BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
BACKGROUND: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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.