From the Department of Orthopaedics, UT Health San Antonio (Dr. Prabhakar, Gonuguntla, Momtaz, Dr. Chaput), San Antonio, TX (Dr. Prabhakar, Dr. Gonuguntla, Dr. Momtaz, and Dr. Chaput); and the Department of Orthopaedics, Boston Children's Hospital/Harvard Medical School, Boston, MA (Dr. Hogue).
J Am Acad Orthop Surg Glob Res Rev. 2023 Aug 15;7(8). doi: 10.5435/JAAOSGlobal-D-23-00061. eCollection 2023 Aug 1.
Ehlers-Danlos syndrome (EDS) is a rare inherited connective tissue disorder characterized by collagen synthesis disruption, resulting in joint hyperlaxity, skin and vascular fragility, and bleeding diathesis. Patients with EDS are susceptible to spinal deformities, with scoliosis accounting for up to 23.4% of musculoskeletal abnormalities. Conservative management is often trialed initially; however, severe scoliosis can lead to significant sagittal imbalance and cardiopulmonary compromise. Surgical intervention for scoliosis correction in patients with EDS presents unique challenges because of tissue fragility and an increased risk of vascular and wound complications. This case report discusses a 20-year-old man with type II EDS and scoliosis, who experienced retroperitoneal compartment syndrome, significant left lower extremity weakness, and loss of sensation after scoliosis correction surgery. The report also provides an overview of the existing literature on scoliosis surgery outcomes in patients with EDS, highlighting the need for heightened vigilance and cautious surgical approaches.
埃勒斯-当洛斯综合征(EDS)是一种罕见的遗传性结缔组织疾病,其特征是胶原合成障碍,导致关节过度松弛、皮肤和血管脆弱以及出血倾向。EDS 患者易发生脊柱畸形,其中脊柱侧凸占骨骼肌肉异常的 23.4%。最初通常会尝试保守治疗;然而,严重的脊柱侧凸可导致明显的矢状面失衡和心肺功能受损。由于组织脆弱和血管及伤口并发症风险增加,EDS 患者脊柱侧凸矫正的手术干预存在独特的挑战。本病例报告讨论了一名 20 岁的 II 型 EDS 伴脊柱侧凸患者,其在脊柱侧凸矫正手术后出现了腹膜后间隙综合征、左侧下肢严重无力和感觉丧失。报告还概述了 EDS 患者脊柱侧凸手术结果的现有文献,强调需要高度警惕和谨慎的手术方法。