Hemophilic arthritis is a joint disorder due to hemophilia which involves especially large joints like Knee, ankle, and hip.
Hemophilia is a hereditary coagulation disorder characterized by the occurrence of hemorrhages that appear significantly or as a result of insignificant trauma
Etiology
Genetically determined
Affects Factor VII, IX, and XI
Classification
Hemophilia A
Classical hemophilia
80% of cases of hemophilic arthritis
Occurs in males, gene carried on X-chromosome and is transmitted by unaffected females
- VII deficiency
- Antihemophilic factors
- Antihemophilic globulins
Hemophilia B
Chrismas disease
Factor IX ( Plasmin thromboplastin components)
X-chromosome
Hemophilia C
Mild form
Due to deficiency of factor XI, the precursor of thromboplastin
Autosomal dominant inheritance
Arthropathy is not seen in this disorder
von Willebrand’s disease
Genetic disorder
Missing or defective vWF which binds to factor VII and platelets in the blood
Helps in the clotting process
Pathology
- Blood + synovial fluid = Irritant to synovial membrane
- Removal of blood by synovium, hemosiderin is seen intracellularly in the superficial cellular layer
- Macrophages, leukocytes: Disintegrate erythrocytes
- It is a standard resorptive mechanism but later it goes exhausted
- Hemosiderin accumulates predominantly in synovium and cartilages
- Synovium
- Undergoes hyperplasia
- Villous formation
- Hypervascular
- Granulation tissue and fibrosis
- Perivascular cuffing with PMNs, monocytes form PANNUS
- Cartilages
- It interferes with chondrocytes’ mechanism
- Diffusion in cartilages become difficult=> Damage cartilage
- Pannus -> release collagenase => Damage cartilages
- Cartilage damage
- Loses matrix components
- Unable to bear verticle loads/ stress
- microscopically develop clefting and chrondan formation similar to OA vertically
- Losses of surface cartilage, and erosion occurs in an irregular manner
- Characteristic Map like
- Sub -chondral cortex become thinned and worn through
- Produce cyst (due to bone resorption)
- Secondary Articular changes (OA)
During the growth period due to hyperemia, the epiphysis of the affected joint becomes enlarged
Rate of longitudinal deformity may be increased/decreased/ valgus/ varus deformity
- Hemorrhages (Large) accumulate within the muscles/ beneath muscles and their sheath or in the vascular sub-periosteal area where they remain as slowly enlarging Pseudo-tumor
- Hemophilic cysts become circles by the fibrous capsule
- When stripping the periosteum due to sudden expansion
- Pathological fractures and burst from skin=> Fatal Hemorrhage
Clinical Features
History of uncontrolled hemorrhages
Mostly involved in knee
Outline sudden effusion in the synovial cavity
The acute phase lasts for a few days-> the Chronic phase (Boggy synovial tissue in synovitis)
Degenerative arthritis has seen at an early age
Fibrous ankylosis
Muscle atrophied
Final picture: As End stage Rheumatoid arthritis
Bulbous tumor along the shaft of long bone-> Pseudotumor
X-ray findings
| Staging | Radiological features |
|---|---|
| Stage 1 | Soft tissue swelling (Synovial thickening, blood in joint) |
| Stage 2 | Epiphyseal overgrowth, osteoporosis, maintained joint space integrity |
| Stage 3 | Marked epiphyseal growth, early incongruity (beginning cartilage destruction) |
| Stage 4 | Narrowing, cyst formation (advancing cartilaginous destruction); femoral intercondylar notch is widened |
| Stage 5 | Severe loss of joint space associated with fixed fibrous contracture |
In the site of pseudotumor, bulbous opacity in close approximation to the cortex of the adjacent bone
Common location:
- Anterior thigh
- Calf
- The inner aspect of the ilium
- Volar aspect of the forearm
Treatment of Hemophilic arthritis
Involvement of Hematological and Orthopaedic specialist
Medical management
- Replacement of missing factors
- Lyophilized cryoprecipitate
- Replacement therapy with factor VIII, factor IX
- Maintainance of plasma level is essential
Orthopedics nonsurgical management
- Joint immobilized with a posterior splint, pillow over the skin, cast
- Extremities elevated
- AVOID ASPIRATION: (Decrease IA counter pressure and increase the chance of infection)
- If aspiration is done, plasma level should be elevated by 30%-40%
When replacement therapy fails to control hemorrhages
- A small dose of corticosteroid can be given
- Later, Knee deformity, muscle atrophy, flexion contracture, instability
- So, prolonged immobilization is discouraged, joint motion encouraged, Isometric exercises and knee ROM exercises
- Non-weight bearing in the lower limb is mandatory with crutches
- I.A. Corticosteroid is contraindicated
- If chronic synovitis and hemarthrosis are under control light daily activities (swimming, hiking ) can be initiated
Knee Flexion contracture
- Develops prior to the development of chronic hemophilic arthropathy
- The deformity can be overcome by gentle traction and by a turnbuckle type of cast
- X-ray has been taken everyday -> to check posterior subluxation of the tibia
Orthopedics surgical management
- A pneumatic tourniquet is used
- Vessels are ligated rather than electrocautery
- Suction tube kept for 24 hours
- Avoid drugs that alter the clotting mechanism
Hemophilic pseudotumor
- May compress vital structures
- If mass appears static-> period of watchful waiting is permissible to determine whether mass will be received or be replaced by fibrosis
- Preliminary replacement therapy and adequate provision of blood transfusion
- Followed by excision
- Human anti-hemophilic globin: Apply as a paste locally
- Radiation therapy for small inaccessible tumors
Synovectomy
Indicated in repeated uncontrollable hemarthrosis with chronic synovitis
Fractures
Osteoporotic fractures=> Should not be delayed
Supracondylar Osteotomy for severe flexion deformity of the knee
TKA for advanced arthropathy
Arthrodesis: Severely destroyed joints
Achilles tendon lengthening: In contracture of tricep surae and the equinus deformity of the foot after bleeding
See also: Osteoarthritis of Knee
See also: Management of Rheumatoid arthritis
