Páscoa Pinheiro Joana, Fernandes Pedro, Sarmento Marco
Centro Hospitalar Universitário Lisboa Norte (North Lisbon University Hospital Centre), Lisbon, Portugal.
JBJS Case Connect. 2023 Feb 3;13(1). doi: e22.00217. eCollection 2023 Jan 1.
We describe an adult patient with bilateral Sprengel deformity (BSD) with bilateral omovertebral bone and thoracic spinal failure of segmentation (T2-T4) successfully surgically treated only at 19-year-old. After resection of the bilateral omovertebral bone and simultaneous relocation of the scapula, disabilities of the arm, shoulder, and hand score improved from 26.7 preoperatively to 2.5 at the 3-year follow-up.
The surgical treatment of Sprengel deformity is typically recommended between 3 and 7 years of age when significant shoulder impairment and/or severe cosmetic concerns are present. The authors were able to achieve a successful surgical outcome in a patient with BSD despite an unusual late presentation.
我们描述了一名患有双侧先天性高肩胛症（BSD）的成年患者，其双侧存在肩胛舌骨肌和T2 - T4节段性胸椎发育不全，仅在19岁时成功接受了手术治疗。在切除双侧肩胛舌骨肌并同时重新定位肩胛骨后，手臂、肩部和手部功能障碍评分从术前的26.7改善至3年随访时的2.5。
当存在明显的肩部功能障碍和/或严重的美容问题时，通常建议在3至7岁之间对先天性高肩胛症进行手术治疗。尽管该患者表现异常且就诊较晚，但作者仍成功实现了手术治疗效果。
