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Hemophilic arthritis
KHETAN
Explanation
• Hemophilic Arthropathy is a systemic arthropathy most
commonly caused by hemophilia, and characterized by
repetitive hemarthroses and progressive joint disease.
• Incidence
• has decreased significantly due to home factor treatment
• Demographics
• young males
• baffects patients between 3-15 years old
• Anatomic location
• knee is most commonly affected
• elbow, ankle, shoulder and spine are also involved
Pathophysiology
• mechanism of injury
• persistent minor trauma
• root bleeding disorder may be
• hemophilia A
• X-linked recessive
• B. decrease factor VIII
• hemophilia B – Christmas disease
• X-linked recessive
• decreased factor IX
• von Willebrand’s disease
• rare cause of joint bleeds
• more commonly mucosal bleeding
• autosomal dominant
• abnormal factor VIII with platelet dysfunction
Classification (Arnold-Hilgartner Staging)
Stage 1
Shows swelling of
the soft tissues
Stage 2
Shows osteoporotic changes
Stage 3
Shows development of subchondral cysts
Joint is grossly intact
Stage 4
Shows cartilage loss with narrowing of the
joint
Stage 5
Demonstrates severe arthritis of affected joint
Symptoms
1. painful range of motion of joints
2. hemarthrosis
3. the knee is most commonly affected
4. acute
5. presentation will show a painful and tense joint effusion
6. subacute
7. occurs after two prior bleeds
8. chronic
9. presentation will demonstrate contractures or arthritis
10. paresthesias
11. in the L4 distribution
12. caused by iliacus hematoma that compress femoral nerve
Prognosis
1. degree of factor deficiency
2. determines severity of disease
3. mild: 5-25%
4. moderate: 1-5%
5. severe: 0-1%
6. presence of factor VIII inhibitors (including IgG antibodies)
7. IgG antibody inhibits response of therapeutic factor treatment
(monocolonal recombinant factor VIII)
8. found in 5-25% of hemophiliac patients
9. is a relative contraindication for surgical interventions
10. should be screened for preoperatively
Diagnosis (x-ray)
• Knee
• Squaring of patella and femoral condyles (Jordan’s sign)
• ballooning of distal femur
• widening of intercondylar notch
• joint space narrowing
• patella appear long and thin on lateral
• ankle
• joint arthritis
• elbow
• joint arthritis
• epiphyseal overgrowth
• generalized osteopenia
• fractures
Other diagnosis method
• MRI
• can be used to identify early degeneratve joint disease
• Ultrasound
• often helpful to follow intramuscular hematomas
• Labs
• screening for factor VIII inhibitors (including IgG antibodies)
• indicated prior to surgery as presence will negate effects of
factor treatment
Management
• Nonoperative
• compressive dressings, analgesics, short term immobilization
followed by rehabilitation
• Modalities
• steroids for to help reduce inflammation
• splints and braces
• physical therapy to prevent contracture development
Operative method
• Operative
• synovectomy
• indications
• recurrent hemarthroses recalcitrant to medical management
• techniques
• increase factor VIII to 40-50%
• outcomes
• decreases incidence of recurrent hemarthroses
• limits pain and swelling
synoviorthesis
• Indications
• chronic hemophiliac synovitis that is recalcitrant to medical
management
• technique
• destruction of synovial tissue with intra-articular injection of
radioactive agent
• colloidal phosphorus-32 chromic phosphate
Total joint arthroplasties
• Indications
• end stage arthropathy
• perioperative care
• increase factor VIII to 100% for first week postoperatively then
maintain at > 50% for second week postoperatively
arthrodesis
• Indications
• arthropathy of the ankle
• perioperative care
• increase factor VIII to 100% for first week postoperatively then
maintain at > 50% for second week postoperatively
Physical examination by PT
• Palpation of joints at rest and during active range of motion to detect crepitus, synovitis,
oedema or temperature.
• Girth measurement to assess oedema/ muscle atrophy.
• Atypical Joint End feel detection via the passive range of motion.
• Manual Muscle Testing to assess muscular strength.
• Muscle Flexibility test.
• Sensation and proprioception.
• Balance and fall assessment.
• Posture and alignment assessment.
• Assessment of functional activities.
• Gait analysis.
• Neuromotor assessment.
• Musculoskeletal Ultrasound.
Physiotherapy role
• Kinesio Tape
• It was shown the Kinesio tape plays a role in haemophilia by
supporting the muscles, allowing joint movement with support,
decreasing pain and decreasing hematoma.
• Transcutaneous Electrical Neurostimulation (TENS)
• It was shown that TENS has an impact on reducing pain for
patients with haemophilia. It is applied as a low intensity
electrical impulse to stimulate peripheral nerves. That inhibits
pain information transmission along nerves.
• Manual therapy such as fascial therapy, passive
mobilization and stretching; manual orthopaedic therapy and
proprioception training are recommended for physical therapy
management for cases with haemophilia,but joint traction as a
manual therapy technique.

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Hemophilic arthritis.pdf

  • 2. Explanation • Hemophilic Arthropathy is a systemic arthropathy most commonly caused by hemophilia, and characterized by repetitive hemarthroses and progressive joint disease. • Incidence • has decreased significantly due to home factor treatment • Demographics • young males • baffects patients between 3-15 years old • Anatomic location • knee is most commonly affected • elbow, ankle, shoulder and spine are also involved
  • 3. Pathophysiology • mechanism of injury • persistent minor trauma • root bleeding disorder may be • hemophilia A • X-linked recessive • B. decrease factor VIII • hemophilia B – Christmas disease • X-linked recessive • decreased factor IX • von Willebrand’s disease • rare cause of joint bleeds • more commonly mucosal bleeding • autosomal dominant • abnormal factor VIII with platelet dysfunction
  • 4. Classification (Arnold-Hilgartner Staging) Stage 1 Shows swelling of the soft tissues
  • 6. Stage 3 Shows development of subchondral cysts Joint is grossly intact
  • 7. Stage 4 Shows cartilage loss with narrowing of the joint
  • 8. Stage 5 Demonstrates severe arthritis of affected joint
  • 9. Symptoms 1. painful range of motion of joints 2. hemarthrosis 3. the knee is most commonly affected 4. acute 5. presentation will show a painful and tense joint effusion 6. subacute 7. occurs after two prior bleeds 8. chronic 9. presentation will demonstrate contractures or arthritis 10. paresthesias 11. in the L4 distribution 12. caused by iliacus hematoma that compress femoral nerve
  • 10. Prognosis 1. degree of factor deficiency 2. determines severity of disease 3. mild: 5-25% 4. moderate: 1-5% 5. severe: 0-1% 6. presence of factor VIII inhibitors (including IgG antibodies) 7. IgG antibody inhibits response of therapeutic factor treatment (monocolonal recombinant factor VIII) 8. found in 5-25% of hemophiliac patients 9. is a relative contraindication for surgical interventions 10. should be screened for preoperatively
  • 11. Diagnosis (x-ray) • Knee • Squaring of patella and femoral condyles (Jordan’s sign) • ballooning of distal femur • widening of intercondylar notch • joint space narrowing • patella appear long and thin on lateral • ankle • joint arthritis • elbow • joint arthritis • epiphyseal overgrowth • generalized osteopenia • fractures
  • 12. Other diagnosis method • MRI • can be used to identify early degeneratve joint disease • Ultrasound • often helpful to follow intramuscular hematomas • Labs • screening for factor VIII inhibitors (including IgG antibodies) • indicated prior to surgery as presence will negate effects of factor treatment
  • 13. Management • Nonoperative • compressive dressings, analgesics, short term immobilization followed by rehabilitation • Modalities • steroids for to help reduce inflammation • splints and braces • physical therapy to prevent contracture development
  • 14. Operative method • Operative • synovectomy • indications • recurrent hemarthroses recalcitrant to medical management • techniques • increase factor VIII to 40-50% • outcomes • decreases incidence of recurrent hemarthroses • limits pain and swelling
  • 15. synoviorthesis • Indications • chronic hemophiliac synovitis that is recalcitrant to medical management • technique • destruction of synovial tissue with intra-articular injection of radioactive agent • colloidal phosphorus-32 chromic phosphate
  • 16. Total joint arthroplasties • Indications • end stage arthropathy • perioperative care • increase factor VIII to 100% for first week postoperatively then maintain at > 50% for second week postoperatively
  • 17. arthrodesis • Indications • arthropathy of the ankle • perioperative care • increase factor VIII to 100% for first week postoperatively then maintain at > 50% for second week postoperatively
  • 18. Physical examination by PT • Palpation of joints at rest and during active range of motion to detect crepitus, synovitis, oedema or temperature. • Girth measurement to assess oedema/ muscle atrophy. • Atypical Joint End feel detection via the passive range of motion. • Manual Muscle Testing to assess muscular strength. • Muscle Flexibility test. • Sensation and proprioception. • Balance and fall assessment. • Posture and alignment assessment. • Assessment of functional activities. • Gait analysis. • Neuromotor assessment. • Musculoskeletal Ultrasound.
  • 19. Physiotherapy role • Kinesio Tape • It was shown the Kinesio tape plays a role in haemophilia by supporting the muscles, allowing joint movement with support, decreasing pain and decreasing hematoma. • Transcutaneous Electrical Neurostimulation (TENS) • It was shown that TENS has an impact on reducing pain for patients with haemophilia. It is applied as a low intensity electrical impulse to stimulate peripheral nerves. That inhibits pain information transmission along nerves.
  • 20. • Manual therapy such as fascial therapy, passive mobilization and stretching; manual orthopaedic therapy and proprioception training are recommended for physical therapy management for cases with haemophilia,but joint traction as a manual therapy technique.