Introduction Chronic and progressive joint disease following loss of protective sensation leads to destruction of joints and surrounding bony structures Epidemiology incidence rare condition in the upper extremity location of neuropathic joints shoulder & elbow (this topic) foot & ankle (see diabetic Charcot foot) Pathophysiology neurotrauma loss of peripheral sensation and proprioception leads to repetitive microtrauma to the joint poor fine motor control generates unnatural pressure on certain joints leading to additional microtrauma neurovascular neuropathic patients have dysregulated reflexes and desensitized joints that receive significantly greater blood flow the resulting hyperemia leads to increased osteoclastic resorption of bone Genetics molecular biology RANK/RANKL/OPG triad pathway is thought to be involved Associated conditions shoulder syringomyelia most common etiology of neuropathic arthropathy of the upper extremity 25% of Charcot joints are a result of syringomyelia monoarticular (shoulder > elbow) Hansen's disease (leprosy) second most common cause of upper extremity neuropathic arthropathy syphilis usually affects the knee can be polyarticular diabetes most common cause of foot and ankle neuropathic joints Arnold-Chiari malformation cervical spondylosis adhesive arachnoiditis and TB arachnoiditis posttraumatic syringomyelia alcoholism elbow syringomyelia syphilis congenital insensitivity to pain diabetes Charcot-Marie-Tooth Classification Eichenholtz Classification Stage 0 • Joint edema • Radiographs are negative • Bone scan may be positive in all stages Stage 1 • Joint edema • Radiographs show osseous fragmentation with joint dislocation Stage 2 • Decreased local edema • Radiographs show coalescence of fragments and absorption of fine bone debris Stage 3 • No local edema • Radiographs show consolidation and remodeling of fracture fragments Presentation Symptoms swollen shoulder or elbow 50% have pain, 50% are painless loss of function Physical exam inspection swollen, warm, erythematous joint mimics infection motion joint may be mechanically unstable loss of active motion, but passive motion is maintained neurovascular a neurologic evaluation is essential Imaging Radiographs recommended views standard views of affected joint AP and scapula Y of the shoulder AP and lateral of the elbow findings early changes degenerative changes may mimic osteoarthritis late changes obliteration of joint space fragmentation of both articular surfaces of a joint leading to subluxation or dislocation scattered "chunks" of bone in fibrous tissue joint distention by fluid surrounding soft tissue edema heterotopic ossification fracture MRI indications MRI of cervical spine to rule out syrinx when neuropathic shoulder arthropathy is present Bone scan technetium bone scan findings may be positive (hot) for neuropathic joints and osteomyelitis indium WBC scan findings will be negative (cold) for neuropathic joints and positive (hot) for osteomyelitis useful to differentiate from osteomyelitis Studies Labs ESR and WBC can be elevated making it difficult to differentiate from osteomyelitis Histology synovial hypertrophy detritic synovitis (cartilage and bone distributed in synovium) Differential Osteomyelitis/septic joint difficult to distinguish from osteomyelitis based on radiographs and physical exam common findings in both conditions swelling, warmth elevated WBC and ESR technetium bone scan is "hot" unique to Charcot joint disease indium leukocyte scan will be "cold" (negative) will be "hot" (positive) for osteomyelitis Treatment Nonoperative rest, elevation, protected immobilization with a sling, and restriction of activity indications neuropathic shoulder joint functional bracing indications neuropathic elbow joint technique should allow flexion-extension, but neutralizes varus-valgus stresses Operative arthrodesis do not attempt during acute inflammatory stage (Eichenholtz 0-2) because of continued bone erosion only perform during quiescent stage (Eichenholtz 3) requires long periods of immobilization total joint replacement indications Charcot joint is a contraindication to total joint replacement due to poor bone stock, prosthetic loosening, instability, and soft-tissue compromise
QUESTIONS 1 of 2 1 2 Previous Next (OBQ05.259) A 62-year-old female presents with chronic shoulder pain. She denies any recent or remote history of trauma or infection. A radiograph is provided in Figure A. Which of the following is the most common cause of her findings? Review Topic QID: 1145 FIGURES: A 1 Diabetes 15% (218/1429) 2 Syphilis 4% (59/1429) 3 Alcoholism 14% (204/1429) 4 Syringomyelia 62% (884/1429) 5 Uremia 4% (54/1429) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 4 (OBQ04.168) A 50-year-old wheelchair-bound male with a history of traumatic spinal cord injury presents with 6 months of progressive, painless left shoulder weakness and decreased range of motion. He is afebrile and CBC, ESR, and C-reactive protein levels are normal. A radiograph is shown in Figure A. Early management should include: Review Topic QID: 1273 FIGURES: A 1 HIV testing 10% (102/1046) 2 cervical spine MRI 79% (823/1046) 3 repeat ESR, C-reactive protein, CBC 4% (38/1046) 4 emergent open reduction and internal fixation 2% (20/1046) 5 emergent irrigation and drainage 6% (61/1046) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 2