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Acta Neurochirurgica
Published in: Acta Neurochirurgica 7/2020

01-07-2020 | Syringomyelia | Review Article - Neurosurgery general

Chiari type 1—a malformation or a syndrome? A critical review

Authors: Radek Frič, Per Kristian Eide

Published in: Acta Neurochirurgica | Issue 7/2020

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Abstract

Objective

As the understanding of pathophysiology behind Chiari malformation still is limited, the treatment of Chiari malformation type 1 remains rather empirical. This may result in suboptimal treatment strategy and outcome in many cases. In this review, we critically address whether the condition known today as Chiari malformation type I should rather be denoted Chiari syndrome.

Methods

The current knowledge of Chiari malformation type 1 is summarized from the historical, etymological, genetic, clinical, and in particular pathophysiological perspectives.

Results

There are several lines of evidence that Chiari malformation type 1 represents a condition significantly different from types 2 to 4. Unlike the other types, the type 1 should rather be considered a syndrome, thus supporting the reasons to reappraise the traditional classification of Chiari malformations.

Conclusion

We propose that Chiari malformation type 1 should rather be denoted Chiari syndrome, while the notation malformation is maintained for types 2–4.
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Metadata
Title
Chiari type 1—a malformation or a syndrome? A critical review
Authors
Radek Frič
Per Kristian Eide
Publication date
01-07-2020
Publisher
Springer Vienna
Published in
Acta Neurochirurgica / Issue 7/2020
Print ISSN: 0001-6268
Electronic ISSN: 0942-0940
DOI
https://doi.org/10.1007/s00701-019-04100-2

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