Updated: 3/25/2019

Hemophilic Arthropathy

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https://upload.orthobullets.com/topic/9051/images/Xray - knee - col_moved.jpg
Introduction
  • A condition characterized by repetitive hemarthroses and ultimately joint deformation in patients with bleeding disorders
  • Epidemiology
    • incidence
      • has decreased significantly due to home factor treatment
    • demographics
      • young males
      • affects patients between 3-15 years old
    • location
      • knee is most commonly affected
        • elbow, ankle, shoulder and spine are also involved
  • Pathophysiology
    • mechanism of injury
      • persistent minor trauma
    • root bleeding disorder may be
      • hemophilia A
        • X-linked recessive 
        • decrease factor VIII
      • hemophilia B - Christmas disease
        • X-linked recessive
        • decreased factor IX
      • von Willebrand's disease
        • rare cause of joint bleeds
          • more commonly mucosal bleeding
        • autosomal dominant
        • abnormal factor VIII with platelet dysfunction
    • pathoanatomy
      • synovitis -> cartilage destruction (enzyme based) -> joint deformity
  • Associated conditions
    • orthopaedic manifestations
      • hemarthrosis
      • intramuscular hematoma (pseudotumor)
        • may lead to nerve compression
          • femoral nerve palsy may be caused by iliacus hematomas
      • leg length discrepancy
        • due to epiphyseal overgrowth
      • fractures
        • due to generalized osteopenia
        • normal healing chronology
    • medical conditions and comorbidities
      • HIV
        • prevalence up to 90% in hemophiliacs
  • Prognosis
    • prognostic variables
      • degree of factor deficiency
        • determines severity of disease
          • mild: 5-25%
          • moderate: 1-5%
          • severe: 0-1%
      • presence of factor VIII inhibitors (including IgG antibodies)
        • IgG antibody inhibits response of therapeutic factor treatment (monocolonal recombinant factor VIII)
        • found in 5-25% of hemophiliac patients 
        • is a relative contraindication for surgical interventions
          • should be screened for preoperatively
Classification
 
 Arnold-Hilgartner Staging
Stage 1  • Shows swelling of the soft tissues

Stage 2  • Shows osteoporotic changes

Stage 3  • Shows development of subchondral cysts
 • Joint is grossly intact

Stage 4  • Shows cartilage loss with narrowing of the joint
Stage 5
 • Demonstrates severe arthritis of affected joint
 
Presentation
  • Symptoms
    • painful range of motion of joints
    • hemarthrosis
      • the knee is most commonly affected
      • acute
        • presentation will show a painful and tense joint effusion
      • subacute
        • occurs after two prior bleeds
      • chronic
        • presentation will demonstrate contractures or arthritis
    • paresthesias
      • in the L4 distribution  
        • caused by iliacus hematoma that compress femoral nerve
Imaging
  • Radiographs
    • knee  
      • squaring of patella and femoral condyles (Jordan's sign)
      • ballooning of distal femur
      • widening of intercondylar notch
      • joint space narrowing
      • patella appear long and thin on lateral
    • ankle
      • joint arthritis
    • elbow
      • joint arthritis 
    • epiphyseal overgrowth
    • generalized osteopenia
    • fractures
  • MRI
    • can be used to identify early degeneratve joint disease 
  • Ultrasound
    • often helpful to follow intramuscular hematomas
Studies
  • Labs
    • screening for factor VIII inhibitors (including IgG antibodies)
      • indicated prior to surgery as presence will negate effects of factor treatment
  • Histology
    • hypertrophy and hyperplastic changes to the synovium
Differential
  • Septic arthritis
    • concomitant infection should be ruled out by physical exam and joint aspiration
Treatment
  • Nonoperative
    • compressive dressings, analgesics, short term immobilization followed by rehabilitation
      • indications
        • joint pain
      • modalities
        • steroids for to help reduce inflammation
        • splints and braces
        • physical therapy to prevent contracture development
    • factor administration
      • indications
        • vigorous physical therapy
          • increase factor VIII to 20%
        • acute hematomas (including intramuscular hematomas)
          • increase factor VIII to 30%
        • acute hemarthrosis and soft tissue surgery 
          • increase factor VIII to 40-50%
        • skeletal surgery
          • increase factor VIII to 100% for first week following surgery then maintain at > 50% for second week following surgery
      • modalities
        • home transfusion therapy
          • has reduced the severity of arthropathies
    • desmopressin
      • indications
        • mild or moderate hemophillia A
  • Operative 
    • synovectomy
      • indications
        • recurrent hemarthroses recalcitrant to medical management
      • techniques
        • increase factor VIII to 40-50%
      • outcomes
        • decreases incidence of recurrent hemarthroses
        • limits pain and swelling
    • synoviorthesis
      • indications
        • chronic hemophiliac synovitis that is recalcitrant to medical management
      • technique
        • destruction of synovial tissue with intra-articular injection of radioactive agent
          • colloidal phosphorus-32 chromic phosphate
    • total joint arthroplasties
      •  indications
        • end stage arthropathy
      • perioperative care
        • increase factor VIII to 100% for first week postoperatively then maintain at > 50% for second week postoperatively
    • arthrodesis
      • indications
        • arthropathy of the ankle 
      • perioperative care
        • increase factor VIII to 100% for first week postoperatively then maintain at > 50% for second week postoperatively
 

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Questions (2)

(SAE13BS.93) A 48-year-old man who is scheduled to undergo total knee replacement has an X-linked clotting disorder that leads to abnormal bleeding and recurrent, spontaneous hemarthrosis. Before undergoing surgery, he should have replacement therapy of Review Topic

QID: 8334
1

protein C and S.

9%

(4/46)

2

vitamin K.

2%

(1/46)

3

von Willebrand factor.

17%

(8/46)

4

factor VIII.

72%

(33/46)

ML 2

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PREFERRED RESPONSE 4
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