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Ehlers-Danlos syndrome (EDS) is a rare hereditary condition that can result in ligamentous laxity and hypermobility of the cervical spine. A subset of patients can develop clinical instability of the craniocervical junction associated with pain and neurological dysfunction, potentially warranting treatment with occipitocervical fixation (OCF). Surgical decision-making in patients with EDS can be complicated by difficulty distinguishing from hypermobility inherent in the disease and true pathological instability necessitating intervention. Here we comprehensively review the available medical literature to critically appraise the evidence behind various proposed definitions of instability in the EDS population, and summarize the available outcomes data after OCF. Several radiographic parameters have been used, including the clivo-axial angle, basion-axial interval, and pB-C2 measurement. Despite increasing recognition of EDS by spine surgeons, there remains a paucity of data supporting proposed radiographic parameters for spinal instability among EDS patients. Furthermore, there is a lack of high-quality evidence concerning the efficacy of surgical treatments for chronic debilitating pain prevalent in this population. More standardized clinical measures and rigorous study methodologies are needed to elucidate the role of surgical intervention in this complex patient population. [ABSTRACT FROM AUTHOR]