Evidence map›Paper›PMID 42057764›Full record

GuidelineJournal of pediatric gastroenterology and nutrition2026

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, Annika Mutanen, Roel Bakx, Kristin Bjørnland, Duccio Cavalieri, Piotr Czauderna, Anne Dariel, Mark Ellebæk and 26 more

Abstract readPractice Guideline
In one paragraph

Guideline in Journal of pediatric gastroenterology and nutrition, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed, 2 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

5 citing papers in PubMed, 2 syntheses or guidelines pooled it.

  1. Pooled it
  2. Guideline
  3. Observational
  4. Review
  5. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

36 authors.

Daniel RossiDepartment of Pediatric Surgery, Erasmus MC Sophia Children's Hospital, Rotterdam, the Netherlands.ORCID https://orcid.org/0000-0002-1430-596X
Willemijn IrvineDepartment of Pediatric Surgery, Erasmus MC Sophia Children's Hospital, Rotterdam, the Netherlands.
Anna Löf GranströmDepartments of Women's and Children's Health and Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Stockholm, Sweden.
Annika MutanenDepartment of Pediatric Surgery, Children's Hospital, University of Helsinki, Helsinki, Finland.
Roel BakxDepartment of Pediatric Surgery, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam, the Netherlands.
Kristin BjørnlandDepartment of Pediatric Surgery, Oslo University Hospital and University of Oslo, Oslo, Norway.
Duccio CavalieriDepartment of Biology, University of Florence, A. Mor. Hi, The Italian Association for Hirschsprung's Disease, Florence, Italy.
Piotr CzaudernaDepartment of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Gdansk, Poland.
Anne DarielDepartment of Pediatric Surgery, Assistance Publique Des Hôpitaux De Marseille, Hôpital Timone Enfants, Marseille, France.
Mark EllebækResearch Unit for Surgery, Odense University Hospital and University of Southern Denmark, Odense, Denmark.
Francesco Fascetti LeonPediatric Surgery, Department of Women's and Children's Health, University Hospital of Padua, Padua, Italy.
Fabio FusaroNeonatal Surgery Unit, Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, IRCSS, Rome, Italy.
Dirk-Jan GloudemansPatient Advocacy, Dutch Association for Hirschsprung's Disease, 's-Hertogenbosch, the Netherlands.
Marietta JankDepartment of Pediatric Surgery, University Medical Center Mannheim, Heidelberg University, Mannheim, Germany.
Peter Kolja KvistDepartment of Pediatric Surgery, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark.
Martin LacherDepartment of Pediatric Surgery, University of Leipzig, Leipzig, Germany.
Annette LemliSoMA, The German Patient Support Organization for Anorectal Malformations and Hirschsprung Disease, Munich, Germany.
Lucas MatthyssensDepartment of Gastrointestinal and Paediatric Surgery, Princess Elisabeth Children's Hospital, Ghent University Hospital, Ghent University, Ghent, Belgium.
Louise MontalvaDepartment of Paediatric Surgery, Robert-Debré Children's University Hospital, Paris, France.
Pedro PalazonDepartment of Pediatric Surgery, Hospital Sant Joan de Déu-Universitat de Barcelona, Barcelona, Spain.
Alessio Pini PratoDepartment of Pediatric Surgery, Alessandria Children Hospital, Alessandria, Italy.
Lucie PosDepartment of Paediatric Surgery, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic.
Roxana Rassouli-KirchmeierDepartment of Pediatric Surgery, Amalia Children's Hospital, Radboudumc, Radboud University, Nijmegen, the Netherlands.
Konrad ReinshagenDepartment of Pediatric Surgery, University Medical Center Hamburg-Eppendorf, University of Hamburg, Hamburg, Germany.
Nagoud SchukfehDepartment of Pediatric and Adolescent Surgery, Hannover Medical School, Hannover, Germany.
Rony SfeirDepartment of Pediatric Surgery, Centre Hospitalier Régional Universitaire de Lille, Lille, France.
Olivia SpivackDepartment of Pediatric Surgery, Erasmus MC Sophia Children's Hospital, Rotterdam, the Netherlands.
Pernilla StenströmDepartment of Pediatric Surgery, Skåne University Hospital, Lund, Sweden.
Lovisa TelbornDepartment of Pediatric Surgery, Skåne University Hospital, Lund, Sweden.
Eelke ToxopeusDivision of Paediatric Surgery, Department of Surgery, University Medical Centre Groningen, Groningen, the Netherlands.
Alejandra VilanovaDepartment of Pediatric Surgery, Urogenital and Colorectal Unit, La Paz University Hospital Children Hospital, Madrid, Comunidad de Madrid, Spain.
Miriam WilmsSoMA, The German Patient Support Organization for Anorectal Malformations and Hirschsprung Disease, Munich, Germany.
Rene WijnenDepartment of Pediatric Surgery, Erasmus MC Sophia Children's Hospital, Rotterdam, the Netherlands.
Cornelius E J SlootsDepartment of Pediatric Surgery, Erasmus MC Sophia Children's Hospital, Rotterdam, the Netherlands.
Mikko PakarinenDepartment of Pediatric Surgery, Children's Hospital, University of Helsinki, Helsinki, Finland.
Tomas WesterDepartments of Women's and Children's Health and Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Stockholm, Sweden.

Funding

European Union ERNICA2023-2027European Union ERNICA2023-2027, project no. 101155831
6 · The paper itself

Abstract

objectivesTo 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.

methodsThe 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.

resultsTen 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.

conclusionThis 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.

Indexed as

Hirschsprung DiseaseEuropeHumansconsensuslong‐term outcomesmultidisciplinary carepediatric surgeryrare diseases

Identifiers

PMID42057764
PMCPMC13342790

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.