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 login to view 2 more bullets hemophilia B - Christmas disease login to view 2 more bullets von Willebrand's disease login to view 4 more bullets pathoanatomy synovitis -> cartilage destruction (enzyme based) -> joint deformity Associated conditions orthopaedic manifestations hemarthrosis intramuscular hematoma (pseudotumor) login to view 2 more bullets leg length discrepancy login to view 1 more bullet fractures login to view 2 more bullets medical conditions and comorbidities HIV login to view 1 more bullet 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 login to view 1 more bullet subacute login to view 1 more bullet chronic login to view 1 more bullet paresthesias in the L4 distribution login to view 1 more bullet 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 login to view 1 more bullet modalities login to view 3 more bullets factor administration indications login to view 8 more bullets modalities login to view 2 more bullets desmopressin indications login to view 1 more bullet Operative synovectomy indications login to view 1 more bullet techniques login to view 1 more bullet outcomes login to view 2 more bullets synoviorthesis indications login to view 1 more bullet technique login to view 2 more bullets total joint arthroplasties indications login to view 1 more bullet perioperative care login to view 1 more bullet arthrodesis indications login to view 1 more bullet perioperative care login to view 1 more bullet Prognosis Prognostic variables degree of factor deficiency determines severity of disease login to view 3 more bullets 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 login to view 1 more bullet