This document provides an overview of coagulopathy in children. It begins with objectives and definitions. It then discusses various types of bleeding disorders including quantitative and qualitative platelet disorders, coagulation factor disorders like hemophilia, and acquired disorders like disseminated intravascular coagulation. For each condition, it covers causes, clinical features, laboratory findings, and treatment strategies. It concludes with perioperative considerations and strategies for managing coagulopathy in children undergoing surgery.

1. WELCOME
TO ALL

2. Coagulopathy in
children
Dr. Mohammed Saiful Islam
Resident ( Phase:A),
Department of Paediatric Surgery, BSMMU.
Bangladesh.
20/09/2016, Venue: Paediatric surgery class room. 2

3. Objectives
• To define & understand the extent of
coagulopathy.
• A review of coagulation pathways.
• Discussions on Platelet disorders.
• Key points discussions associated with
coagulation factor disorders.
• Perioperative strategies to meet the
challenges of coagulopathy in case of
children.

4. Definition:
Group of medical
conditions resulting from deficiency
of clotting factors, inhibition of
coagulation process or excessive
activity of the fibrinolytic system.
Reff: de Gruchy 5th

5. Bleeding
disorders
Coagulopathy

6. Extent of Coagulopaty:
• VESSEL WALL ABNORMALITIES:
• PLATELETS DISORDER:
• COAGULATION DISORDER:

7. INTRINSIC EXTRINSIC
PROTHROMBIN(II) THROMBIN(III)
FIBRINOGEN
FIBRIN
(I)
V
X
Tissue ThromboplastinCollagen
XII
XI
IX
VIII
VII
PT
PTT Vit.K,
Liver
CLOTTING MECHANISM (Easy to keep in mind)

8. PLATELETS DISORDERS:
• QUANTITATIVE PLATELETS
DISORDERs.
• QUALITATIVE PLATELET
DYSFUNCTION.

9. QUANTITATIVE PLATELETS DISORDER
(Thrombocytopenia)
Mechanisms:
1. Failure of megakaryocytic maturation.
2. Excessive platelets consumption after
their release into circulation i.e ITP,
DIC etc.
3. Platelets sequestration in enlarged
spleen i.e HYPERSPLEENISM.

10. QUANTITATIVE PLATELETS DISORDER
(Continued…)
S/S:
• Petechial cutaneous bleeding,
• Intracranial bleeding,
• Oozing from mucus membrane & skin
surface.
Lab: Decreased platelets count
and prolong bleeding time.

11. (Thrombocytopenia) Causes:
Marrow Disorder
• Aplastic anemia.
• Haematological
malignancy.
• Myelodysplastic
disorder.
• B12 defficiency.
Non Marrow Disorder
• Immune disorders.
• ITP, Drug induced.
• Sec: ALL, SLE.
• Post transfusion.
• DIC.
• Human immunodeficiency
syndrome.
• Hyperspleenism.
• Haemangiomas.
• Sepsis.
• Viral infection.

12. Thrombocytopenia (Continued…)
• Management:
– Rx Underlying cause.
– Platelet transfusion.

13. IDIOPATHIC THROMBOCYTOPENIC
PURPURA(ITP)
• An autoimmune antibody IgG is
formed against unknown antigen of
platelets membrane/surface.
• Antipletelet antibody binds to
complement, but platelets are not
destroyed by direct lysis.
• Destruction takes place in spleen,
where spleenic macrophages
destroyes antibody coated platelets.

14. IDIOPATHIC THROMBOCYTOPENIC PURPURA. (Continued…)
Clinical Features:
Often precipitated by viral infection and
usually self limiting.
 Asymptomatic, not febrile.
 Present with mucosal/skin bleeding,
mennorrhagia, purpura, petechiae.
 Females are more affected (2:1
male/female ratio).

15. IDIOPATHIC THROMBOCYTOPENIC PURPURA. (Continued…)
Δ LAB:
Platelets below 10,000 /ml.
Bone marrow will appear normal.
Rx
PREDNISONONE: 1-2 mg/kg/day.
Immunoglobulin: 1g/kg/day 2-3 days.
DANAZOLE: 600mg/day response rate is 50%
IMMUNOSUPPERESSIVE DRUGS: i-e vincristine,
vinblastine, azathioprine, cyclosporin,
cyclophosphomide.
SPLEENECTOMY.
Prognosis:
Good, if disease is initially controlled with
prednisolone,
Spleenectomy is definite Rx.

16. QUALITATIVE PLATELET DISORDER
CONGENITAL:
• Glanzmann’s
thrombasthenia.
• Bernard souliar
syndrome.
• Von Willibrand’s
disease.
ACQUIRED
• Myeloproliferative
disorder.
• Uremia.
• Drugs i-e NSAIDS
Aspirin.
• Autoantibody.
• Fibrin degradation
products.

17. QUALITATIVE PLATELET DISORDER
GLANZMANN’s THROMBASTHENIA:
Autosomal recessive disorder.
Lack of receptors (glycoprotein Ib & IIIa)
for fibrinogen on platelets.
Platelets fails to aggregate in respons to
ADP, collagen, thrombin.
Clinical Features: Mucosal bleeding
LAB:
Platelets no’s and morphology are normal
B.T is prolonged
Rx:
Platelet transfusion

18. QUALITATIVE PLATELET DISORDER
BERNARD SOULIER SYNDROME:
Autosomal recessive intrinsic platelets disorder.
Due to lack of glycoprotein (Gp1b), receptor for
vonWillibrand’s factor.
Clinical Features:
Presents with mucosal bleeding and post operative
oozes.
LAB:
Thrombocytopenia may be present, and Platelets
are abnormally large in size.
BT is prolonged
Von Willibrand’s factor Normal .
Rx:
Platelet transfusion

19. QUALITATIVE PLATELET DISORDER
VON-WILLIBRAND’S DISEASE:
• Autosomal dominant (gene for vWF
is located on chromosome 12.)
• vWF is synthesized by endothelial
cells and megakaryocytes.
• It acts as carrier protein for
factor VIII by non-covalent bond.
• A defect therefore leads to
decreased plasma factor VIII level.

20. VON-WILLIBRAND’S DISEASE (Continued….)
• It also forms bridges between
platelets and sub endothelium.
• There fore defect of vWF leads
to prolonged bleeding.

21. VON-WILLIBRAND’S DISEASE (Continued….)
Figure: Structure and function of factor VIII-von Willebrand factor (vWF) complex.

22. VON-WILLIBRAND’S DISEASE (Continued….)
Clinical Features:
• Mucosal bleeding (mild-massive)
LAB:
• Reduced level of vWF.
• Secondary reduction in factor VIII.
• Prolonged bleeding time (B.T).

23. VON-WILLIBRAND’S DISEASE (Continued….)
Rx:
• MILD HAEMORRHAGES:
Desmopressin 0.3 μg/kg, after
which vWF levels usually raise 3 units in
30-90 minutes.
• MASSIVE HAEMORRHAGES:
Factor VIII transfusion.

24. COAGULATION DISORDER:
Coagulation factor disorder
can be either due to single factor
def., i.e. a “congenital deficiency”,
e.g factor VIII resulting in
HAEMOPHILIA-A
or due to multiple factor
defficiency which is an ‘’acquired
condition” e.g Sec: to liver disease
or warfarin therapy.

25. • HEAMOPHILIA – A (CLASSIC / TRUE
HAEMOPHILIA)
• HAEMOPHILLIA – B (CHRISTMAS
DISEASE).
• Other specific factor deficiency (e.g.
Factor І, ІІ, ІІІ etc. deficiency).
COAGULATION DISORDER:
CONGENITAL BLEEDING DISORDER:

26. COAGULATION DISORDER
ACQUIRED BLEEDING DISORDER
• DIC
• Liver disease
• Renal disease
• Vitamin K deficiency
• Massive blood transfusion
• Anticoagulant drugs.

27. HAEMOPHILIA
• X-linked recessive disorder.
• Due to deficiency of factor VIII (Haemophiia
A) or factor IX (Haemophiia B) .

28. HAEAMOPHILIA (Continued….)
C/F:
• Bleeding occurs as bruising at the age of 6
months.
• Trauma results in excessively bleeding.
• Recurrent bleeding /hemorrhage in knee, elbow,
ankle, and hip. (Hemarthrosis).
• Mucus membrane /internal bleeding of mouth,
lips, gums, brain and kidney also occur.
• Muscle haematoma esp. calf and Psoas muscle .
Rx
• Cryoprecipitate or Factor VIII infusion ( in
Haemophiia A) & Factor IX infusion (in
Haemophilia B)

29. HAEAMOPHILIA (Continued….)
LONG TERM COMPLICATIONs
COMPLICATION due to repeated
hemorrhage:
– Arthropathy of large joints eg knee, elbow
– Muscle atrophy due to haematoma
– Mononeuropathy due to pressure of
haematoma.
COMPLICATION due to therapy
– Antifactor VIII antibody develops.
– Virus transmission Hepatitis B,C + HIV etc.

30. HAEAMOPHILIA (Continued….)
Figure: Haemarthrosis & Arthropathy.

31. SURGERY IN A CHILD WITH
HAEMOPHILLIA:
• In minor Surgery:
Factor VIII should be at least 50% of
the normal predicted value.
• In Major Surgery:
 Factor VIII should be 100%.
 Half life of Factor VIII is 8 to 12
hours.
 Factor VIII should be substituted just
before surgery & 12 hours after the
initial dose ( Target is to pass the
time of reactionary haemorrhage).

32. Calculation of Factor VІІІ
% Desired Χ Wt in Kg
• Amount of Factor VІІІ =
K
Here, value of K is 1.5 for Haemophilia A
& 0.75 for Haemophilia B.

33. DISSAMINATED INTRAVASCULAR
COAGULATION
Disseminated Intravascular
Coagulation (DIC) is a thrombo-
haemorrhagic disorder appearing as a
secondary complication of several diseases,
where there is consumption of platelets
and clotting factors in the circulation as
well as secondary activation of plasminogen
fibrinolytic system.

34. DIC:
CAUSES
A Infection:
1. Gram negative
septicaemia
2. Meningococcaemia
3. Pneumococcaemia
4.Malaria
B. Massive injury:
1. Massive trauma
2. Extensive burn
(severe burn)
3. Extensive surgery
(brain, lung, prostate, etc.)
C. Carcinoma:
1. Pancreas
2. Lungs
3. Stomach
4. Prostate, etc.
D. Others:
1. Transfusion
reaction
2. Fat embolism
3. Snake bite
4. Shock, etc.

35. Fig: Pathophysiology of disseminated intravascular coagulation.

36. DIC (Continued….)
CLINICAL FEATURES:
• Bleeding & thrombosis, bleeding is more
than thrombosis.
• Features of complications.
LAB:
• Thrombocytopenia
• Prolong PT
• APTT may be normal/increased
• Low fibrinogen
• Increased level FDP/D-dimmer

37. Treatment of DIC
Rx. Underlying cause.
General Measures: Correction & prevention of :
• Dehydration
• Renal failure
• Acidosis and
• Shock
Replacement:
• Platelets transfusion if platelets counts below
10,000/ml
• Cryoprecipitate to maintain plasma fibrinogen
level above 150 mg/dl
• FFP
• Heparin, if there is DVT, Pulmonary thrombosis.

38. WARCRAFT FOR SURGERY IN
A CHILD HAVING
ANTICOAGULANT THERAPY
• If patient on oral anticoaguant:
Dose adjustment for INR within 2.5 to 3.5.
During surgery INR should be 1.5 or less.
• If patient on Parenteral anticoaguant:
Per operative APTT should be within 1.5 to
2.5 times of normal range.

39. WARCRAFT FOR SURGERY (Continued….)
• During emergency surgery:
 Inj. Vitamin K should be
administered.
FFP & Factor concentrate of ІІ,
VІІ, ІX & X should be kept in hand.
Per operative & Post operative
monitoring of PT, INR.

40. WARCRAFT FOR SURGERY (Continued….)
• During elective surgery:
• Minor surgery:
» Oral anticoagulant should be stopped 2
to 3 days prior to surgery.
• Major surgery:
» Oral anticoagulant should be stopped 4
to 5 days prior to surgery.
» Then low molecular weight heparin
should be started.
» Heparin should be stopped 4 hours prior
to surgery & restarted 12 hours after
surgery.

41. THANK YOU