Hemophilic arthropathy

1. HEMOPHILIC ARTHROPATHY
DR AKHIL JOSE

2. • systemic arthropathy
• most commonly caused by hemophilia
• characterized by repetitive hemarthroses and
progressive joint disease.
• Diagnosis - recurrent atraumatic hemarthroses in a
patient with a bleeding disorder such as hemophilia A,
hemophilia B or von Willibrand's disease.
• Treatment -compressive dressings, analgesics, and
bleeding factor administration.
• Joint arthroplasty is indicated in cases of severe end-
stage degenerative joint disease.

3. EPIDEMIOLOGY
• young males
• affects patients between 3-15 years old
• knee is most commonly affected
• elbow, ankle, shoulder and spine are also
involved

4. Pathophysiology
Root bleeding disorder may be
A)hemophilia A
• X-linked recessive
• decrease factor VIII
B)hemophilia B - Christmas disease
• X-linked recessive
• decreased factor IX
C)von Willebrand's disease
• rare cause of joint bleeds
• more commonly mucosal bleeding
• autosomal dominant
• abnormal factor VIII with platelet dysfunction

11. PATHOANATOMY
• persistent minor trauma
• synovitis -> enzyme based cartilage
destruction -> joint deformity

14. ASS CONDITIONS
• hemarthrosis
• intramuscular hematoma (pseudotumor)
• nerve compression(femoral nerve palsy -iliacus
hematomas)
• leg length discrepancy(epiphyseal overgrowth)
• Fractures( generalized osteopenia)
• medical conditions and comorbidities-HIV( 90% in
hemophiliacs)

15. 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

18. CLINICAL PRESENTATION
• painful range of motion of joints
• hemarthrosis
• M.C –KNEE JOINT
• Acute- painful and tense joint effusion
• Subacute-occurs after two prior bleeds
• Chronic- contractures or arthritis
• Paresthesias- L4 distribution( iliacus
hematoma -femoral nerve)

26. IMAGING
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 AND ELBOW
• joint arthritis
• epiphyseal overgrowth
• generalized osteopenia
• fractures

27. • Plain radiograph
• joint effusion is seen in the setting of hemarthrosis
• periarticular osteoporosis: from hyperemia
• epiphyseal enlargement with associated gracile diaphysis: from hyperemia (appearances can be
similar to juvenile rheumatoid arthritis and paralysis)
• secondary degenerative disease: symmetrical loss of joint cartilage involving all compartments
equally with periarticular erosions and subchondral cysts, osteophytes and sclerosis
• knee 3
– widened intercondylar notch
– squared inferior margin of the patella
– bulbous femoral condyles
– flattened condylar surfaces
– changes can be classified through the Arnold-Hilgartner classification 4
• elbow 2
– enlarged radial head
– widened trochlear notch
• ankle 1
– talar tilt: relative undergrowth of the lateral side of the tibial epiphysis leads to a pronated foot

31. MRI
can be used to identify early
degeneratve joint disease
Ultrasound
often helpful to follow intramuscular
hematomas

32. • Labs
• Screening tests-platelet count,BT,PT,APTT
• screening for factor VIII inhibitors (including IgG
antibodies)
• indicated prior to surgery as presence will negate
effects of factor treatment
• Prefered test-platelet aggregometry
• Histology-hypertrophy and hyperplastic changes
to the synovium

33. • Nuclear medicine
• bone scintigraphy
– sensitive for detecting areas of disease over the entire
skeleton
– follow-up scans can monitor treatment response
• radiosynoviorthesis
– radioisotopes can be injected therapeutically into a
joint to decrease bleeding and synovitis
– rhenium186 is emerging as the preferred isotope over
phosphorus32 and yttrium90 particularly in medium-
sized joints

35. D/D s
• Septic arthritis
• concomitant infection should be ruled out by
physical exam and joint aspiration

36. • Differential diagnosis
• juvenile rheumatoid arthritis (plain
radiograph)
• paralysis
• tenosynovial giant cell tumor (MRI)
• synovial osteochondromatosis (MRI

37. • 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

42. • 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

50. 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

53. • 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

54. 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 preoperativel

60. jra