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Portal hypertension in children
1. Portal Hypertension
in children
Dr. Manori Gamage
(MBBS, MD, DCH, MRCPCH)
Senior lecturer in paediatrics
Faculty of Medical Scinces
University of Sri Jayewardenepura
Sri Lanka
2. Liver gets it’s blood supply from Hepatic artery and Portal
vein.
Portal vein is formed by union of Splenic vein and
Superior Mesentric vein which drains blood from all major
intra abdominal organs.
When, vascular resistance and/or
blood flow
Through this portal vein on system portal hypertension
4. Normal portal venous pressure is 7 -10 mmHg.
When portal venous pressure is > 10mmHg
is defined as
Portal Hypertension (PH).
Main pathological consequence of PH is,
formation of collaterals between portal
circulation and systemic circulation.
5.
6. Causes of PH
Is classified into three categories.
Hepatic
Congenital
Hepatic fibrosis
Polycystic liver
disease
Cirrhosis
Pre hepatic
Portal vein
thrombosis
Umbilical vein
catheterization
Omphalitis
Coagulation
defects
In 50% cause
unknown
Post hepatic
Budd - chiari
syndrome
IVC obstruction
RH failure
8. Clinical presentation
Main manifestations
▪ GI bleeding
▪ Splenomegaly
▪ Ascites
Abnormal abdominal venous pattern (Caput medusae)
Growth failure
Encephalopathy when liver is affected.
9. GI bleeding is due to,
▪ ruptured oesophageal varices
▪ secondary portal gastropathy
▪ rectal varices
Age of the first GI bleed is related to the underlying cause of
PH.
Know to get precipitated with
intercurrent respiratory illness
and NSAID usage.
rectal varices
10. Splenomegaly
Common
May get haematological consequences.
▪ Hypersplenism
Ascites
Ascites is usually seen in patients with PH due to cirrhosis.
There is Sodium and fluid retension.
11. Abnormal venous pattern,
develop due to porto-collateral shunting
through subcutaneous veins.
Rarely,
▪ Hepatopulmonary syndrome
▪ Porto pulmonary hypertension
▪ Portal hypertensive enteropathy
Portal hypertensive enteropathy
12. Diagnosis
Aims
▪ Identify the underlying cause.
▪ Assess for complications of PH.
So,
LFT to assess liver
Clotting profile
FBC - to assess hypersplenism
Doppler study - assess portal venous system
USS Abdomen - liver, spleen, Ascites
UGI Endoscopy
CT/MRI - very useful
13. Management
Two steps.
▪ Prevention of variceal bleeding.
▪ Treatment of variceal bleeding.
It’s described in 3 steps.
a. Primary prophylaxis of first bleeding.
b. Management of an acute bleeding.
c. Secondary prophylaxis for secondary bleeding episodes.
14. Primary prophylaxis
Aim is to prevent first variceal bleed.
Non specific β blockers - portal pressure by cardiac
output and by inducing splenic vasoconstriction.
Endoscopic variceal ligation - But this is not routinely
recommended. If patient has poor access to medical care in
case of emergency , this is useful.
15. Acute variceal haemorrhage
Commonest severe complication.
Stabilising the patient
Monitor vitals
Blood transfusion
Nil by mouth
NG Tube - free drainage
Octreotide
Omeprazole
Lactulose
17. Secondary prophylaxis
Children who had one bleeding episode, need to offer
secondary prophylaxis to risk of further bleeding.
Endoscopy -
6 - 12 months.
In younger children sclerotherapy is more feasible than
banding .
18. Porto systemic shunting
Divert portal blood flow to decrease portal venous pressure.
Shunt is formed,
Between Superior Mesentric vein and IVC -
Mesocaval shunt
Between Portal vein and IVC -
Portocaval shunt
Between Splenic vein and Left Renal vein -
Splenorenal shunt