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

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A Correction to this article was published on 17 November 2021

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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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Acknowledgements

The authors would like also to thank the following Patients’ Associations for their participation and support (in alphabetical order): AICRA for Craniosynostosis (Italy), AISMAC (Italy), APAISER (France), ASAP (USA), Bobby Jones Chiari & Syringomyelia Foundation’s (USA), Deutsche Syringomyelie und Chiari Malformation (Germany), FEMACPA (Spain), National Syringomyelia Association (Bulgaria), SACA (Ireland).

International Experts Jury of the Chiari & Syringomyelia Consensus Conference: list of affiliations

  • Andrea Barbanera, Department of Neurosurgery, “SS Antonio e Biagio e Cesare Arrigo” Hospital, Alessandria, Italy

  • Alessandro Bertuccio, Department of Neurosurgery, “SS Antonio e Biagio e Cesare Arrigo” Hospital, Alessandria, Italy

  • Paolo Bolognese, Chiari Neuosurgical Center, Mount Sinai, South Nassau, Oceanside (NY), US

  • Andrew Brodbelt, Consultant Neurosurgeon, The Walton Centre NHS Foundation Trust, Liverpool, UK

  • Carlo Celada, AISMAC, Italy

  • Luisa Chiapparini, Service of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

  • Palma Ciaramitaro, CRESSC, Department of Neuroscience, AOU Citta’ della Salute e della Scienza di Torino, Torino, Italy

  • Dario Cocito, Istituti Clinici Scientifici Maugeri, Torino, Italy

  • Marcella Curone, Casa di Cura del Policlinico, Igea Headache Center, Milan, Italy

  • Grazia Devigili, Department of Clinical Neuroscience, Fondazione IRCCS Istituto Neurologico Carlo Besta. Milan, Italy

  • Alessandra Erbetta, Service of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

  • Marilena Ferraris, Service of Neuroradiology, Diagnostic Imaging Department, AOU Citta’ della Salute e della Scienza di Torino, Torino, Italy

  • Marika Furlanetto, Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

  • Diego Garbossa, Department of Neurosurgery, University of Torino, Torino, Italy

  • Mado Gilanton, APAISER, France

  • George Jallo, Johns Hopkins University Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, US

  • Marieta Karadjova, Neurology Department, University of Sofia, Sofia, Bulgaria

  • Jörg Klekamp, Christliches Krankenhaus Quakenbrück, Department of Neurosurgery, Quakenbrück, Germany

  • Fulvio Massaro, Department of Neurosurgery, University of Torino, Torino, Italy

  • Luca Massimi, Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy

  • Sylvia Morar, Neurosurgery Department, Reference Center Rares Diseases C-MAVEM, CHU Bicetre APHP, Paris, France

  • Fabrice Parker, Neurosurgery Department, Reference Center Rares Diseases C-MAVEM, CHU Bicetre APHP, Paris, France

  • Paola Peretta, Pediatric Neurosurgery, Ospedale Infantile Regina Margherita, AOU Citta’ della Salute e della Scienza di Torino, Torino, Italy

  • Paolo Perrini, Department of Translational Research and of New Surgical and Medical Technologies, University of Pisa, Pisa, Italy

  • Maria Antonia Poca, Neurosurgery and Pediatric Neurosurgery, Vall d’Hebron Hospital Universitari, Neurotrauma and Neurosurgery Research Unit, and Universitat Autònoma de Barcelona, Barcelona, Spain.

  • Juan Sahuquillo, Neurosurgery and Pediatric Neurosurgery, Vall d’Hebron Hospital Universitari, Neurotrauma and Neurosurgery Research Unit, and Universitat Autònoma de Barcelona, Barcelona, Spain.

  • Marcus Stoodley, The Australian School of Advanced Medicine, Macquarie University, NSW 2109, Australia.

  • Giuseppe Talamonti, Department of Neurosurgery, ASST Niguarda, Milan, Italy

  • Fabio Triulzi, Neuroradiology Unit, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy

  • Maria Consuelo Valentini, Service of Neuroradiology, Diagnostic Imaging Department, AOU Citta’ della Salute e della Scienza di Torino, Torino, Italy

  • Massimiliano Visocchi, Department of Neurosurgery, Catholic University School of Medicine, Rome, Italy

  • Laura Valentini, Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

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Authors and Affiliations

  1. CRESSC, SSD Coordinamento Neurofisiologia Clinica and Neurosurgery Unit, Department of Neuroscience, University of Torino, Torino, Italy

    Palma Ciaramitaro & Diego Garbossa

  2. Neuroscience Dpt, Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino, Presidio CTO, via Zuretti, 29, Torino, Italy

    Palma Ciaramitaro

  3. Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy

    Luca Massimi

  4. Department of Neurosurgery, “SS Antonio e Biagio e Cesare Arrigo” Hospital, Alessandria, Italy

    Alessandro Bertuccio & Andrea Barbanera

  5. Neuroepidemiology Unit – Scientific Directorate, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Alessandra Solari & Mariangela Farinotti

  6. Pediatric Neurosurgery, Ospedale Infantile ReginaMargherita, AOU Citta’ della Salute e della Scienza di Torino, Torino, Italy

    Paola Peretta

  7. Developmental Neurology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Veronica Saletti

  8. Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Luisa Chiapparini

  9. Chiari Neuosurgical Center, Mount Sinai, South Nassau, Oceanside, NY, USA

    Paolo Bolognese

  10. Consultant Neurosurgeon, The Walton Centre NHS Foundation Trust, Liverpool, UK

    Andrew Brodbelt

  11. AISMAC, Torino, Italy

    Carlo Celada

  12. Istituti Clinici Scientifici Maugeri, Torino, Italy

    Dario Cocito

  13. Casa di Cura del Policlinico, Igea Headache Center, Milan, Italy

    Marcella Curone

  14. Department of Clinical Neuroscience, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Grazia Devigili

  15. Service of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Alessandra Erbetta

  16. Service of Neuroradiology, Diagnostic Imaging Department, AOU Citta’ della Salute e della Scienza di Torino, Torino, Italy

    Marilena Ferraris & Maria Consuelo Valentini

  17. Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Marika Furlanetto & Laura Valentini

  18. APAISER, Treillières, France

    Mado Gilanton

  19. Johns Hopkins University Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA

    George Jallo

  20. Neurology Department, University of Sofia, Sofia, Bulgaria

    Marieta Karadjova

  21. Christliches Krankenhaus Quakenbrück, Department of Neurosurgery, Quakenbrück, Germany

    Jorg Klekamp

  22. Department of Neurosurgery, University of Torino, Torino, Italy

    Fulvio Massaro

  23. Neurosurgery Department, Reference Center Rares Diseases C-MAVEM, CHU Bicetre APHP, Paris, France

    Sylvia Morar & Fabrice Parker

  24. Department of Translational Research and of New Surgical and Medical Technologies, University of Pisa, Pisa, Italy

    Paolo Perrini

  25. Neurosurgery and Pediatric Neurosurgery, Vall d’Hebron Hospital Universitari, Neurotrauma and Neurosurgery Research Unit, and Universitat Autònoma de Barcelona, Barcelona, Spain

    Maria Antonia Poca & Juan Sahuquillo

  26. The Australian School of Advanced Medicine, Macquarie University, Sydney, NSW, 2109, Australia

    Marcus Stoodley

  27. Department of Neurosurgery, ASST Niguarda, Milan, Italy

    Giuseppe Talamonti

  28. Neuroradiology Unit, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy

    Fabio Triulzi

  29. Department of Neurosurgery, Catholic University School of Medicine, Rome, Italy

    Massimiliano Visocchi

Authors
  1. Palma Ciaramitaro
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  2. Luca Massimi
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  3. Alessandro Bertuccio
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  4. Alessandra Solari
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  5. Mariangela Farinotti
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  6. Paola Peretta
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  7. Veronica Saletti
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  8. Luisa Chiapparini
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  9. Andrea Barbanera
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  10. Diego Garbossa
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  11. Paolo Bolognese
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  13. Carlo Celada
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  14. Dario Cocito
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  15. Marcella Curone
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  18. Marilena Ferraris
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  19. Marika Furlanetto
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  20. Mado Gilanton
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  21. George Jallo
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  22. Marieta Karadjova
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  23. Jorg Klekamp
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  24. Fulvio Massaro
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  25. Sylvia Morar
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  26. Fabrice Parker
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  27. Paolo Perrini
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  28. Maria Antonia Poca
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  29. Juan Sahuquillo
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  30. Marcus Stoodley
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  31. Giuseppe Talamonti
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  32. Fabio Triulzi
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  33. Maria Consuelo Valentini
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  34. Massimiliano Visocchi
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  35. Laura Valentini
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Consortia

On behalf of the International Experts Jury of the Chiari Syringomyelia Consensus Conference, Milan, November 11-13, 2019

Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by Palma Ciaramitaro, Luca Massimi, Alessandro Bertuccio, Alessandra Solari, Mariangela Farinotti, Paola Peretta, Veronica Saletti, Andrea Barbanera, Diego Garbossa, and Laura Valentini. The first draft of the manuscript was written by Palma Ciaramitaro and Laura Valentini, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Palma Ciaramitaro.

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Ciaramitaro, P., Massimi, L., Bertuccio, A. et al. Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document. Neurol Sci 43, 1327–1342 (2022). https://doi.org/10.1007/s10072-021-05347-3

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