Updated: 6/17/2021

Hemophilic Arthropathy

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https://upload.orthobullets.com/topic/9051/images/stage1.jpg
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  • summary
    • Hemophilic Arthropathy is a systemic arthropathy most commonly caused by hemophilia, and characterized by repetitive hemarthroses and progressive joint disease.
    • Diagnosis is made with with recurrent atraumatic hemarthroses in a patient with a bleeding disorder such as hemophilia A, hemophilia B or von Willibrand's disease.
    • Treatment is usually compressive dressings, analgesics, and bleeding factor administration. Joint arthroplasty is indicated in cases of severe end-stage degenerative joint disease. 
  • Epidemiology
    • Incidence
      • has decreased significantly due to home factor treatment
    • Demographics
      • young males
      • affects patients between 3-15 years old
    • Anatomic location
      • knee is most commonly affected
        • elbow, ankle, shoulder and spine are also involved
  • Etiology
    • 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
  • 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
  • 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
Flashcards (4)
Cards
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Questions (3)

(OBQ18.7) Which of the following bleeding disorders is caused by an X-linked recessive mutation?

QID: 212903
1

Factor VIII deficiency

22%

(349/1555)

2

Factor IX deficiency

4%

(65/1555)

3

Factor XI deficiency

1%

(15/1555)

4

Von Willibrand factor deficiency

10%

(150/1555)

5

Both 1 & 2

62%

(966/1555)

L 3 A

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(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

QID: 8334
1

protein C and S.

3%

(10/324)

2

vitamin K.

4%

(12/324)

3

von Willebrand factor.

10%

(34/324)

4

factor VIII.

82%

(267/324)

L 2 D

Select Answer to see Preferred Response

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