Rickert Mariel M, Cannon Jennifer G, Kirkpatrick John S
University of Central Florida College of Medicine, Orlando, Florida.
Orlando Veterans Affairs Medical Center, Orlando, Florida.
JBJS Rev. 2019 Oct;7(10):e1. doi: 10.2106/JBJS.RVW.18.00155.
Neuropathic arthropathy of the shoulder is a chronic progressive process characterized by joint destruction in the presence of a neurosensory deficit. Causes include syringomyelia, syphilis, diabetes, chronic alcoholism, and leprosy, with syringomyelia accounting for the vast majority of upper-extremity Charcot joints. Early presentation of this rare condition includes nonspecific symptoms such as swelling, erythema, sensory symptoms, and decreased functionality, making diagnosis challenging.
We systematically reviewed 32 case reports published between 1924 and 2016. A total of 59 shoulders from 56 patients are included in this analysis. Variables include patient demographic characteristics, presentation, etiology, diagnostic techniques, treatment, outcome, and follow-up of Charcot shoulder.
We compiled a total of 25 right shoulders (42%), 24 left shoulders (41%), and 10 shoulders (17%) with unspecified laterality. The mean patient age (and standard deviation) was 49 ± 11 years, and the median age was 47 years. There was a higher prevalence in men (37 shoulders [63%]) compared with women (22 shoulders [37%]). Presenting symptoms included reduced range of motion (53 shoulders [90%]), paresthesia or hypoesthesia (45 [76%]), swelling (44 [75%]), weakness (40 [68%]), pain (31 [53%]), and reduction in deep tendon reflexes (22 [37%]). Shoulder radiographs were made in all cases. The presence of a syrinx was detected in 45 shoulders (76%) with magnetic resonance imaging, myelography, or clinical diagnosis. Sixteen shoulders (27%) reported exposure to trauma, with a 69% decrease in time from presentation to diagnosis compared with non-traumatic cases. Treatment was categorized as solely nonoperative management (14 [24%]), operative management (13 [22%]), combined therapy (20 [34%]), and no treatment listed (10 [17%]). Two surgical cases (3%) were excluded from our treatment group analysis as they were treated for unrelated or misdiagnosed conditions.
Our study increases awareness and understanding of this complex, progressive disease to reduce delay and misdiagnosis and to contribute to the standard-of-care recommendations.
Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
肩部神经性关节病是一种慢性进行性疾病，其特征是在存在神经感觉缺陷的情况下出现关节破坏。病因包括脊髓空洞症、梅毒、糖尿病、慢性酒精中毒和麻风病，其中脊髓空洞症是上肢夏科关节的绝大多数病因。这种罕见疾病的早期表现包括肿胀、红斑、感觉症状和功能下降等非特异性症状，这使得诊断具有挑战性。
我们系统回顾了1924年至2016年间发表的32例病例报告。本分析共纳入了56例患者的59个肩部。变量包括患者的人口统计学特征、表现、病因、诊断技术、治疗、结果以及夏科肩部的随访情况。
我们共汇总了25个右肩（42%）、24个左肩（41%）和10个未明确侧别的肩部（17%）。患者的平均年龄（及标准差）为49±11岁，年龄中位数为47岁。男性患病率较高（37个肩部[63%]），女性患病率较低（22个肩部[37%]）。出现的症状包括活动范围减小（53个肩部[90%]）、感觉异常或感觉减退（45个[76%]）、肿胀（44个[75%]）、无力（40个[68%]）、疼痛（31个[53%]）以及深部腱反射减弱（22个[37%]）。所有病例均进行了肩部X线检查。通过磁共振成像、脊髓造影或临床诊断，在45个肩部（76%）中检测到脊髓空洞症。16个肩部（27%）报告有外伤史，与非外伤病例相比，从出现症状到诊断的时间缩短了69%。治疗分类为单纯非手术治疗（14个[24%]）、手术治疗（13个[22%]）、联合治疗（20个[34%]）以及未列出治疗方式（10个[17%]）。有2例手术病例（3%）被排除在我们的治疗组分析之外，因为它们是因无关或误诊的疾病接受治疗。
我们的研究提高了对这种复杂的进行性疾病的认识和理解，以减少延误和误诊，并为护理标准建议做出贡献。
治疗性四级。有关证据水平的完整描述，请参阅作者指南。
