Chiari I Deformity: Beyond 5 mm below the Foramen Magnum

医学 大孔 畸形 解剖 放射科
作者
James Ryan Loftus,Catherine Wassef,Shehanaz Ellika
出处
期刊:Radiographics [Radiological Society of North America]
卷期号:44 (9) 被引量:1
标识
DOI:10.1148/rg.230227
摘要

Although originally described as a consecutive series of pathologic changes, Chiari syndrome represents a spectrum of disease divided into two subsets: development deformities of the paraxial mesoderm manifesting after birth (types 0-1.5) and true congenital malformations due to failure of neural tube closure present in utero (types 2-5). Heterogeneity among patients with a Chiari deformity and incomplete understanding of its pathophysiologic characteristics have led to inconsistency in radiologic reporting and difficulty in defining appropriate management strategies tailored to an individual patient's condition. The radiologist is tasked with going beyond the criteria for cerebellar tonsillar herniation to define an individual patient's disease state, determine candidacy for surgery, and assist in selecting the proper surgical approach. In addition, the radiologist must be able to identify conditions that result in cerebellar tonsillar herniation that are not related to Chiari deformity to avoid inappropriate surgery. Last, the radiologist must be able to interpret postoperative imaging examinations to assess for adequacy of treatment and complications. The authors summarize recent literature regarding the pathophysiologic basis of Chiari 1 and related deformities and detail the ideal morphologic and physiologic imaging assessment, focusing on Chiari 1 and related deformities (Chiari 0, 0.5, and 1.5). Also discussed are surgical techniques and "pearls" of postsurgical imaging, including complications that must be recognized. This review provides clarity to a commonly encountered but less understood condition to optimize outcomes for patients with Chiari 1 and related deformities.
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