Updated: 5/13/2020

Neuropathic (Charcot) Joint of Shoulder

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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
  • Overview
    • neuropathic (charcot) shoulder is a chronic and progressive joint disease most commonly caused by syringomyelia leading to the destruction of the joint and surrounding structures
      • treatment should be individualized based on degree of functional limitation and underlying condition 
  • Epidemiology
    • incidence 
      • overall, very rare (around 70 total cases reported in literature)
      • 25% of individuals with syrinxes develop neuropathic arthropathy, with 80% of cases occurring in upper extremity
    • demographics
      • mean age at diagnosis is ~50
      • 2:1 male:female ratio 
    • location
      • shoulder (this topic)
      • elbow 
      • foot & ankle 
  • 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
    • shoulder 
      • syringomyelia  
        • most common etiology of neuropathic arthropathy of the upper extremity 
          • 25% of Charcot joints are a result of syringomyelia
        • monoarticular (shoulder > elbow)
        • cervical syringomyelia is the cause of 75% cases of charcot shoulder 
      • 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
      • Arnold-Chiari malformation
        • most common cause of syringomyelia
      • multiple sclerosis
      • end-stage renal disease
      • adhesive arachnoiditis and TB arachnoiditis
      • meningomyelocele
      • chronic alcoholism
Classification

 Eichenholtz classification has not been validated for charcot shoulder 

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
  • History
    • 30% of patients report trauma to the shoulder as the inciting event
  • Symptoms
    • swollen shoulder
    • 50% are painless
    • loss of function
    • joint instability
  • Physical exam
    • inspection
      • swollen, warm, erythematous joint
        • can mimic an infection
    • motion
      • loss of motion is most common finding (90%)
      • crepitus 
      • joint laxity with mechanically instability
    • neurovascular
      • decreased upper extermity muscle strength
      • sensory and temperature changes along patient's back and arms in cape-like distribution
      • asymmetric reflexes
        • areflexia common in late-stage disease
Imaging
  • Radiographs
    • recommended views
      • standard views of affected joint
        • AP and scapular Y of the shoulder
    • findings
      • gold-standard in diagnosis of Charcot shoulder
        • early changes
          • degenerative changes may mimic osteoarthritis
        • late changes
          • superomedial flattening of the humeral head 
          • periarticular soft-tissue calcifications 
          • glenoid sclerosis
          • extensive bone resorption 
          • joint destruction 
          • eventual joint subluxation and dislocation
  • CT scan
    • indications
      • if significant concern osteomyelitis/chronic infection
    • findings
      • helpful in evaluating for intraosseous gas, cortical destruction, and sequestra
  • MRI
    • indications
      • MRI of cervical spine to rule out syrinx when neuropathic shoulder arthropathy is present  
    • findings
      • syrinx has signal intensity equal to or higher than CSF on T1-weighted images 
  • 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
  • Synovial chondromatosis
  • Soft tissue sarcoma
  • Tumeral calcinosis
  • Winchester syndrome 
  • Gorham's disease
  • Milwaukee shoulder syndrome
Treatment
  • Nonoperative
    • rest, NSAIDs, protected immobilization with a sling, restriction of activity and treatment of underlying disease
      • indications
        • first line treatment for neuropathic shoulder joint
      • outcomes
        • 50% of patients reported improvement after non-operative management
    • intra-articular corticosteroid injection
      • indications
        • severe shoulder pain 
      • outcomes
        • some case reports have shown temporary 80% reduction in pain following glenohumeral CSI
  • 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 regrowth of glenoid fossa following syrinx decompression
    • shoulder arthrodesis
      • indications
        • severe charcot shoulder pain having failed conservative management
      • outcomes
        • previously was only operative management offered for charcot shoulder
    • shoulder arthroplasty 
      • indications
        • neuropathy arthropathy is listed as STRICT contraindication for majority of FDA-approved shoulder arthroplasties due to concerns of prosthetic loosening
        • arthroplasty for this condition should be physician-directed application or off-label use
        • newer literature states that arthroplasty is a viable option for patients with charcot shoulder who have failed conservative management and have had underlying condition treated/managed
      • outcomes
        • 70% patients reported improved function with off-label hemiarthroplasty or reverse TSA combined with physical therapy at 5 year followup 
Techniques
  • Rest, NSAIDs, protected immobilization with a sling, restriction of activity and treatment of underlying disease
    • technique
      • immobilization slows the progression of ligamentous and soft-tissue laxity
      • gentle physical therapy, passive stretching, range-of-motion exercises allow for reduction of pain and swelling
  • Intra-articular corticosteroid injection
    • technique
      • glenohumeral 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
  • Shoulder arthrodesis
    • approach
      • S-shaped skin incision beginning over scapular spine, traversing anteriorly over acromion, and extending down the anterolateral aspect of arm
    • technique
      • fusion position
        • goal is to allow patients to reach their mouths for feeding 
        • think "30°-30°-30°" 
          • 20°-30° of abduction
          • 20°-30° of forward flexion
          • 20°-30° of internal rotation
  • Shoulder arthroplasty
    • technique
      • hemiarthroplasty, anatomic TSA, reverse TSA and shoulder resurfacing have all been previously performed off-label for treatment of charcot shoulder with encouraging results.
    • complications
      • progressive glenoid erosion in hemiarthroplasty cases
      • acromial stress fractures in rTSA

Complications

  • Infection
    • risk factors
      • high risk with surgical intervention without management of underlying condition
  • Upper extremity DVT
    • risk factors
      • any surgical intervention
  • Acromial stress fracture
    • risk factors
      • reverse TSA for treatment of charcot shoulder

 

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Questions (2)

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(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 | Tested Concept

QID: 1145
FIGURES:
1

Diabetes

15%

(321/2213)

2

Syphilis

4%

(81/2213)

3

Alcoholism

14%

(312/2213)

4

Syringomyelia

63%

(1401/2213)

5

Uremia

4%

(86/2213)

L 3 D

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(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 | Tested Concept

QID: 1273
FIGURES:
1

HIV testing

10%

(126/1223)

2

cervical spine MRI

77%

(944/1223)

3

repeat ESR, C-reactive protein, CBC

4%

(52/1223)

4

emergent open reduction and internal fixation

2%

(27/1223)

5

emergent irrigation and drainage

6%

(72/1223)

L 2 C

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Evidences (12)
Topic COMMENTS (7)
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