Working Diagnosis:
Charcot Joint of the left shoulder secondary to Chiari I malformation

Treatment:
Given the extent of her pathology, total shoulder replacement is unlikely to return her shoulder function. A complex proximal humeral revision arthroplasty versus arthrodesis could be contemplated but risk new-onset pain with higher failure rate. Therefore, after patient education and discussion of her options she has elected to proceed with physical therapy and continued conservative treatment.

Outcome:
The patient will begin strength training with physical therapy to regain as much function in her arm as possible. There are no restrictions on her return to activity other than functional limitation. Should she develop any pain or change in symptoms she will follow up as needed.

Author's Comments:
Research has shown 25% of Chiari I malformations with syringomyelia are complicated by Charcot joint. Of the two proposed theories, the neurotraumatic theory suggests that loss of proprioception leads to repetitive trauma to the bones of the joint. The neurovascular theory proposes that the decreased neural activity, and therefore decreased sympathetic tone, results in vasodilation and hyperemia which promote bone resorption, likely what our patient suffered from. At this time, neuropathic arthropathy is listed as a contraindication for most FDA approved shoulder arthroplasty devices due to concerns the implant will fail due to bone destruction.

Editor's Comments:
The author presents a very unique cause of this patients shoulder pain and immobility. A recent literature review (4) found 34 cases of neuropathic arthropathy caused by Chiari Malformation with syringomyelia. The mean age of patients was 45 years old, with a female predominance, and 50% of the cases involved the shoulder. Goals of treatment include early diagnosis, therapy to keep range of motion and treatment of the underlying disease by neurosurgery.

References:
1. Meyer GA, Stein J, Poppel MH. Rapid osseous changes in syringomyelia. Radiology. 1957;69(3): 415-418.
2. Jones EA, Manaster BJ, May DA, Disler DG. Neuropathic osteoarthropathy: diagnostic dilemmas and differential diagnosis. Radiographics. 2000;20: S279-93.
3. Rickert MM, Cannon JG, Kirkpatrick JS. Neuropathic arthropathy of the shoulder: a systematic review of classifications and treatments. JBJS Rev. 2019;7(10).

4. Wang X, Li Y, Gao J, Wang T, Li Z. Charcot arthropathy of the shoulder joint as a presenting feature of basilar impression with syringomyelia: A case report and literature review. Medicine (Baltimore). 2018;97(28):e11391. doi:10.1097/MD.0000000000011391

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