A Morphometric Assessment of type I Chiari Malformation above the McRae line: A Retrospective Case-Control Study in 302 Adult Female Subjects

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Thank you to Audrey Braun for her hard work on this project.

Abstract

Purpose. Type I Chiari malformation (CMI) is a radiologically-defined structural dysmorphism of the hindbrain and posterior cranial fossa (PCF). Traditional radiographic identification of CMI relies on the measurement of the cerebellar tonsils in relation to the foramen magnum with or without associated abnormalities of the neuraxis. The primary goal of this retrospective study was to comprehensively assess morphometric parameters above the McCrea line in a group of female CMI patients and normal controls.

Material and Methods. 29 morphological measurements were taken on 302 mid-sagittal MR images of adult female CMI patients (N=162) and healthy controls (N=140).  All MR images were voluntarily provided by CMI subjects through an online database and control participant images were obtained through the Human Connectome Project and a local hospital system. 

Results. Analyses were performed on the full dataset of adult female MR images and a restricted dataset of 229 participants that were equated for age, race, and body mass index. 18 group differences were identified in the PCF area that grouped into three clusters; PCF structures heights, clivus angulation, and odontoid process irregularity. 14 group differences persisted after equating CMI and control groups on demographic characteristics. 

Conclusion. PCF structures reliably differ in adult female CMI patients relative to healthy controls. These differences reflect structural abnormalities in the osseous and soft tissue structures of the clivus, odontoid process, and cerebellum. Clinical and pathophysiological implications are discussed.

Contributors

James R. Houston, Maggie S. Eppelheimer, Soroush Heidari Pahlavian, Dipankar Biswas, Aintzane Urbizu, Bryn A. Martin, Jayapalli Rajiv Bapuraj, Mark Luciano, Philip A. Allen, Francis Loth

Publication

Journal of Neuroradiology