{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T19:52:41Z","timestamp":1790452361903,"version":"4.1.0"},"reference-count":164,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T00:00:00Z","timestamp":1777507200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T00:00:00Z","timestamp":1777507200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100000780","name":"European Commission","doi-asserted-by":"publisher","award":["ERNICA2023\u20102027, project no. 101155831"],"award-info":[{"award-number":["ERNICA2023\u20102027, project no. 101155831"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The update followed a structured five\u2010step process: identifying new evidence (2018\u20132024), prioritizing recommendations using the Update Priority Tool, and revising selected topics using the Grading of Recommendations Assessment, Development and Evaluation\u2010Evidence 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>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\u2010up, transition to adult care, and the role of botulinum toxin in managing Hirschsprung's\u2010associated enterocolitis (HAEC). Routine preventive anal dilatations were no longer recommended. The guideline highlights the importance of structured, multidisciplinary, and patient\u2010centered care throughout the lifespan and identifies several research gaps, including nutrition, transition of care, fertility, and HAEC definitions.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This updated guideline reflects current evidence and expert consensus, supporting equitable, patient\u2010centered 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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpn3.70442","type":"journal-article","created":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T08:36:01Z","timestamp":1777538161000},"page":"185-207","source":"Crossref","is-referenced-by-count":8,"title":["Updated  European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies guidelines for the management of rectosigmoid Hirschsprung's disease 2025"],"prefix":"10.1002","volume":"83","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1430-596X","authenticated-orcid":false,"given":"Daniel","family":"Rossi","sequence":"first","affiliation":[{"name":"Department of Pediatric Surgery Erasmus MC Sophia Children's Hospital Rotterdam the Netherlands"},{"name":"Departments of Women's and Children's Health and Division of Pediatric Surgery Astrid Lindgren Children's Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Willemijn","family":"Irvine","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Erasmus MC Sophia Children's Hospital Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna L\u00f6f","family":"Granstr\u00f6m","sequence":"additional","affiliation":[{"name":"Departments of Women's and Children's Health and Division of Pediatric Surgery Astrid Lindgren Children's Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annika","family":"Mutanen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Children's Hospital University of Helsinki Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roel","family":"Bakx","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Emma Children's Hospital, Amsterdam UMC University of Amsterdam and Vrije Universiteit Amsterdam Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristin","family":"Bj\u00f8rnland","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Oslo University Hospital and University of Oslo Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Duccio","family":"Cavalieri","sequence":"additional","affiliation":[{"name":"Department of Biology University of Florence, A. 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