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Craniocervical Instability in Ehlers-Danlos Syndrome-A Systematic Review of Diagnostic and Surgical Treatment Criteria.

Laura-Nanna LohkampNandan Amrit MaratheMichael G Fehlings
Published in: Global spine journal (2022)
There is a lack of high quality, prospective evidence regarding the evaluation of suspected CCI in patients with EDS. Based on our systematic review, we recommend that the CXA, Harris measurement, Grabb-Mapstone-Oakes measurement, and the angular displacement of C1 to C2 be used to evaluate suspected CCI in EDS patients. Surgical fixation of suspected CCI should only be performed in cases with clear radiographic presence of instability and concordant symptoms/signs. Consensus-based guidelines and care pathways are required.
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