A Critical Update of the Classification of Chiari and Chiari-like Malformations
Sahuquillo Barris, Juan (Universitat Autònoma de Barcelona. Departament de Cirurgia)
Moncho, Dulce (Vall d'Hebron Institut de Recerca (VHIR))
Ferré Masó, Alex (Vall d'Hebron Institut de Recerca (VHIR))
López-Bermeo, Diego (Vall d'Hebron Institut de Recerca (VHIR))
Sahuquillo-Muxi, Aasma (Vall d'Hebron Institut de Recerca (VHIR))
Poca Pastor, María Antonia (Universitat Autònoma de Barcelona. Departament de Cirurgia)

Date:	2023
Abstract:	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.
Grants:	Instituto de Salud Carlos III PI22/01082 Agència de Gestió d'Ajuts Universitaris i de Recerca 2021/SGR-00810
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Subject:	Arnold-Chiari Malformation ; Rare diseases ; Syringomyelia ; Chiari malformation ; Classification
Published in:	Journal of clinical medicine, Vol. 12 Num. 14 (July 2023) , ISSN 2077-0383

DOI: 10.3390/jcm12144626
PMID: 37510741

31 p, 4.6 MB

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