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

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 GilantonGeorge 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

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

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.
Original languageEnglish
Pages (from-to)1327-1342
Number of pages16
JournalNeurological Sciences
Volume43
Issue number2
Early online date15 Jun 2021
DOIs
Publication statusPublished - Feb 2022

Bibliographical note

A correction exists for this article, and has been included in the final published version.

Keywords

  • Chiari malformation
  • Syringomyelia
  • Adults
  • Consensus
  • Classification
  • Surgery

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