Updated: 6/6/2021

Neuropathic (Charcot) Joint of the Elbow

Review Topic
  • summary
    • Neuropathic Charcot Joint of the Elbow is a chronic and progressive joint disease most commonly caused by syringomyelia leading to the destruction of the elbow joint and surrounding bony structures.
    • Diagnosis is made with radiographs of the elbow and supplemented with cervical spine MRI to assess for a syrinx.
    • Treatment should be individualized based on degree of functional limitation and underlying neurological condition. Neurosurgical decompression is indicated in the presence of a syrinx.
  • Epidemiology
    • Incidence
      • rare condition in the upper extremity (~ 40 cases reported in literature)
    • Anatomic location
      • elbow (this topic)
      • shoulder
      • foot & ankle (see diabetic Charcot foot)
  • Etiology
    • Pathophysiology
      • syrinx formation
        • syrinx formation (fluid-filled cavity) in spinal cord causes damage to the decussating fibers of the lateral spinothalamic tract leading to loss of pain and temperature sensation
          • loss of pain/temperature leads to dissociative anesthesia in which proprioception and motor function are preserved but pain and temperature are not
        • as syrinx enlarges, damage to dorsal column and anterior horn of spinal cord lead to areflexia, loss of motor strength and muscle atrophy.
      • joint destruction
        • 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
      • orthopedic conditions
        • ulnar neuropathy
      • medical conditions & comorbidities
        • syringomyelia
          • most common etiology of neuropathic arthropathy of the upper extremity
            • 25% of Charcot joints are a result of syringomyelia
          • monoarticular (shoulder > elbow)
        • Arnold-Chiari malformation
          • most common cause of syringomyelia
        • Hansen's disease (leprosy)
          • second most common cause of upper extremity neuropathic arthropathy
        • neurosyphilis (tabes dorsalis)
          • usually affects the knee
          • can be polyarticular
        • diabetes
          • most common cause of foot and ankle neuropathic joints
  • Presentation
    • Symptoms
      • swollen elbow
      • 50% have pain, 50% are painless
      • loss of function
    • Physical exam
      • inspection
        • swollen, warm, erythematous joint
        • mimics infection
        • atrophy due to ulnar nerve entrapment
          • interosseous atrophy
          • hypothenar atrophy
          • clawing
      • motion
        • loss of active motion is most common finding
          • elbow flexion, extension, pronation and supination all affected
        • elbow joint may be mechanically unstable
        • loss of passive motion indicates mechanical block
      • neurovascular
        • a neurologic evaluation is essential
        • ulnar nerve entrapment at the elbow very common
          • paresthesias in ulnar nerve distribution
          • interosseous weakness
  • Imaging
    • Radiographs
      • recommended views
        • standard views of affected joint
          • 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 elbow 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
    • EMG/NCS
      • helpful for confirming ulnar neuropathy associated with diagnosis
    • 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, NSAIDs, functional bracing, restriction of activity and treatment of underlying disease
        • indications
          • first line treatment for neuropathic elbow joint
        • outcomes
          • 50% of patients reported improvement after non-operative management
      • intra-articular corticosteroid injection
        • indications
          • severe elbow pain
    • Operative
      • neurosurgical decompression
        • indications
          • presence of cervical syrinx
        • outcomes
          • decompression of syrinx has shown to slow disease progression, maximize joint function and improve bone quality
            • studies have shown some elbow joint space restoration following syrinx decompression
      • peripheral nerve neurolysis
        • indications
          • ulnar nerve palsies
          • PIN palsies
        • outcomes
          • limited cases series have shown good recovery of nerve function but high recurrent rates
      • elbow arthrodesis
        • indications
          • elbow pain and instability having failed conservative management
        • outcomes
          • limited case series have shown improvement of pain but with functional limitations
      • total joint replacement
        • indications
          • Charcot joint is considered a contraindication to elbow total joint replacement
            • due to poor bone stock, prosthetic loosening, instability, and soft-tissue compromise
        • outcomes
          • limited case reports exist on elbow arthroplasties for charcot elbow with mixed results
  • Techniques
    • Rest, NSAIDs, functional bracing, restriction of activity and treatment of underlying disease
      • technique
        • functional bracing
          • allows flexion-extension, but neutralizes varus-valgus stresses
    • Intra-articular corticosteroid injection
      • technique
        • ulnohumeral joint injection is considered most effective
    • Neurosurgical decompression
      • technique
        • neurosurgical management has been reported to consist of 1 or more of the following
          • posterior fossa decompression
          • craniotomy
          • syringoperitoneal shunt
          • laminectomy
    • Peripheral nerve neurolysis
      • technique
        • cubital tunnel release must be performed with careful soft tissue dissection due to severely altered anatomy noted in patients with charcot elbow
    • Elbow arthrodesis
      • technique
        • optimal position
          • optimal position for most activities is110 degrees flexion whereas 45-60 degrees flexion is optimal for work-related activities.
          • contoured plate fixation most commonly used to achieve arthrodesis
  • Complications
    • Ulnar nerve entrapment
      • most common complication of charcot elbow
    • Infection
      • risk factors
        • high risk with surgical intervention without management of underlying condition
    • Upper extremity DVT
      • risk factors
        • any surgical intervention
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