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01-12-2021 | Magnetic Resonance Imaging | Review Article

The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature

Authors: Veronica Saletti, Mariangela Farinotti, Paola Peretta, Luca Massimi, Palma Ciaramitaro, Saba Motta, Alessandra Solari, Laura Grazia Valentini

Published in: Neurological Sciences | Issue 12/2021

Abstract

In anticipation of the “Chiari and Syringomyelia Consensus Conference” held in Milan in 2019, we performed a systematic literature review on the management of Chiari malformation type 1 (CM1) and syringomyelia (Syr) in children.

We aimed to summarize the available evidence and identify areas where consensus has not been reached and further research is needed.

In accordance with PRISMA guidelines, we formulated seven questions in Patients-Interventions-Comparators-Outcomes (PICO) format. Six PICOs concerned CM1 children with/without additional structural anomalies (Syr, craniosynostosis, hydrocephalus, tethered cord, and cranio-vertebral junction anomalies), and one PICO Syr without CM1. We searched Medline, Embase, Cochrane, and NICE databases from January 1, 1999, to May 29, 2019. Cohort studies, controlled and randomized clinical trials (CCTs, RCTs), and systematic reviews were included, all pertinent only to patients ≤ 18 years of age.

For CM1, 3787 records were found, 460 full texts were assessed and 49 studies (46 cohort studies, one RCT, and two systematic reviews) were finally included. For Syr, 376 records were found, 59 full texts were assessed, and five studies (one RCT and four cohort studies) were included. Data on each PICO were synthetized narratively due to heterogeneity in the inclusion criteria, outcome measures, and length of follow-up of the included studies.

Despite decades of experience on CM1 and Syr management in children, the available evidence remains limited. Specifically, there is an urgent need for collaborative initiatives focusing on the adoption of shared inclusion criteria and outcome measures, as well as rigorous prospective designs, particularly RCTs.

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Title: The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature
Authors: Veronica Saletti
Mariangela Farinotti
Paola Peretta
Luca Massimi
Palma Ciaramitaro
Saba Motta
Alessandra Solari
Laura Grazia Valentini
Publication date: 01-12-2021
Publisher: Springer International Publishing
Keywords: Magnetic Resonance Imaging
Magnetic Resonance Imaging
Hydrocephalus
Hydrocephalus
Syringomyelia
Hydrocephalus
Chiari Malformation
Craniosynostosis
Chiari Malformation
Published in: Neurological Sciences / Issue 12/2021
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI: https://doi.org/10.1007/s10072-021-05565-9

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The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature

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