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Published in: Neurological Sciences 2/2022

01-02-2022 | Magnetic Resonance Imaging | Original Article

Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document

Authors: Palma Ciaramitaro, Luca Massimi, Alessandro Bertuccio, Alessandra Solari, Mariangela Farinotti, Paola Peretta, Veronica Saletti, Luisa Chiapparini, Andrea Barbanera, Diego Garbossa, Paolo Bolognese, Andrew Brodbelt, Carlo Celada, Dario Cocito, Marcella Curone, Grazia Devigili, Alessandra Erbetta, Marilena Ferraris, Marika Furlanetto, Mado Gilanton, George Jallo, Marieta Karadjova, Jorg Klekamp, Fulvio Massaro, Sylvia Morar, Fabrice Parker, Paolo Perrini, Maria Antonia Poca, Juan Sahuquillo, Marcus Stoodley, Giuseppe Talamonti, Fabio Triulzi, Maria Consuelo Valentini, Massimiliano Visocchi, Laura Valentini, On behalf of the International Experts Jury of the Chiari Syringomyelia Consensus Conference, Milan, November 11-13, 2019

Published in: Neurological Sciences | Issue 2/2022

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Abstract

Background

Syringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing. Aim of the study: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults.

Methods

A multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document. After an evidence-based review and further discussions, 63 draft statements grouped in 4 domains (definition and classification/planning/surgery/isolated syringomyelia) were formulated. A Jury of 32 experts in the field of diagnosis and treatment of Chiari and syringomyelia and patient representatives were invited to take part in a three-round Delphi process. The Jury received a structured questionnaire containing the 63 statements, each to be voted on a 4-point Likert-type scale and commented. Statements with agreement <75% were revised and entered round 2. Round 3 was face-to-face, during the Chiari Consensus Conference (Milan, November 2019).

Results

Thirty-one out of 32 Jury members (6 neurologists, 4 neuroradiologists, 19 neurosurgeons, and 2 patient association representatives) participated in the consensus. After round 2, a consensus was reached on 57/63 statements (90.5%). The six difficult statements were revised and voted in round 3, and the whole set of statements was further discussed and approved.

Conclusions

The consensus document consists of 63 statements which benefited from expert discussion and fine-tuning, serving clinicians and researchers following adults with Chiari and syringomyelia.
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Metadata
Title
Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document
Authors
Palma Ciaramitaro
Luca Massimi
Alessandro Bertuccio
Alessandra Solari
Mariangela Farinotti
Paola Peretta
Veronica Saletti
Luisa Chiapparini
Andrea Barbanera
Diego Garbossa
Paolo Bolognese
Andrew Brodbelt
Carlo Celada
Dario Cocito
Marcella Curone
Grazia Devigili
Alessandra Erbetta
Marilena Ferraris
Marika Furlanetto
Mado Gilanton
George Jallo
Marieta Karadjova
Jorg Klekamp
Fulvio Massaro
Sylvia Morar
Fabrice Parker
Paolo Perrini
Maria Antonia Poca
Juan Sahuquillo
Marcus Stoodley
Giuseppe Talamonti
Fabio Triulzi
Maria Consuelo Valentini
Massimiliano Visocchi
Laura Valentini
On behalf of the International Experts Jury of the Chiari Syringomyelia Consensus Conference, Milan, November 11-13, 2019
Publication date
01-02-2022
Publisher
Springer International Publishing
Published in
Neurological Sciences / Issue 2/2022
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI
https://doi.org/10.1007/s10072-021-05347-3

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