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

Neurol Sci. 2021 Dec;42(12):4965-4995. doi: 10.1007/s10072-021-05565-9. Epub 2021 Sep 30.


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.

Keywords: Chiari malformation type 1; Children; Literature review; Management; Outcome; Syringomyelia.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Arnold-Chiari Malformation* / complications
  • Arnold-Chiari Malformation* / epidemiology
  • Arnold-Chiari Malformation* / therapy
  • Child
  • Humans
  • Neural Tube Defects*
  • Prospective Studies
  • Syringomyelia* / complications
  • Syringomyelia* / therapy