklund, Kristiina
AU - Leon, Francesco Fascetti
AU - Fusaro, Fabio
AU - Thapar, Nikhil
AU - Dariel, Anne
AU - Sloots, Cornelius E.J.
AU - Miserez, Marc
AU - Lemli, Annette
AU - Alexander, Sabine
AU - Lambe, Cecile
AU - Crétolle, Célia
AU - Qvist, Niels
AU - Schukfeh, Nagoud
AU - Lacher, Martin
AU - Cavalieri, Duccio
AU - van Heurn, Ernst
AU - Sfeir, Rony
AU - Pakarinen, Mikko P.
AU - Bjørnland, Kristin
AU - Wester, Tomas
N1 - Publisher Copyright:
© 2024 The Authors
PY - 2024
Y1 - 2024
N2 - 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.
AB - 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.
KW - Hirschsprung's disease
KW - Long-term management
KW - Poor bowel function
KW - Surgical treatment
KW - Total colonic aganglionosis
KW - 3123 Gynaecology and paediatrics
KW - 3126 Surgery, anesthesiology, intensive care, radiology
U2 - 10.1016/j.jpedsurg.2024.04.019
DO - 10.1016/j.jpedsurg.2024.04.019
M3 - Article
AN - SCOPUS:85193547113
SN - 0022-3468
VL - 59
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 10
M1 - 161565
ER -