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.
1University of Central Florida College of Medicine, Orlando, Florida
2Orlando Veterans Affairs Medical Center, Orlando, Florida
E-mail address for M.M. Rickert: firstname.lastname@example.org
Investigation performed at the University of Central Florida College of Medicine, Orlando, Florida
Disclosure: There was no source of external funding for this study. The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSREV/A522).