Sahuquillo Juan, Moncho Dulce, Ferré Alex, López-Bermeo Diego, Sahuquillo-Muxi Aasma, Poca Maria A
Department of Neurosurgery, Vall d'Hebron University Hospital, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
Neurotraumatology and Neurosurgery Research Unit, Vall d'Hebron Institut de Recerca (VHIR), Vall d'Hebron Barcelona Hospital Campus, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
J Clin Med. 2023 Jul 11;12(14):4626. doi: 10.3390/jcm12144626.
Chiari malformations are a group of craniovertebral junction anomalies characterized by the herniation of cerebellar tonsils below the foramen magnum, often accompanied by brainstem descent. The existing classification systems for Chiari malformations have expanded from the original four categories to nine, leading to debates about the need for a more descriptive and etiopathogenic terminology. This review aims to examine the various classification approaches employed and proposes a simplified scheme to differentiate between different types of tonsillar herniations. Furthermore, it explores the most appropriate terminology for acquired herniation of cerebellar tonsils and other secondary Chiari-like malformations. Recent advances in magnetic resonance imaging (MRI) have revealed a higher prevalence and incidence of Chiari malformation Type 1 (CM1) and identified similar cerebellar herniations in individuals unrelated to the classic phenotypes described by Chiari. As we reassess the existing classifications, it becomes crucial to establish a terminology that accurately reflects the diverse presentations and underlying causes of these conditions. This paper contributes to the ongoing discussion by offering insights into the evolving understanding of Chiari malformations and proposing a simplified classification and terminology system to enhance diagnosis and management.
Chiari畸形是一组颅颈交界区异常，其特征为小脑扁桃体疝入枕骨大孔以下，常伴有脑干下移。现有的Chiari畸形分类系统已从最初的四类扩展到九类，引发了关于是否需要更具描述性和病因学的术语的争论。本综述旨在探讨所采用的各种分类方法，并提出一种简化方案以区分不同类型的扁桃体疝。此外，还探讨了小脑扁桃体后天性疝及其他继发性Chiari样畸形的最合适术语。磁共振成像（MRI）的最新进展揭示了1型Chiari畸形（CM1）的更高患病率和发病率，并在与Chiari所描述的经典表型无关的个体中发现了类似的小脑疝。当我们重新评估现有分类时，建立一个能准确反映这些病症的多样表现和潜在病因的术语变得至关重要。本文通过深入了解对Chiari畸形的不断演变的认识，并提出一个简化的分类和术语系统以加强诊断和管理，为正在进行的讨论做出了贡献。
