- classic disease results from deficiency of factor VIII clotting activity;
- hemophilia A is not caused by an absolute deficiency of factor VIII, but from an abnormal molecule making up part of the VIII clotting factor;
- hemophilic arthropathy: pathologic changes (coag pathway / coag labs);
- inheritance pattern:
- sex-linked recessive;
- affected male will have normal sons but daughters who are carriers;
- female carriers may have abnormal coag studies or even mild bleeding diathesis, only affected males demonstrate bleeding problems;
- Von Willebrand's disease:
- autosomal dominant transmission, long bleeding time, decreased factor VIII antigen level, & decrease in ristocetin induced platelet aggregation;
- infrequently causes musculoskeletal problems, may also exhibit low levels of factor VIII activity;
- Hemophilia B: (Christmas disease)
- dx is confirmed by abnormal PTT & low factor IX activity;
- results from a deficiency of clotting factor IX;
- although not as common as hemophilia A, factor IX deficiency is similar in that both diseases have sex-linked recessive inheritance, exhibit spontaneous muscle or joint hemorrhages, and commonly produce crippling arthritides;
- clinical stages
- patients may present with a recurrent knee hemarthrosis;
- diagnosis of hemophilia A is confirmed by an abnormal PTT, a normal bleeding time, and, most importantly, by a low level of factor VIII activity;
- extraarticular lesions
Management of Specific Joints:
- arm and forearm:
- w/ moderate destructive changes of hemophilic arthropathy, including marked hypertrophy of the radial head;
- radial head resection w/ synovectomy: (see radial head excision)
- indicated even in presence of advanced disease;
- will maintain joint function and will reduce frequency of hemarthrosis;
- hip joint:
- there is a high rate of loosening of cemented femoral components (35% over 8 yrs);
- higher rate of infection when there is concomitant HIV infection (11%);
- knee joint:
- synovectomy is indicated when recurrent hemarthrosis continues despite optimal medical management;
- Synovectomy of the knee in young patients with hemophilia.
- Arthroscopy of the knee in hemophilia.
- Synovectomy of the knee for hemophilic arthropathy.
- Synovectomy and continuous passive motion (CPM) in hemophiliac patients.
- Open and arthroscopic synovectomy in hemophilic arthropathy of the knee.
- Arthroscopic synovectomy for chronic hemophilic synovitis of the knee.
- Long term follow-up of arthroscopic synovectomy for chronic hemophilic synovitis.
- Synovectomy of the knee in hemophilia.
- total knee arthroplasty:
- ankle joint:
- Arthropathy of the ankle in hemophilia
- Synovectomy of the Ankle for Hemophilic Arthropathy.
- HIV infection was common in the past common due to tainted factor VIII;
- Antibodies to factor VIII;
- develops in approximately 15% of classical hemophiliacs;
- these pts have inhibitors to usual replacement therapy & therefore elective surgery is contraindicated
- Surgical management of advanced hemophilic arthropathy. An overview of 20 years' experience.
- Radiocolloids in the management of hemophilic arthropathy in children and adolescents.
- On the natural history of hemophilic pseudotumor.
- Surgical management of musculoskeletal involvement in hemophilic patients.
- Intraarticular bleeding in children with hemophilia: the prevention of arthropathy.
- Synovectomy in hemophilic arthropathy. A retrospective review of 17 cases.
- Management of fractures in hemophilia.
- Fractures and dislocations in hemophilia.
- Synoviorthesis with Colloidal Phosphorus-32 Chromic Phosphate for the Treatment of Hemophilic Arthropathy.
- Orthopaedic problems in hemophilia.
Original Text by Clifford R. Wheeless, III, MD.
Last updated by Data Trace Staff on Wednesday, November 1, 2017 12:57 pm