Updated: 6/27/2020

Neuropathic (Charcot) Joint of the Elbow

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Introduction
  • Overview
    • neuropathic (charcot) elbow is a chronic and progressive joint disease most commonly caused by syringomyelia leading to the destruction of the joint and surrounding bony structures
      • treatment should be individualized based on degree of functional limitation and underlying condition. 
  • Epidemiology
    • incidence 
      • rare condition in the upper extremity (~ 40 cases reported in literature)
    • location of neuropathic joints
      • elbow (this topic)
      • shoulder 
      • foot & ankle (see diabetic Charcot foot) 
  • 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 post
  • 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 fusion position for most activities is 110 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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