Embryology and pathophysiology of the Chiari I and II malformations: A comprehensive review

Clin Anat . 2018 Mar;31(2):202-215. doi: 10.1002/ca.22939. Epub 2017 Dec 4.

Mohammadali M Shoja, Jaspreet Johal, W Jerry Oakes,  R Shane Tubbs

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Abstract

Although the Chiari malformations are well-studied and described developmental anomalies, there remains some incongruity in regards to their underlying etiologies. A number of theories have been proposed with the purpose of accounting for the embryology and pathogenesis of the Chiari I and II malformations and their associated complications and clinical syndromes. The present review aims to review the pertinent literature for all of the main theories that have been proposed, and outline their validity and relevance to our contemporary understanding of these anomalies. Clin. Anat. 31:202-215, 2018. © 2017 Wiley Periodicals, Inc.

Keywords: Arnold-Chiari malformation; cerebrospinal fluid pressure; etiology; hydrocephalus; mechanism; syringomyelia.