Palma Ciaramitaro1,2, Luca Massimi3, Alessandro Bertuccio4, Alessandra Solari5, Mariangela Farinotti5, Paola Peretta6, Veronica Saletti7, Luisa Chiapparini8, Andrea Barbanera4, Diego Garbossa9, Paolo Bolognese10, Andrew Brodbelt11, Carlo Celada12, Dario Cocito13, Marcella Curone14, Grazia Devigili15, Alessandra Erbetta16, Marilena Ferraris17, Marika Furlanetto18, Mado Gilanton19, George Jallo20, Marieta Karadjova21, Jorg Klekamp22, Fulvio Massaro23, Sylvia Morar24, Fabrice Parker24, Paolo Perrini25, Maria Antonia Poca26, Juan Sahuquillo26, Marcus Stoodley27, Giuseppe Talamonti28, Fabio Triulzi29, Maria Consuelo Valentini17, Massimiliano Visocchi30, Laura Valentini18. 1. CRESSC, SSD Coordinamento Neurofisiologia Clinica and Neurosurgery Unit, Department of Neuroscience, University of Torino, Torino, Italy. palma.ciaramitaro@gmail.com. 2. Neuroscience Dpt, Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino, Presidio CTO, via Zuretti, 29, Torino, Italy. palma.ciaramitaro@gmail.com. 3. Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. 4. Department of Neurosurgery, "SS Antonio e Biagio e Cesare Arrigo" Hospital, Alessandria, Italy. 5. Neuroepidemiology Unit - Scientific Directorate, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. 6. Pediatric Neurosurgery, Ospedale Infantile ReginaMargherita, AOU Citta' della Salute e della Scienza di Torino, Torino, Italy. 7. Developmental Neurology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. 8. Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. 9. CRESSC, SSD Coordinamento Neurofisiologia Clinica and Neurosurgery Unit, Department of Neuroscience, University of Torino, Torino, Italy. 10. Chiari Neuosurgical Center, Mount Sinai, South Nassau, Oceanside, NY, USA. 11. Consultant Neurosurgeon, The Walton Centre NHS Foundation Trust, Liverpool, UK. 12. AISMAC, Torino, Italy. 13. Istituti Clinici Scientifici Maugeri, Torino, Italy. 14. Casa di Cura del Policlinico, Igea Headache Center, Milan, Italy. 15. Department of Clinical Neuroscience, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. 16. Service of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. 17. Service of Neuroradiology, Diagnostic Imaging Department, AOU Citta' della Salute e della Scienza di Torino, Torino, Italy. 18. Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. 19. APAISER, Treillières, France. 20. Johns Hopkins University Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA. 21. Neurology Department, University of Sofia, Sofia, Bulgaria. 22. Christliches Krankenhaus Quakenbrück, Department of Neurosurgery, Quakenbrück, Germany. 23. Department of Neurosurgery, University of Torino, Torino, Italy. 24. Neurosurgery Department, Reference Center Rares Diseases C-MAVEM, CHU Bicetre APHP, Paris, France. 25. Department of Translational Research and of New Surgical and Medical Technologies, University of Pisa, Pisa, Italy. 26. Neurosurgery and Pediatric Neurosurgery, Vall d'Hebron Hospital Universitari, Neurotrauma and Neurosurgery Research Unit, and Universitat Autònoma de Barcelona, Barcelona, Spain. 27. The Australian School of Advanced Medicine, Macquarie University, Sydney, NSW, 2109, Australia. 28. Department of Neurosurgery, ASST Niguarda, Milan, Italy. 29. Neuroradiology Unit, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy. 30. Department of Neurosurgery, Catholic University School of Medicine, Rome, Italy.
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.
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.
Authors: K L Brickell; N E Anderson; A J Charleston; J K A Hope; A P L Bok; P A Barber Journal: J Neurol Neurosurg Psychiatry Date: 2006-03-20 Impact factor: 10.154