Updated: 10/10/2016

Neuropathic (Charcot) Joint of Shoulder & Elbow

Topic
Review Topic
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Questions
2
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Evidence
3
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https://upload.orthobullets.com/topic/9044/images/xray shoulder_moved.jpg
https://upload.orthobullets.com/topic/9044/images/neuropathic shoulder.jpg
https://upload.orthobullets.com/topic/9044/images/neuropathic elbow 1.jpg
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 post
  • 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
 

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Questions (2)

(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:
1

Diabetes

14%

(222/1608)

2

Syphilis

4%

(62/1608)

3

Alcoholism

13%

(215/1608)

4

Syringomyelia

65%

(1041/1608)

5

Uremia

4%

(58/1608)

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:
1

HIV testing

10%

(103/1072)

2

cervical spine MRI

79%

(843/1072)

3

repeat ESR, C-reactive protein, CBC

4%

(41/1072)

4

emergent open reduction and internal fixation

2%

(21/1072)

5

emergent irrigation and drainage

6%

(62/1072)

ML 2

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
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Topic COMMENTS (7)
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