Presentation is loading. Please wait.

Presentation is loading. Please wait.

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

Similar presentations


Presentation on theme: "HAEMOPHILIA IN ORTHOPAEDICS PRESENTER: DR. LEMAYIAN DISCUSSANT: DR.KARANU."— Presentation transcript:

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  1 st 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

12

13

14

15

16

17

18 SUBSEQUENT TESTS  p24-Reactive  CD 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)

19

20

21

22

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

24

25

26

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- 44.3%; 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. i.v. tramadol 50mg BD ii. i.v ceftriaxone 1g BD  Discharged through both orthopaedic and haematology clinics

30


Download ppt "HAEMOPHILIA IN ORTHOPAEDICS PRESENTER: DR. LEMAYIAN DISCUSSANT: DR.KARANU."

Similar presentations


Ads by Google