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

Neurol Sci. 2022 Feb;43(2):1327-1342. doi: 10.1007/s10072-021-05347-3. Epub 2021 Jun 15.


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.

Keywords: Adults; Chiari malformation; Classification; Consensus; Surgery; Syringomyelia.

MeSH terms

  • Adult
  • Arnold-Chiari Malformation* / diagnosis
  • Arnold-Chiari Malformation* / diagnostic imaging
  • Child
  • Humans
  • Rare Diseases
  • Surveys and Questionnaires
  • Syringomyelia* / diagnosis
  • Syringomyelia* / diagnostic imaging