HAEMOPHILIA IN ORTHOPAEDICSPRESENTER: DR. LEMAYIANDISCUSSANT: 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

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

Reason for refferal

Surgical debridement done twice at the hospital resulted in excessive bleeding

FURTHER MANAGEMENT

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

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

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

EXAMINATION UPON ADMISSION FGC , wasted, Mildly pale BP-109/67mmHg, PR-60/minMSS:Fused right knee in fixed extension, valgus

deformityWasted LL musculature bilaterallyNecrotic wound on the dorsal aspect of right

footL knee also deformed in fixed flexionNo signs of any soft tissue swellingsSensation was intactOTHER SYSTEMS: Essentially normal

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

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

SUBSEQUENT TESTS

p24-Reactive CD4+-205 cells/microlitre Viral load-? Substitution TestsNormal plasma+ pt’s plasma(1:1)—39.5sFVIII def plasma+ pt’s plasma(1:1)—75.1sFIX def plasma + pt’s plasma(1:1)—50.4sDEFINITIVE DX: FVIII def (Haemophilia A)

Progressively:

Sepsis worsened despite several surgical debridements

Metartarsals became exposed Nutritionist involvement—nutritional

supplementation Haematologist review Initial Decision-midfoot amputation

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

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

Transfused 2 units pre-op, 1 unit intra-op and 1 unit post-op

BKA done on 13/8/12 Post-op medication:i. i.v. tramadol 50mg BDii. i.v ceftriaxone 1g BD Discharged through both orthopaedic

and haematology clinics