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HAEMOPHILIA IN ORTHOPAEDICS

HAEMOPHILIA IN ORTHOPAEDICS. PRESENTER: DR. LEMAYIAN DISCUSSANT: DR.KARANU. B.O. MALE 35YRS OLD DISTRICT OF ORIGIN: MARSABIT DATE OF ADMISSION: 2/3/2012 DATE OF DISCHARGE: 21/8/2012. Referral from Marsabit District Hospital as a known haemophiliac

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HAEMOPHILIA IN ORTHOPAEDICS

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  1. HAEMOPHILIA IN ORTHOPAEDICS PRESENTER: DR. LEMAYIAN DISCUSSANT: DR.KARANU

  2. B.O. • MALE • 35YRS OLD • DISTRICT OF ORIGIN: MARSABIT • DATE OF ADMISSION: 2/3/2012 • DATE OF DISCHARGE: 21/8/2012

  3. Referral from Marsabit District Hospital as a known haemophiliac • 1/12 h/o septic wound dorsal aspect of right foot • Involved in an RTA 1/12 prior sustaining degloving injury to the dorsal aspect of the right foot

  4. Reason for refferal • Surgical debridement done twice at the hospital resulted in excessive bleeding • FURTHER MANAGEMENT

  5. PMHx/PSHx • Excessive bleeding during circumcision • Recurrent swelling of the knee joints since he was 5yrs old • Occurred with trivial falls and while playing • Small cuts and bruises that would bleed for long • Admitted severally to Marsabit D.H. with DX of BLEEDING DISORDER • Multiple whole blood transfusions • Progressive joint deformation esp RT. Worsened in 1997 resulting in pathological fusion

  6. FSHx • 1st born in a family of 5 siblings(2 other brothers and 2 sisters) • Both brothers are also known haemophiliacs • DX made at KNH in one of the younger brothers in 1990 when he was referred with similar severe bleeding disorder • Patient works as a shop attendant in Marsabit

  7. Maternal Side-9 of his mother’s relatives had died due to excessive bleeding post circumcision

  8. EXAMINATION UPON ADMISSION • FGC , wasted, Mildly pale • BP-109/67mmHg, PR-60/min MSS: Fused right knee in fixed extension, valgus deformity Wasted LL musculature bilaterally Necrotic wound on the dorsal aspect of right foot L knee also deformed in fixed flexion No signs of any soft tissue swellings Sensation was intact OTHER SYSTEMS: Essentially normal

  9. INVX AT ADMISSION • CBC- Hb 9.38g/dl -WBC 5.23; Neutrophil 50% -platelets 294 • Coagulation profile -PT test 19s -PTI 73.7% -INR 1.36

  10. -APTT test >120s -APTT control 30s • U/E/CR-normal • XRAY L KNEE-complete joint fusion -subchondral cysts -disuse osteopaenia

  11. SUBSEQUENT TESTS • p24-Reactive • CD4+-205 cells/microlitre • Viral load-? • Substitution Tests Normal plasma+ pt’s plasma(1:1)—39.5s FVIII def plasma+ pt’s plasma(1:1)—75.1s FIX def plasma + pt’s plasma(1:1)—50.4s DEFINITIVE DX: FVIII def (Haemophilia A)

  12. Progressively: • Sepsis worsened despite several surgical debridements • Metartarsals became exposed • Nutritionist involvement—nutritional supplementation • Haematologist review • Initial Decision-midfoot amputation

  13. In view of worsening and ascending infection (as well as patient preference) decision made to do BKA

  14. Pre-Op blood workup • CBC- Hb 7.38g/dl; WBC 4.47; Neutrophils- 44.3%; Platelets- 320 • U/E/CR-normal(Na+ 131; k+ 3.52; Urea 2.3 mmol/l) • GXM 4 Units of cryoprecipitate

  15. Transfused 2 units pre-op, 1 unit intra-op and 1 unit post-op • BKA done on 13/8/12 • Post-op medication: • i.v. tramadol 50mg BD • i.vceftriaxone 1g BD • Discharged through both orthopaedic and haematology clinics

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