Du Jerry, Aichmair Alexander, Lykissas Marios, Girardi Federico
Weill Cornell Medical College, New York, New York, United States.
Department of Spine and Scoliosis Surgery, Hospital for Special Surgery, New York, New York, United States.
Evid Based Spine Care J. 2014 Apr;5(1):57-62. doi: 10.1055/s-0034-1368669.
Study Design Case report. Objective Amyoplasia-type arthrogryposis is a rare congenital disease that presents as multiple contractures involving various areas of the body. To the authors' knowledge, there have been no reports of adults with amyoplasia in the current literature. We report a case of an adult patient with cervical stenosis in the setting of amyoplasia. Patients and Methods A 48-year-old patient with amyoplasia and over 30 previous orthopedic reconstructive surgeries presented with neck pain radiating down his left shoulder and into the fingers, dysesthesia in his fingertips, and left-sided periauricular headache. A diagnosis of central spinal canal stenosis and bilateral foraminal stenosis at C3-C7 with radiculopathy was made based on computed tomography scans. Because of a prior right-side sternocleidomastoid muscle transfer, a left-side C3-C4, C5-C7 anterior cervical discectomy and fusion procedure was performed. Results The patient experienced significant improvement in symptoms that was transient. Symptoms returned to preoperative values after 1 year, despite significant and persistent improvement in stenosis. Conclusions Both amyoplasia and cervical stenosis can manifest in neurologic symptoms. Distinguishing the causing pathology can be challenging. The radiographic improvement of cervical stenosis in a patient with amyoplasia is not always associated with long-standing pain relief.
研究设计 病例报告。目的 先天性多发性关节挛缩症是一种罕见的先天性疾病,表现为身体多个部位的挛缩。据作者所知,目前文献中尚无成人先天性多发性关节挛缩症的报道。我们报告一例先天性多发性关节挛缩症成年患者合并颈椎管狭窄的病例。患者与方法 一名48岁先天性多发性关节挛缩症患者,既往接受过30多次骨科重建手术,现出现颈部疼痛,放射至左肩并延伸至手指,指尖感觉异常,以及左耳周左侧头痛。根据计算机断层扫描结果,诊断为C3 - C7节段中央椎管狭窄和双侧椎间孔狭窄伴神经根病。由于既往行右侧胸锁乳突肌转移术,遂行左侧C3 - C4、C5 - C7前路颈椎间盘切除融合术。结果 患者症状有显著改善,但为短暂性。尽管狭窄有显著且持续的改善,但1年后症状恢复到术前水平。结论 先天性多发性关节挛缩症和颈椎管狭窄均可表现为神经症状。区分病因病理可能具有挑战性。先天性多发性关节挛缩症患者颈椎管狭窄的影像学改善并不总是与长期疼痛缓解相关。