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 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 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

18. 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)

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

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

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

29. 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.v ceftriaxone 1g BD
Discharged through both orthopaedic and haematology clinics