The Chiari-I Malformation Associated Syringomyelia

Background: Syringomyelia secondary to the Chiari-I malformation (CMI) is often referred for surgical treatment, with a form of posterior fossa decompression (PFD) as the most recommended surgical approach. Many characteristics regarding the formation, signs, and symptoms, and natural history of the disease remain contentious.

Objective: The purpose of this study is to evaluate the composition of the literature for CMI associated-syringomyelia and to identify topics requiring additional research.

Methods: A comprehensive search of the PubMed, Web of Science, Scopus, Embase, and Cochrane databases was independently performed through March 2020. This study adhered to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. Studies were reviewed for topics including natural history, treatment, theories of development, and syrinx characteristics.

Results: A total of 106 studies published between 2000 and 2020 were included. Studies were reviewed for topic inclusion based on the categories of natural history, treatment, theories of development, fluid dynamics, syrinx characteristics, pediatric, and non-CMI origins. Studies were not excluded from multi categorization. The most discussed category was treatment, with 52 studies. Theories of development and syrinx characteristics were also well represented, with 25 and 30 studies, respectively. The least mentioned topic was natural history, with only five studies mentioning the natural course of syringomyelia.

Conclusion: While treatment for CMI-associated syringomyelia has been well explored by the literature, little is known about the natural history of the disease. Smaller syrinxes that present without significant signs and symptoms may not be urgent candidates for surgical correction. More studies should be conducted to determine the progression of the disease without treatment.

Publikationsverlauf

Artikel online veröffentlicht:
12. Februar 2021