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, which comprised 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 female-to-male ratio of approximately 2.6:1 and Type I versus Type II diastematomyelia in an estimated ratio of 2:1.

Conceptualization: All authors.

Data curation: J.F.Z.

Formal analysis: J.F.Z.

Funding acquisition: N/A

Investigation: All authors.

Methodology: All authors.

Project administration: J.A.F., O.F., and L.D.T.

Resources: N/A

Supervision: J.A.F., O.F., and L.D.T.

Validation: All authors.

Writing—original draft: J.F.Z., O.S., and O.K.

Writing—reviewing and editing: All authors.

Written and verbal consents were obtained from all patients or their health care proxies for all aspects related to this report and prior to any procedures which were performed.

Data supporting the findings of this study will be made available by the corresponding author upon request.

*Both contributed equally as co-second authors.

Received: 17 October 2023

Accepted: 29 February 2024

Accepted Manuscript online:
04 May 2024

Article published online:
24 May 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany