Department of Neurosurgery, Oslo University Hospital - Rikshospitalet, Oslo, Norway.
Faculty of Medicine, University of Oslo, Oslo, Norway.
Acta Neurochir (Wien). 2020 Jul;162(7):1513-1525. doi: 10.1007/s00701-019-04100-2. Epub 2019 Oct 28.
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.
The current knowledge of Chiari malformation type 1 is summarized from the historical, etymological, genetic, clinical, and in particular pathophysiological perspectives.
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.
We propose that Chiari malformation type 1 should rather be denoted Chiari syndrome, while the notation malformation is maintained for types 2-4.
由于对 Chiari 畸形背后的病理生理学的理解仍然有限，Chiari 畸形 1 型的治疗仍然相当经验性。在许多情况下，这可能导致治疗策略和结果不理想。在这篇综述中，我们从历史、词源学、遗传学、临床特别是病理生理学的角度批判性地探讨了当今被称为 Chiari 畸形 1 型的疾病是否更应被称为 Chiari 综合征。
从历史、词源学、遗传学、临床特别是病理生理学的角度总结了 Chiari 畸形 1 型的现有知识。
有几条证据表明，Chiari 畸形 1 型与 2 型至 4 型明显不同。与其他类型不同，1 型更应被视为一种综合征，从而支持重新评估 Chiari 畸形传统分类的理由。
我们建议 Chiari 畸形 1 型应更名为 Chiari 综合征，而 2 型至 4 型则保留畸形的命名。
