Neuropathic arthropathy of the shoulder as a presenting feature of Chiari malformation with syringomyelia: a case report with a systematic literature review

Background Neuropathic arthropathy (NA) of the shoulder is a chronic progressive process characterized by joint destruction in the presence of a neurosensory deficit. Syringomyelia, a spinal cord disease, is the leading cause of NA in the upper extremity. Objective We present a systematic review of NA with syringomyelia cases alongside a case report of an adult with NA of the shoulder that occurs a few 4 years after a revelation and surgical management of a Chiari malformation with syringomyelia. Methods A systematic review was conducted following PRISMA guidelines. A PubMed, Scopus, Isiknowledge, and manual search through references of relevant publications were used to identify all published case reports of NA. Data were collected from each case report on patient characteristics. Results The systematic review identified 56 publications and 85 patients (including ours): nearly the same number of males ( n = 41) and females ( n = 44). The mean age was 50,69. Presentations included reduction of mobility ( n = 66), swelling ( n = 61) and sensory disorder ( n = 63). The pain was absent in 41 cases. In the majority of reported cases 56 (65.1%), syringomyelia was revealed by neuropathic arthropathy, and eleven patients (12.9%) had a history of syringomyelia. Treatment was categorized into non-operative management (37[43.5%]), operative management (27[31.7%]). Following-up was non-reported in 31 (36%) cases. Improvement was reported more with patients who underwent a surgical approach than medical one 28.5% versus 8.1%. Conclusion Physicians need to be more aware of this destructive joint disease, rare, and often misdiagnosed. Also, it is imperative to integrate clinical, pathological, and imaging findings for accurate diagnosis and for delivering appropriate therapy.