Surgery - Surgical Bleeding Disorders

Question 1

What is Haemophilia A?

Answer 1

Factor VIII deficiency

• X-linked recessive
• high rate of de novo mutations

Question 2

What is Haemophilia B?

Answer 2

Factor IX deficiency

-X-linked recessive

Question 3

What are the clinical features of Haemophilia?

Answer 3

Major bleeds following minor trauma
Recurrent haemarthroses –> crippling arthropathies
Compartment syndrome/nerve palsies

Question 4

What are the appropriate investigations in suspected haemophilia?

Answer 4

Raised APTT

Low factor VIII/IX

Question 5

What are the management options for haemophilia?

Answer 5

Avoid NSAIDs/IM injections
Minor bleeding
   -compression & elevation
   -desompressin
Major bleeding
   -recombinant factor VIII/IX to raise factor levels (50% of normal)
Life threatening
   -recombinant factor VIII/IX to raise factor levels (100% of normal)

Question 6

What are the common causes of clotting factor deficiency?

Answer 6

Haemophilia
vWD
Liver disease
DIC
Vit K deficiency
Anticoagulant drugs

Question 7

What is Von Willebrand’s Disease?

Answer 7

Absence of vWF OR presence of abnormally functioning vWF

Question 8

What are the sx of vWD?

Answer 8

Epistaxis
Menorrhagia
Haemarthroses rare

Question 9

What is the inheritance of vWD?

Answer 9

Autosomal recessive (20%)
-complete absence of detectable vWF
Autosomal dominant (80%)
-less severe depletion of vWF

Question 10

What are the appropriate investigations in suspected vWD?

Answer 10

APTT (increased)
INR (normal)
Platelets (normal)

Question 11

What are the management options for vWD?

Answer 11

Tranexamic acid (mild bleeds)
Desompressin/recombinant factor VIII (severe bleeds)

Question 12

What questions would identify potential bleeding disorders?

Answer 12

Site of bleed?
Duration of hx/family hx?
Severity?
Surgical hx?
PMH?
Drug hx?

Question 13

What factors about the site of bleed may help identify a bleeding disorder?

Answer 13

Muscle/joint bleeds
   -coagulation issue
Purpura/epistaxis/menorrhagia/GI haemorrhage
   -platelet/vWD
Recurrent bleeds
   -local endothelial abnormality

Question 14

What factors about the surgical hx may help identify a bleeding disorder?

Answer 14

If starts immediately = platelet

If starts after sev hours = coagulation

Question 15

What signs may suggest a bleeding disorder?

Answer 15

Skin (purpura, bruises, telangiectasia)
Joints (haemarthroses)
Abdomen (splenomegaly, hepatic dysfunction)

Question 16

What is DIC?

Answer 16

Systemic activation of coagulation pathway

• extensive intravascular coagulation
• fibrin clot development
• thrombotic occlusion of arterial microvasculature
• depletion of clotting factors/platelet consumption

Question 17

What are the common causes of DIC?

Answer 17

Infection
Trauma
Malignancy
Obstetric complication (amniotic emboli, pre-eclampsia)
Severe liver failure
Tissue destruction (pancreatitis/burns)
Toxic/immunogenic stimuli

Question 18

What are the clinical features of DIC?

Answer 18

Bruising
Excessive bleeding from any site
Renal failure

Question 19

What are the appropriate investigations in suspected DIC?

Answer 19

Low platelets
Low fibrinogen
Raised PT & APTT
Raised D-dimer
Blood film (shistocytes)

Question 20

What are the management options for DIC?

Answer 20

Treat the cause
Aggressive resus
   -replacing platelets
   -coag factors
   -fibrinogen
Protein C (reduces mortality in multi-organ failure/sepsis)