| Introduction |
 A bleeding disorder that leads to repeat hemarthrosis due to minor trauma, eventually leading to
- synovitis
- cartilage destruction
- joint deformity
- Forms include
- hemophillia A
- von Willebrand's disease
- abnormal factor VIII with platelet dysfunction
- hemophillia B - Christmas disease
- Genetics
- X-linked recessive disorder
- Disease severity determined by degress of factor deficiency
- mild: 5-25%
- moderate: 1-5%
- severe: 0-1%
- Joint involvment in descreasing order
- knee (most common)
- elbow
- ankle
- shoulder
- spine
- Immunoglobulin G antibody inhibitors
- a IgG antibody to clotting factor proteins which inhibits response of therapeutic factor replacement
- present in 4-20% of hemophillia patients and can develop at any time
- presence is a relative contraindication for surgery
- HIV prevalence up to 90% in hemophiliacs
|
| Presentation |
- Orthopaedic manifestations
- hemarthrosis
- commonly affects knee
- presents with painful swollen joint with decreased ROM
- aspirate joint to rule out concomitant infection
- intramuscular hematoma (pseudotumor)
- may lead to nerve compression
- iliacus hematoma may compress femoral nerve and presents with paresthesias in the L4 distribution
 
- leg length discrepancy
- due to epiphyseal overgrowth
- fractures
- due to generalized osteopenia
- will heal in normal time
|
| Imaging |
- Radiographs
- knee
- squaring of patella and femoral condyles (Jordan's sign)
- balooning of distal femur
- widening of intercondylar notch
- joint space narrowing
- patella appear long and thin on lateral
- epiphyseal overgrowth
- generalized osteopenia
- fractures
- Ultrasound
- often helpful to follow intramuscular hematomas
|
| Treatment |
- Nonoperative
- home transfusion therapy
- has reduced the severity of arthropathies
- desmopressin
- can be used to treat mild or moderate hemophillia A
- factor administration
- administer factor VIII in the following situations
- vigorous physical therapy
- increase factor VIII to 20%
- acute 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
- Operative treatment
- may be indicated for sequelae of bleeding and includes
- contracture release
- synovectomy
- has been shown to reduce incidence of recurrence
- synoviorthesis
- destruction of synovial tissue with intra-articular injection fo radioactive agent
- osteotomies
- total joint arthroplasties
- for end stage arthropathy
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