Updated ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease 2025

OBJECTIVES: To revise the 2018 European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies (ERNICA) clinical guideline for the management of rectosigmoid Hirschsprung's disease (HSCR) based on new evidence and evolving clinical priorities, ensuring continued relevance, trustworthiness, and consistency in care. METHODS: The update followed a structured five-step process: identifying new evidence (2018-2024), prioritizing recommendations using the Update Priority Tool, and revising selected topics using the Grading of Recommendations Assessment, Development and Evaluation-Evidence to Decision (GRADE EtD) framework. A multidisciplinary panel of pediatric surgeons, patient representatives, and methodologists from 12 countries oversaw the process. Revised recommendations were developed through evidence review and consensus meetings. RESULTS: Ten prioritized clinical topics were revised. Key updates include refined recommendations on histopathological staining in diagnosis, preoperative screening for renal anomalies and malnutrition, postoperative anastomotic calibration, individualized dietary guidance, sexual and fertility health during follow-up, transition to adult care, and the role of botulinum toxin in managing Hirschsprung's-associated enterocolitis (HAEC). Routine preventive anal dilatations were no longer recommended. The guideline highlights the importance of structured, multidisciplinary, and patient-centered care throughout the lifespan and identifies several research gaps, including nutrition, transition of care, fertility, and HAEC definitions. CONCLUSION: This updated guideline reflects current evidence and expert consensus, supporting equitable, patient-centered HSCR care. Implementation will be supported via the European Pediatric Surgical Audit (EPSA), and future updates will be informed by registry data and ongoing research.

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Publication Details

Journal
Journal of Pediatric Gastroenterology and Nutrition
Published
2026-04-30
DOI
https://doi.org/10.1002/jpn3.70442
Primary Topic
Congenital gastrointestinal and neural anomalies
Type
article
Field-Weighted Citation Impact
0.00

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article

Updated ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease 2025

Rony Sfeir, Marietta Jank, Fabio Fusaro, Roxana Rassouli‐Kirchmeier et al.
Journal of Pediatric Gastroenterology and Nutrition
Congenital gastrointestinal and neural anomalies
article

Updated ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease 2025

Rony Sfeir, Marietta Jank, Fabio Fusaro, Roxana Rassouli‐Kirchmeier, Alessio Pini Prato, Miriam Wilms, Martin Lacher, Roel Bakx, Anne Dariel, Nagoud Schukfeh, Louise Montalva, Rene M. H. Wijnen, Lovisa Telborn, Daniel Rossi, Lucie Poš, Annette Lemli, Pernilla Stenström, Piotr Czauderna, Francesco Fascetti Leon, Tomas Wester, Dirk‐Jan Gloudemans, Anna Löf Granström, Pedro Palazon, Olivia Spivack, Peter Kolja Kvist, Kristin Bjørnland, Duccio Cavalieri, Lucas Matthyssens, Mark Ellebæk, Annika Mutanen, Mikko Pakarinen, Konrad Reinshagen, Alejandra Vilanova, Cornelius E J Sloots, Eelke Toxopeus, Willemijn Irvine
article en

Abstract

OBJECTIVES: To revise the 2018 European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies (ERNICA) clinical guideline for the management of rectosigmoid Hirschsprung's disease (HSCR) based on new evidence and evolving clinical priorities, ensuring continued relevance, trustworthiness, and consistency in care. METHODS: The update followed a structured five-step process: identifying new evidence (2018-2024), prioritizing recommendations using the Update Priority Tool, and revising selected topics using the Grading of Recommendations Assessment, Development and Evaluation-Evidence to Decision (GRADE EtD) framework. A multidisciplinary panel of pediatric surgeons, patient representatives, and methodologists from 12 countries oversaw the process. Revised recommendations were developed through evidence review and consensus meetings. RESULTS: Ten prioritized clinical topics were revised. Key updates include refined recommendations on histopathological staining in diagnosis, preoperative screening for renal anomalies and malnutrition, postoperative anastomotic calibration, individualized dietary guidance, sexual and fertility health during follow-up, transition to adult care, and the role of botulinum toxin in managing Hirschsprung's-associated enterocolitis (HAEC). Routine preventive anal dilatations were no longer recommended. The guideline highlights the importance of structured, multidisciplinary, and patient-centered care throughout the lifespan and identifies several research gaps, including nutrition, transition of care, fertility, and HAEC definitions. CONCLUSION: This updated guideline reflects current evidence and expert consensus, supporting equitable, patient-centered HSCR care. Implementation will be supported via the European Pediatric Surgical Audit (EPSA), and future updates will be informed by registry data and ongoing research.

Journal of Pediatric Gastroenterology and Nutrition
Oslo University Hospital (NO), Hospital Sant Joan de Déu Barcelona (ES), University Medical Center Groningen (NL), University of Padua (IT), Radboud University Nijmegen (NL), Universität Hamburg (DE), University of Southern Denmark (DK), Charles University (CZ), Heidelberg University (DE), Hospital Universitario La Paz (ES), Ghent University Hospital (BE), Odense University Hospital (DK), University Hospital Heidelberg (DE), Copenhagen University Hospital (DK), Centre Hospitalier Universitaire de Lille (FR), Medizinische Hochschule Hannover (DE), Assistance Publique Hôpitaux de Marseille (FR), Helsinki Children's Hospital (FI), Emma Kinderziekenhuis (NL), University Hospital in Motol (CZ), University Medical Center Hamburg-Eppendorf (DE), Amalia Kinderziekenhuis (NL), Bambino Gesù Children's Hospital (IT), Italian University Line (IT), Hôpital Robert-Debré (FR), Azienda Sanitaria Locale Alessandria (IT), Skåne University Hospital (SE), Erasmus MC - Sophia Children’s Hospital (NL), St Göran's Children's Hospital (SE), University Medical Centre Mannheim (DE), Hôpital de la Timone (FR), HAS green academy (NL), Gdańsk Medical University (PL), Vrije Universiteit Amsterdam (NL), University of Amsterdam (NL), Leipzig University (DE)
European Commission
Zero hunger
Openalex Percentile: Top 14%
Congenital gastrointestinal and neural anomalies
DOISourceOpenAlex
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