Journal Article

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

Mado, Gilanton, Celada Carlo, Farinotti Mariangela, Massaro Fulvio, Valentini, Maria Consuelo, Triulzi Fabio, Chiapparini Luisa, Curone Marcella, Sahuquillo Juan, Garbossa Diego, Jallo, George, Perrini Paolo, Poca Maria Antonia, Talamonti Giuseppe, Brodbelt, Andrew, Solari, Alessandra, Stoodley, Marcus, Furlanetto Marika, Massimi Luca, Barbanera, Andrea, Klekamp Jorg, Devigili Grazia, Valentini, Laura, Bolognese Paolo, Erbetta Alessandra, Parker, Fabrice, Cocito Dario, Visocchi Massimiliano, Karadjova Marieta, Bertuccio Alessandro, Morar, Sylvia, Ferraris Marilena, Saletti Veronica, Palma, Ciaramitaro, Peretta Paola

2022

Overview

BackgroundSyringomyelia 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.MethodsA 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).ResultsThirty-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.ConclusionsThe consensus document consists of 63 statements which benefited from expert discussion and fine-tuning, serving clinicians and researchers following adults with Chiari and syringomyelia.

• Publisher: Springer Nature B.V
• Subject: Brain / Congenital defects / Diagnosis / Medical diagnosis / Neural tube defects / Neurosciences / Patients / Pediatrics / Rare diseases / Spinal cord