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.

Ehlers-Danlos 综合征 (EDS) 是一种罕见的遗传性疾病，可导致颈椎韧带松弛和过度活动。一部分患者可能会出现与疼痛和神经功能障碍相关的颅颈交界临床不稳定，可能需要接受枕颈固定 (OCF) 治疗。由于难以区分疾病固有的过度活动和需要干预的真正病理不稳定，EDS 患者的手术决策可能会变得复杂。在这里，我们全面回顾了可用的医学文献，以批判性地评估 EDS 人群中各种提议的不稳定性定义背后的证据，并总结了 OCF 后可用的结果数据。已经使用了几个射线照相参数，包括斜轴角，基底轴间隔和 pB-C2 测量。尽管脊柱外科医生对 EDS 的认识越来越多，但仍然缺乏支持 EDS 患者脊柱不稳定的放射学参数的数据。此外，缺乏关于手术治疗该人群中普遍存在的慢性衰弱性疼痛疗效的高质量证据。需要更标准化的临床措施和严格的研究方法来阐明手术干预在这一复杂患者群体中的作用。缺乏高质量的证据表明手术治疗对这一人群中普遍存在的慢性衰弱性疼痛的疗效。需要更标准化的临床措施和严格的研究方法来阐明手术干预在这一复杂患者群体中的作用。缺乏高质量的证据表明手术治疗对这一人群中普遍存在的慢性衰弱性疼痛的疗效。需要更标准化的临床措施和严格的研究方法来阐明手术干预在这一复杂患者群体中的作用。