Neuropathic (Charcot) Joint of Shoulder & Elbow

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Topic updated on 05/24/13 7:28am
Introduction
  • A chronic and progressive joint disease following loss of protective sensation
    • leads to destruction of joints and surrounding bony structures.
    • commonly involves
      • shoulder & elbow (this topic)
      • foot & ankle (see diabetic Charcot foot) 
  • Radiographic patterns 
    • atrophic type 
      • hip, shoulder, foot
      • massive bone destruction
    • hypertrophic type
      • knee, elbow, ankle
      • severe joint destruction
      • periarticular new bone formation
      • osteophytes, fractures and osseous debris
  • Location
    • shoulder  
      • associated conditions
        • syringomyelia, leprosy, syphilis, DM, Arnold-Chiari malformation, cervical spondylosis, adhesive arachnoiditis, TB arachnoiditis, posttraumatic syringomyelia
    • elbow  
      • associated conditions
        • syringomyelia, syphilis, congenital insensitivity to pain, DM, Charcot-Marie-Tooth
  • Etiology
    • CNS (hypertrophic) form
      • syringomyelia  
        • most common etiology of neuropathic shoulder arthropathy 
        • 25% of these patients develop a neuropathic joint
        • monoarticular (shoulder > elbow)
      • Hansen's disease (leprosy)
        • second most common cause of neuropathic shoulder arthropathy
      • myelomeningocele
        • leads to Charcot in ankle and foot
      • spinal cord tumors
        • affect lower extremities
      • syphilis
        • affects the knee
        • can be polyarticular
      • other causes
        • Charcot-Marie-Tooth, multiple sclerosis, chronic demyelinating polyradiculopathy, gigantism
    • PNS (atrophic) form
      • diabetes
        • most common cause of foot and ankle neuropathic joints
      • alcoholism
    • pediatrics
      • congenital insensitivity to pain
      • spinal dysraphism
      • Riley-Day syndrome
  • 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 de-sensitized joints receive significantly greater blood flow.
      • the resulting hyperemia leads to increased osteoclastic resorption of bone
  • Molecular biology
    • RANK/RANKL/OPG triad pathway thought to be involved post
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
    • swollen, warm, erythematous joint
      • mimics infection
    • joint may be mechanically unstable
    • loss of active motion, but passive motion is maintained
Imaging
  • Radiographs
    • views
      • obtain standard views of affected joint
    • 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
  • MRI
    • obtain an MRI of cervical spine to rule out syrinx whenever a patient has neuropathic shoulder arthropathy  
  • Bone scan
    • technecium bone scan
      • may be positive for neuropathic joint and osteomyelitis
    • indium WBC scan
      • will be negative (cold) for neuropathic joints and positive (hot) for osteomyelitis
      • this is a valuable study to differentiate from osteomyelitis
Studies
  • Laboratory
    • 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
    • very difficult to distinguish from osteomyelitis based on radiographs and physical exam
      • common to both conditions are
        • swelling, warmth
        • variable 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 for a total joint replacement
          • because of poor bone stock, prosthetic loosening and instability secondary to soft-tissue compromise

 

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Qbank (2 Questions)

TAG
(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? Topic Review Topic
FIGURES: A          

1. Diabetes
2. Syphilis
3. Alcoholism
4. Syringomyelia
5. Uremia

PREFERRED RESPONSE ▶
TAG
(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: Topic Review Topic
FIGURES: A          

1. HIV testing
2. cervical spine MRI
3. repeat ESR, C-reactive protein, CBC
4. emergent open reduction and internal fixation
5. emergent irrigation and drainage

PREFERRED RESPONSE ▶



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