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Hemophilia 286

 

synonyms:

Hemophilia ICD-9

  • 286 Coagulation defects
  • 286.0 Factor VIII; antihemophilic globulin (AHG)
  • 286.1 Factor IX; Christmas disease
  • 286.2 factor XI; hemophilia C
  • 286.3 congenital defiency of clotting factors
  • 286.4 von Willebrand's disease

Hemophilia Etiology / Epidemiology / Natural History

  • Hemophilia A and B are the congenital deficiencies of coagulation Factors VIII and IX
  • Hemophilia A = Factors VIII
  • Hemophilia B = Factors IX
  • Recurrent hemarthroses leads to synovitis, cartilage destruction, and arthritis / hemophilic arthropathy.

Hemophilia Anatomy

  • See associated joint.

Hemophilia Clinical Evaluation

  • Knee is most common location for spontaneous bleeding in children followed by the elbows, ankles, hips, and shoulders.
  • Hemarthroses are heralded by a prodrome of joint pain and warmth, followed by swelling/effusion.

Hemophilia Xray / Diagnositc Tests

  • A/P and lateral views of the affected joints.

Hemophilia Classification / Treatment

  • Severe hemophiliacs (factor level b1%) may have spontaneous hemarthrosis
  • Moderate (factor level 1%–5%) typically only bleed after trauma.
  • Mild hemophilia (factor level N5%) typically only bleed after trauma.
  • Generally treated with factor VIII administration until patient reaches 40-50% of normal.
  • Plasma level rises 2% for every unit (per kg body weight) of factor VIII administered
  • Pre-op levels should be 100% of normal and maintained at 100% of normal for 5 days post-op
  • Even patients with severe hemophilia can often avoid orthopaedic sequela with prophylactic administration of factor VIII
  • Synovectomy is indicated for patients with recurrent hemarthrosis dispite optimal medical management. Synovectomy decreases joint bleeding, but may not improve ROM or prevent hemophilic arthropathy and does not reverese articular damage. (Dunn AL, JPO 2004;24:414).
  • Historically hemophiliacs were advised to avoid sports. 
  • With factor prophylaxis sporting activites may be possible
  • DVT prophylaxis:  Patients with known bleeding disorders such as hemophilia and active liver disease generally treated with a pneumatic compression device alone to minimize risk for excessive bleeding and wound complications.

Hemophilia Associated Injuries / Differential Diagnosis

Hemophilia Complications

  • Persistent bleeding
  • Subcutaneous hematoma
  • Stiffness
  • Infection

Hemophilia Follow-up Care

  • Factor levels should be maintained at 50% of normal during any post-operative physical therapy program.

Hemophilia Review References

  • Buzzard BM. Sports and hemophilia: antagonist or protagonist. Clin Orthop 1996;328:25–30.
  • Arnold WD, Hilgartner MW. Hemophilic arthropathy. Current concepts of pathogenesis and management. J Bone Joint Surg Am 1977;59(3):287– 305.
  • Rodriguez-Merchan EC, et al. Hemophilic synovitis of the knee and the elbow. Clin Orthop 1997;343:47–53.
  • Wiedel JD. Arthroscopic synovectomy: state of the art. Haemophilia 2002;8(3):372 –4.
  • Wiedel JD. Arthroscopic synovectomy of the knee in hemophilia: 10-to-15 year followup. Clin Orthop 1996;328:46– 53.
  • Luck Jr JV, Kasper CK. Surgical management of advanced hemophilic arthropathy. An overview of 20 years’ experience. Clin Orthop 1989;242:60– 82.
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