目的:平山病(HD)是一种罕见的疾病,非家族性,自我限制,进行性下脊髓型颈椎病,导致上肢远端运动障碍,模仿高尺骨神经病,下躯干臂丛病,或C8-T1神经根病。虽然大多数文献集中在病理生理学和疾病进展的预防,关于改善疾病稳定患者上肢功能的治疗讨论仍然有限.回顾了HD的上肢表现以及恢复手功能的手术选择。
方法:对接受重建以改善手功能的HD患者进行回顾性分析。人口统计数据,术前电诊断和肌电图,收集体格检查结果。结果数据涉及术后抓地力,捏,和功能评估记录在临床访问。描述了外科技术的定性描述。
结果:在确定的六名患者中,4例符合纳入标准,接受了肌腱转移和选定的关节.所有患者都被诊断为青少年,右手占主导地位,三个是男性。一名患者出现单侧症状,其余患者出现双侧症状。所有患者都接受了拇指对位的肌腱转移治疗,抓,反爪子,拇指指间关节固定术。所有患者术后握力均有改善。平均随访3.2年。
结论:平山病是一种罕见的疾病,通常由脊柱外科医生和神经科医师管理,他们可能不知道恢复手功能缺陷的选择。尚未充分描述改善HD手功能的技术策略和结果。改善手功能的手术选择适合缺陷,包括肌腱转移,选择关节,和/或肌腱。必须小心管理手部重建后疾病进展和期望的风险。
■治疗性V.
OBJECTIVE: Hirayama disease (HD) is a rare, nonfamilial, self-limiting, progressive lower cervical myelopathy, resulting in debilitating distal upper-extremity motor deficits, mimicking high ulnar neuropathy, lower trunk brachial plexopathy, or C8-T1 radiculopathy. Although most literature focuses on pathophysiology and prevention of disease progression, there remains limited discussion regarding treatment to improve upper-extremity function in patients with stable disease. The upper-extremity manifestations of HD are reviewed along with surgical options for restoring hand function.
METHODS: A retrospective review of patients with HD who underwent reconstruction to improve hand function was undertaken. Demographic data, preoperative electrodiagnostic and electromyographic, and physical examination findings were collected. Outcome data involved postoperative grip, pinch, and functional assessment documented on clinical visits. Qualitative descriptions of the surgical techniques are described.
RESULTS: Among six patients identified, four met the inclusion criteria and underwent tendon transfers and selected joint arthrodeses. All patients were diagnosed as teenagers, were right hand-dominant, and three were male. Unilateral symptoms were present in one patient and were bilateral in the rest. All patients were treated with tendon transfers for thumb opposition, grasp, anticlaw, and thumb interphalangeal joint arthrodesis. All patients had postoperative grip strength improvement. The average follow-up was 3.2 years.
CONCLUSIONS: Hirayama disease is a rare disease often managed by spine surgeons and neurologists who may be unaware of options for restoring hand function deficits. Technical strategies and outcomes of improving hand function in HD have not been adequately described. Surgical options to improve hand function are tailored to the deficits and include tendon transfers, select joint arthrodeses, and/or tenodeses. Risk of disease progression and expectations following hand reconstruction must be managed carefully.
UNASSIGNED: Therapeutic V.