Department of Surgery, Penn State College of Medicine, 500 University Drive, Hershey, PA, USA.
Sports Medicine Institute, Hospital for Special Surgery, New York, NY, USA.
BMC Musculoskelet Disord. 2024 Oct 24;25(1):846. doi: 10.1186/s12891-024-07937-6.
Patients with Ehlers-Danlos syndromes (EDS) often experience high rates of joint subluxations and dislocations, and associated pain that may require surgical interventions. Orthopaedic surgical management is challenging in this population, and patients will often undergo multiple unsuccessful surgeries. Outcomes data specific to patients with EDS are sparse in the orthopaedic surgery literature. We conducted a scoping review to evaluate the evidence and outcomes for orthopaedic surgery specifically for the EDS population.
PubMed MEDLINE, Embase, The Cochrane Library, Cochrane Controlled Register of Trials (CENTRAL), CINHL, and Scopus from their inception to February 28, 2024 for all studies that reported outcomes for orthopaedic surgery in patients with EDS. Two reviewers independently determined study eligibility, rated study quality, and extracted data. Methodology followed the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR). The studies in this scoping review include Level III (retrospective cohort and case control) and Level IV (case series) evidence.
The literature search yielded a total of 71 citations published between 1990 and 2023. All were primary studies. 38 were single case studies, 14 were case series, and 19 were retrospective cohort studies. No randomized clinical studies or systematic reviews were identified. Overall, the reported findings for the various anatomical sites and procedures indicated that surgery outcomes were inconsistent. Our review highlights the need for future research to determine whether currently established surgical approaches for various orthopaedic conditions offer long-term clinical benefit in patients with EDS. This is clearly a challenging diagnosis, and more rigorous clinical studies are required to identify optimal treatment approaches.
Our review found little evidence-based research to guide optimal surgical treatment in EDS. Established surgical techniques that have been shown to be successful in the wider orthopaedic population should be studied to determine their efficacy in the EDS population.
患有埃勒斯-当洛斯综合征（EDS）的患者常经历高频率的关节半脱位和脱位，以及相关的疼痛，可能需要手术干预。在这种情况下，骨科手术管理具有挑战性，患者通常会经历多次不成功的手术。在骨科手术文献中，针对 EDS 患者的具体结果数据很少。我们进行了范围综述，以评估专门针对 EDS 人群的骨科手术的证据和结果。
从其成立到 2024 年 2 月 28 日，通过 PubMed MEDLINE、Embase、The Cochrane Library、Cochrane 对照试验注册中心（CENTRAL）、CINHL 和 Scopus 检索所有报告 EDS 患者骨科手术结果的研究。两名审查员独立确定研究的合格性、评价研究质量并提取数据。方法遵循系统评价和荟萃分析扩展的首选报告项目（PRISMA-ScR）。本综述中的研究包括 III 级（回顾性队列和病例对照）和 IV 级（病例系列）证据。
文献检索共产生了 1990 年至 2023 年期间发表的 71 篇引文。所有都是原始研究。其中 38 项为单病例研究，14 项为病例系列研究，19 项为回顾性队列研究。未发现随机临床试验或系统评价。总体而言，针对各种解剖部位和手术的报告结果表明，手术结果不一致。我们的综述强调需要进行未来的研究，以确定目前针对各种骨科疾病的手术方法是否为 EDS 患者提供长期的临床益处。这显然是一个具有挑战性的诊断，需要更严格的临床研究来确定最佳治疗方法。
我们的综述发现几乎没有循证研究来指导 EDS 的最佳手术治疗。已经在更广泛的骨科人群中显示出成功的既定手术技术应该进行研究，以确定其在 EDS 人群中的疗效。
