Cervical Diastematomyelia: A Case Presentation and Systematic Review

Abstract

Diastematomyelia is a rare congenital disorder characterized by the separation of the spinal cord by an osseocartilaginous or fibrous septum. While diastematomyelia has been reported to be more common in the thoracic and lumbar regions, the true incidence of cervical diastematomyelia is currently unknown. In this study, we conducted the most comprehensive systematic review to date of all other case reports of diastematomyelia to better characterize the incidence of cervical diastematomyelia, and provide comprehensive statistics on the clinical characteristics of diastematomyelia generally. Ninety-one articles were included in our study, comprised of 252 males (27.9%) and 651 females (72.0%) (and one patient with unspecified gender). In 507 cases, the vertebral level of the diastematomyelia was described, and we recorded those levels as either cervical (n=8, 1.6%), thoracic (n=220, 43.4%), lumbar (n=277, 54.6%), or sacral (n=2, 0.4%). In 719 cases, the type of diastematomyelia was specified as either Type I (n=482, 67.0%) or Type II (n=237, 33.0%). Our study found that diastematomyelia has been reported in the cervical region in only 1.6% of cases, and we provide comprehensive data that this disorder occurs in females to males with an approximately 2.6:1 ratio, and Type I vs. Type II diastematomyelia in an estimated 2:1 ratio.