description on 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

3. Blood supply of liver

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.

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

7. OmphalitisPolycystic
liver
disease
Cirrhosis

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

16. Management - cont…..
Endoscopy
▪ Variceal ligation
▪ Sclerotherapy
Emergency surgery - shunting procedures

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

19. Portocaval shunt
Mesocaval shunt
Splenorenal shunt

20. Questions ???
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21. Thank you!