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Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document
Luca Massimi1, Paola Peretta2, Alessandra Erbetta3
1Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli, 8, 00168, Rome, Italy. lucamax30@hotmail.com.
Insights
This study achieved expert consensus on diagnosing and treating Chiari malformation type 1 (CM1) in children. The resulting 58 statements provide guidance for clinicians and researchers managing this rare condition.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Medical Consensus Building
Background:
- Chiari malformation type 1 (CM1) lacks standardized classification and treatment protocols.
- This rarity necessitates a consensus-driven approach for optimal patient care.
Purpose of the Study:
- To establish international consensus on the diagnosis and treatment of Chiari malformation type 1 (CM1) in pediatric patients.
- To develop evidence-based guidelines for managing CM1.
Main Methods:
- A multidisciplinary panel developed 57 statements based on literature review.
- International experts (34) participated in a three-round Delphi study, rating statements on a 4-point Likert scale.
- Consensus required endorsement by at least 75% of raters.
Main Results:
- Consensus was reached on 58 out of 59 statements (98.3%) after three Delphi rounds.
- Agreement covered key areas: Definition and Classification, Planning, Surgery, and Isolated Syringomyelia.
- Only the definition of 'radiological failure 24 months post-surgery' remained unresolved.
Conclusions:
- A comprehensive consensus document with 58 statements (24 diagnosis, 34 treatment) was finalized.
- This consensus serves as a vital resource for clinicians and researchers caring for children with CM1.
- An international network, registry, and collaborative studies are recommended to enhance the evidence base and long-term care for CM1 patients.
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:
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:
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:
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.
