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. 2022 Feb;43(2):1311-1326.
doi: 10.1007/s10072-021-05317-9. Epub 2021 Jun 7.

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

Luca Massimi  1 , Paola Peretta  2 , Alessandra Erbetta  3 , Alessandra Solari  4 , Mariangela Farinotti  4 , Palma Ciaramitaro  5 , Veronica Saletti  6 , Massimo Caldarelli  7 , Alexandre Casagrande Canheu  8 , Carlo Celada  9 , Luisa Chiapparini  3 , Daniela Chieffo  10 , Giuseppe Cinalli  11 , Federico Di Rocco  12 , Marika Furlanetto  13 , Flavio Giordano  14 , George Jallo  15 , Syril James  16 , Paola Lanteri  17 , Christian Lemarchand  18 , Martina Messing-Jünger  19 , Cecilia Parazzini  20 , Giovanna Paternoster  16 , Gianluca Piatelli  21 , Maria A Poca  22 , Prab Prabahkar  23 , Federica Ricci  24 , Andrea Righini  20 , Francesco Sala  25 , Juan Sahuquillo  22 , Marcus Stoodley  26 , Giuseppe Talamonti  27 , Dominic Thompson  28 , Fabio Triulzi  29 , Mino Zucchelli  30 , Laura Valentini  13 ; International Experts - Jury of the Chiari & Syringomyelia Consensus Conference, “Milan, November 11–13, 2019”
Affiliations

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

Luca Massimi et al. Neurol Sci. 2022 Feb.

Abstract

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.

Keywords: Chiari 1 malformation; Children; Consensus; Craniovertebral decompression; Management; Syringomyelia.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Flowchart of the Delphi study
Fig. 2
Fig. 2
Agreement (%) on each statement of the Children Consensus Document in the three Delphi rounds (35 experts)
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