Updated European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal…

GuidelineJournal of pediatric gastroenterology and nutrition· Jul 2026

Updated European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies guidelines for the management of rectosigmoid Hirschsprung's disease 2025.

Daniel Rossi, Willemijn Irvine, Anna Löf Granström … Tomas Wester

PathologyGuidelineClinical practice

Updated guidelines revised ten clinical topics for Hirschsprung's disease management.

Updated European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies guidelines for the management of rectosigmoid Hirschsprung's disease 2025.

Daniel Rossi … Tomas Wester

Journal of pediatric gastroenterology and nutrition · Jul 2026

Purpose

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.

More results

Ten prioritized clinical topics were revised.

Routine preventive anal dilatations were no longer recommended.

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Conclusion · 1 of 2

This updated guideline reflects current evidence and expert consensus, supporting equitable, patient-centered HSCR care.

Conclusion · 2 of 2

Implementation will be supported via the European Pediatric Surgical Audit (European Pediatric Surgical Audit), and future updates will be informed by registry data and ongoing research.

Updated European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies guidelines for the management of rectosigmoid Hirschsprung's disease 2025.

Updated European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies guidelines for the management of rectosigmoid Hirschsprung's disease 2025.

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