Updated: 6/5/2021

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 shoulder 2.jpg
https://upload.orthobullets.com/topic/9044/images/neuropathic shoulder 3.jpg
https://upload.orthobullets.com/topic/9044/images/syrinx_mri..jpg
https://upload.orthobullets.com/topic/9044/images/superomedial_humerus_flattening..jpg
  •   summary
    • Neuropathic (charcot) shoulder is a chronic and progressive joint disease most commonly caused by syringomyelia leading to the destruction of the shoulder joint and surrounding structures.
    • Diagnosis is made with radiographs of the shoulder 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
      • 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
    • Anatomic location
      • shoulder (this topic)
      • elbow
      • foot & ankle
  • 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
      • 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
  • 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
Flashcards (1)
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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?

QID: 1145
FIGURES:
1

Diabetes

16%

(398/2528)

2

Syphilis

3%

(88/2528)

3

Alcoholism

14%

(354/2528)

4

Syringomyelia

62%

(1573/2528)

5

Uremia

4%

(100/2528)

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:

QID: 1273
FIGURES:
1

HIV testing

11%

(155/1417)

2

cervical spine MRI

77%

(1086/1417)

3

repeat ESR, C-reactive protein, CBC

4%

(59/1417)

4

emergent open reduction and internal fixation

2%

(32/1417)

5

emergent irrigation and drainage

6%

(82/1417)

L 2 C

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Evidence (15)
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