Is there a morphometric cause of Chiari malformation type I? Analysis of existing literature

Abstract

Although many etiologies have been proposed for Chiari malformation type I (CM-I), there currently is no singular known cause of CM-I pathogenesis. Advances in imaging have greatly progressed the study of CM-I. This study reviews the literature to determine if an anatomical cause for CM-I could be proposed from morphometric studies in adult CM-I patients. After conducting a literature search using relevant search terms, two authors screened abstracts for relevance. Full-length articles of primary morphometric studies published in peer-reviewed journals were included. Detailed information regarding methodology and symptomatology, craniocervical instability, syringomyelia, operative effects, and genetics were extracted. Forty-six studies met inclusion criteria, averaging 93.2 CM-I patients and 41.4 healthy controls in size. To obtain measurements, 40 studies utilized MRI and 10 utilized CT imaging, whereas 41 analyzed parameters within the posterior fossa and 20 analyzed parameters of the craniovertebral junction. The most commonly measured parameters included clivus length (n = 30), tonsillar position or descent (n = 28), McRae line length (n = 26), and supraocciput length (n = 26). While certain structural anomalies including reduced clivus length have been implicated in CM-I, there is a lack of consensus on how several other morphometric parameters may or may not contribute to its development. Heterogeneity in presentation with respect to the extent of tonsillar descent suggests alternate methods utilizing morphometric measurements that may help to identify CM-I patients and may benefit future research to better understand underlying pathophysiology and sequelae such as syringomyelia.

Keywords: Anatomy; Chiari; Imaging; Malformation; Morphometric; Morphometric Analysis; Radiography; Review; Symptomatology; Type 1.