Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document.

Chiari 1 malformation Children Consensus Craniovertebral decompression Management Syringomyelia

Journal

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
ISSN: 1590-3478
Titre abrégé: Neurol Sci
Pays: Italy
ID NLM: 100959175

Informations de publication

Date de publication:
Feb 2022
Historique:
received: 16 01 2021
accepted: 08 05 2021
pubmed: 8 6 2021
medline: 28 1 2022
entrez: 7 6 2021
Statut: ppublish

Résumé

Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children. A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three). Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery." The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.

Sections du résumé

BACKGROUND BACKGROUND
Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children.
METHODS METHODS
A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three).
RESULTS RESULTS
Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery."
CONCLUSIONS CONCLUSIONS
The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.

Identifiants

pubmed: 34097175
doi: 10.1007/s10072-021-05317-9
pii: 10.1007/s10072-021-05317-9
pmc: PMC8789635
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1311-1326

Informations de copyright

© 2021. The Author(s).

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Auteurs

Luca Massimi (L)

Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli, 8, 00168, Rome, Italy. lucamax30@hotmail.com.

Paola Peretta (P)

Pediatric Neurosurgery, AOU Citta' della Salute e della Scienza di Torino, Torino, Italy.

Alessandra Erbetta (A)

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

Alessandra Solari (A)

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

Mariangela Farinotti (M)

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

Palma Ciaramitaro (P)

Department of Neuroscience, AOU Citta' della Salute e della Scienza di Torino, Torino, Italy.

Veronica Saletti (V)

Department of Pediatric Neurology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Massimo Caldarelli (M)

Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli, 8, 00168, Rome, Italy.

Alexandre Casagrande Canheu (AC)

Pediatric Neurosurgery Division, Universidade Estadual de Londrina, Londrina, Brazil.

Carlo Celada (C)

"Associazione Italiana Siringomielia e Arnold Chiari", Garino, Italy.

Luisa Chiapparini (L)

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

Daniela Chieffo (D)

Clinical Psychology Unit, Fondazione Policlinico Universitario A Gemelli IRCCS and UCSC, Rome, Italy.

Giuseppe Cinalli (G)

Pediatric Neurosurgery, Santobono-Pausilipon Children's Hospital, Naples, Italy.

Federico Di Rocco (F)

Pediatric Neurosurgery Department, Université de Lyon, INSERM U1033, Hopital Femme Mère Enfant, Lyon, France.

Marika Furlanetto (M)

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

Flavio Giordano (F)

Department of Neurosurgery, Meyer Pediatric Hospital, Florence, Italy.

George Jallo (G)

Institute for Brain Protection Sciences, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.

Syril James (S)

Department of Pediatric Neurosurgery, Necker Enfants Malades Hospital, Paris, France.

Paola Lanteri (P)

Department of Diagnostic and Technology, Neurophysiopathology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Christian Lemarchand (C)

Conseil Scientifique APAISER S&C, Treillières, France.

Martina Messing-Jünger (M)

Pädiatrische Neurochirurgie, Asklepios Kinderklinik, Sankt Augustin, Germany.

Cecilia Parazzini (C)

Department of Pediatric Radiology and Neuroradiology, Children Hospital V. Buzzi, Milan, Italy.

Giovanna Paternoster (G)

Department of Pediatric Neurosurgery, Necker Enfants Malades Hospital, Paris, France.

Gianluca Piatelli (G)

Department of Neurosurgery, Gaslini Children's Hospital, Genoa, Italy.

Maria A Poca (MA)

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

Prab Prabahkar (P)

Department of Neurology, Great Ormond Street Hospital for Children, London, UK.

Federica Ricci (F)

Pediatric Neuropsychiatric Unit, AOU Citta' della Salute e della Scienza di Torino, Torino, Italy.

Andrea Righini (A)

Department of Pediatric Radiology and Neuroradiology, Children Hospital V. Buzzi, Milan, Italy.

Francesco Sala (F)

Section of Neurosurgery, Department of Neurosciences, Biomedicine and Movement Sciences, University Hospital, Verona, Italy.

Juan Sahuquillo (J)

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

Marcus Stoodley (M)

Department of Clinical Medicine, Macquarie University Clinical Associates, Sidney, Australia.

Giuseppe Talamonti (G)

Department of Neurosurgery, ASST Niguarda, Milan, Italy.

Dominic Thompson (D)

Department of Neurosurgery, Great Ormond Street Hospital for Children, London, UK.

Fabio Triulzi (F)

Department of Pathophysiology and Transplantation, Neuroradiology Unit, University of Milan, Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Mino Zucchelli (M)

Neurochirurgia Pediatrica, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.

Laura Valentini (L)

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

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