Erschienen in:
28.10.2019 | Review Article - Neurosurgery general
Chiari type 1—a malformation or a syndrome? A critical review
verfasst von:
Radek Frič, Per Kristian Eide
Erschienen in:
Acta Neurochirurgica
|
Ausgabe 7/2020
Einloggen, um Zugang zu erhalten
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.