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Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document Massimi, Luca Peretta, Paola Erbetta, Alessandra Farinotti, Mariangela Ciaramitaro, Palma Poca Pastor, Ma Antonia Sahuquillo Barris, Joan Solari, Alessandra Institut Català de la Salut [Massimi L] Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy. [Peretta P] Pediatric Neurosurgery, AOU Citta’ della Salute e della Scienza di Torino, Torino, Italy. [Erbetta A] Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. [Solari A, Farinotti M] Neuroepidemiology Unit – Scientific Directorate, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. [Ciaramitaro P] Department of Neuroscience, AOU Citta’ della Salute e della Scienza di Torino, Torino, Italy. [Poca MA, Sahuquillo J] Unitat de Recerca en Neurotrauma i Neurocirurgia, Servei de Neurocirurgia i Neurocirurgia Pediàtrica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain Vall d'Hebron Barcelona Hospital Campus Tub neural - Malformacions Malalties - Presa de decisions Diagnòstic INFORMATION SCIENCE::Information Science::Systems Analysis::Delphi Technique NAMED GROUPS::Persons::Age Groups::Child DISEASES::Nervous System Diseases::Nervous System Malformations::Neural Tube Defects::Arnold-Chiari Malformation Other subheadings::Other subheadings::/therapy CIENCIA DE LA INFORMACIÓN::Ciencias de la información::análisis de sistemas::técnica Delfos DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño ENFERMEDADES::enfermedades del sistema nervioso::malformaciones del sistema nervioso::defectos del tubo neural::malformación de Arnold-Chiari Otros calificadores::Otros calificadores::/terapia Malformació de Chiari 1; Nens; Siringomielia Malformación de Chiari 1; Niños; Siringomielia Chiari 1 malformation; Children; Syringomyelia Background Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children. Methods A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale (“strongly disagree,” “disagree,” “agree,” “strongly agree”). Statements that were endorsed (“agree” or “strongly agree”) by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three). Results Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the “definition of radiological failure 24 month post-surgery.” Conclusions The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population. Open access funding provided by Università Cattolica del Sacro Cuore within the CRUI-CARE Agreement. 2022-02-22T07:45:30Z 2022-02-22T07:45:30Z 2021 2022-02 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Massimi L, Peretta P, Erbetta A, Solari A, Farinotti M, Ciaramitaro P, et al. Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document. Neurol Sci. 2022 Feb;43:1311–1326. 1590-3478 https://hdl.handle.net/11351/7055 10.1007/s10072-021-05317-9 34097175 000658596200001 http://hdl.handle.net/11351/7055 eng Neurological Sciences;43 https://doi.org/10.1007/s10072-021-05317-9 Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess application/pdf Springer Scientia