Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

Luca Massimi*, Paola Peretta, Alessandra Erbetta, Alessandra Solari, Mariangela Farinotti, Palma Ciaramitaro, Veronica Saletti, Massimo Caldarelli, Alexandre Casagrande Canheu, Carlo Celada, Luisa Chiapparini, Daniela Chieffo, Giuseppe Cinalli, Federico Di Rocco, Marika Furlanetto, Flavio Giordano, George Jallo, Syril James, Paola Lanteri, Christian LemarchandMartina Messing-Jünger, Cecilia Parazzini, Giovanna Paternoster, Gianluca Piatelli, Maria A. Poca, Prab Prabahkar, Federica Ricci, Andrea Righini, Francesco Sala, Juan Sahuquillo, Marcus Stoodley, Giuseppe Talamonti, Dominic Thompson, Fabio Triulzi, Mino Zucchelli, Laura Valentini, International Experts - Jury of the Chiari & Syringomyelia Consensus Conference, “Milan, November 11–13, 2019”

*Corresponding author for this work

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    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.

    Original languageEnglish
    Pages (from-to)1311-1326
    Number of pages16
    JournalNeurological Sciences
    Issue number2
    Early online date7 Jun 2021
    Publication statusPublished - Feb 2022

    Bibliographical note

    Copyright the Author(s) 2021. Version archived for private and non-commercial use with the permission of the author/s and according to publisher conditions. For further rights please contact the publisher.


    • Chiari 1 malformation
    • Children
    • Consensus
    • Craniovertebral decompression
    • Management
    • Syringomyelia


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