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