Charcot arthropathy of elbow due to syringomyelia: a case series and systematic review of literature

Abstract

Syringomyelia is an important etiology of Charcot arthropathy of the elbow. We present five interesting patients, along with a systematic literature review summarizing the clinical profile and management of syringomyelia-induced Charcot arthropathy of the elbow. PUBMED, SCOPUS, EMBASE, and Science Direct databases were screened for English articles published between 1980 and 2022 using the search query: "Syringomyelia" AND "elbow" AND ("arthropathy" OR "neuropathic" OR "Charcot"). Articles without full text and/or lack of conclusive evidence of elbow arthropathy due to syringomyelia were excluded. The reference lists of the selected articles were reviewed to identify additional articles describing syringomyelia-induced Charcot arthropathy of the elbow. All five patients in the current series had elbow arthritis with variable motor weakness and dissociated sensory loss. The literature review included 31 reports (45 patients) and five patients from our center (n = 50). The median age at presentation was 45 (13-77) years. The median duration of arthropathy was 24 (0.5-180) months. Thirty-three patients had isolated elbow arthropathies. The other joints affected included the shoulder (n = 13), wrist (n = 7), metacarpophalangeal joints (n = 3), and interphalangeal joints (n = 1). Chiari malformations were present in 33 (66%) patients. Sensory deficits, motor deficits, and ulnar neuropathies were described in 36 (72%), 31 (62%), and 14 (28%) patients, respectively. Surgical decompression for syringomyelia was performed in 13 (26%) patients. The presence of dissociated sensory loss, with or without motor weakness, is key to the suspicion of syringomyelia-induced Charcot arthropathy of elbow. Chiari malformation and ulnar neuropathy are frequently associated with this condition. Key Points • Charcot arthropathy of elbow is not so uncommon as believed • Syringomyelia is an important etiology of Charcot arthropathy of elbow • Therefore, all patients with elbow arthropathy of unknown etiology must be evaluated for dissociative sensory loss • Chiari malformation and ulnar neuropathy are commonly associated with syringomyelia-induced Charcot arthropathy of elbow joint.

Keywords: Charcot arthropathy; Chiari malformation; Elbow; Syringomyelia.