Duke Orthopaedics
presents
Wheeless' Textbook of Orthopaedics

Hemophilia



- Discussion:
    - 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;
    - Diff Dx:
           - 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: (Chistmas 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 Findings:
    - 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

- Radiographic Findings in Hemophilia

- Medical Management of Hemophilia

- Management of Specific Joints:
    - arm and forearm:
           - references:
                  - Hemophilic arthropathy of the upper limb.
                  - Hemophilia presenting as compartment syndrome in the arm following venipuncture. A case report and review of the literature.
    - elbow:
           - 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;
           - references:
                  - Synovectomy of the elbow in young hemophilic patients.  
    - 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:
                  - synovectomy is indicated when recurrent hemarthrosis continues despite optimal medical management;
                  - references:
                         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:
                  - references:
                         Total knee arthroplasty for the treatment of chronic hemophilic arthropathy.
                         Total knee arthroplasty in classic hemophilia.
                         Total knee arthroplasty in chronic hemophilic arthropathy.
    - ankle joint:
           - references:
                  - Arthropathy of the ankle in hemophilia
                  - Synovectomy of the Ankle for Hemophilic Arthropathy.

- Complications:
    - 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


References

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, August 22, 2012 11:12 am

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