This document discusses portal hypertension, beginning with definitions and anatomy. It then covers the causes of portal hypertension including presinusoidal, sinusoidal, and postsinusoidal etiologies. The main clinical manifestations are gastrointestinal bleeding, splenomegaly, and encephalopathy. Diagnosis involves blood tests, imaging, and endoscopy. Treatment of acute bleeding involves fluid resuscitation, antibiotics, vasoactive drugs, endoscopic therapies, and shunt procedures if bleeding persists.

1. PORTAL HYPERTENSION

2. CONTENTS
• INTRODUCTION
• ANATOMY
• ETIOLOGY
• CLINICAL MANIFESTATION
• DIAGNOSIS
• TREATMENT

3. INTRODUCTION
• The normal portal venous pressure is about 5-10 mmhg.
• Portal hypertension is define as an elevation of portal pressure >10-
12 mmhg or hepatic venous pressure gradiant >4 mmhg.
• Portosystemic collaterals starts developing with porto venous
pressure of 10 mmhg.
• Variceal bleeding occur when porto venous pressure >12 mmhg.

4. ANATOMY
• The portal system includes all vein
that carry blood from the
abdoiminal part of the alimentary
tract, spleen, pancreas and gall
bladder.
• The portal vein is form by
confluence of the splenic and
superior mesentric vein posterior
to head of pancreas.
• Inferior mesenteric vein drain into
splenic vein anywhere along its
length.

5. COLLATERAL CIRCUALTION
• It develops to carry the portal blood into the systemic vein when the
portal circulation is obstructed whether it be within or outside the
liver.
• There are four main groups of collaterals form during intrahepatic
obstruction.
• GROUP 1
• (A) at cardia of stomach:
Left gastric vein Intercostal vien
Posterior gastric vein Diaphragmo-oesophagel vein
Short gastric vein Azygos minor veins

6. • (B) at anus :
superior haemorrhoidal vein inferior and middle haemorrhoidal
vein
• GROUP 2
 paraumbilical veins form collaterals with abdominal wall veins.
• GROUP 3
 Where the abdominal organs are in contact with retroperitoneal
tissue or adherent to the abdominal wall.
• GROUP 4
splenic vein forms collaterals with left renal vein via diaphragmatic,
pancreatic, left adrenals or gastric veins.

7. SITES OF PORTAL SYSTEMIC COLLATERAL CIRRCULATION

8. CLASSIFICATION
 Presinusoidal : extrahepatic
intrahepatic
 Sinusoidal : cirrhosis, vitamin A intoxication, cytotoxic drugs
 postsinusoidal : extrahepatic
intrahepatic

9. extrahepatic portal hypertension
• Portal vein agenesis, atresia, stenosis
• Portal vein thrombosis
• Splenic vein thrombosis
• Increase portal flow
• Arteriovenous fistula

10. Intrahepatic portal hypertension
• Hepato-cellular diseases
• Acute and chronic viral hepatitis
• Cirrhosis
• Congenital hepatic fibrosis
• Wilson diseases
• Alpha 1 antitrypsin deficiency
• Glycogen storage disease type 4
• Hepatotoxicity
• Methotrexete
• Parenteral nutrition
• Biliary tract disease
• Extrahepatic biliary atresia
• Cystic fibrosis
• Choledochal cyst
• Sclerosing cholangitis
• Intrahepatic bile duct paucity
• Idiopathic portal hypertension
• Post sinusoidal obstruction
• Budd-chiari syndrome
• Venoocclusive disease

12. CLINICAL MANIFESTATION
• Gastrointestinal bleeding is most common presentation of portal
hypertension.
• Bleeding most commonly occurs from varices in the disatl
oesophagus and gastric cardia, Rectal bleeding is less common.
• Varicel haemorrhage may take the fom of hematemesis,
hematochezia, melena or chronic anemia.
• Splenomegaly is second most common finding in children with portal
hypertension.
• Hypersplenism occurs particularly in children with extrahepatic portal
vein thrombosis.

13. • Encephalopathy: it occurs in children with advanced liver disease
with jaundice and low level of liver dependent coagulation factors or
low albumin level.
• Learning disability ,behavioral abnormalities are manifestations of
encephalopathy in children.
• Children may also have accompanying hyperammonemia.
• Bleeding from nongut site due to severe thrombocytopenia in the
form of hematuria ,menorrhagia,epistaxis,hematochazia.
• ASCITIS is the presenting sign of portal hypertension in 7-21 % of
children. It can develop at any time with cirrhosis .

14. COMPLICATION
• Hypersplenism
• Portal hypertensive gastropathy and duodenopathy
• Portal colopathy
• Portal biliopathy
• Ascitis
• Hepatic encephalopathy
• Hepatopulmonary syndrome
• Hepatorenal syndrome
• Portopulmonary hypertension

15. DIAGNOSIS
• Hemogram for hypersplenism
• Liver function test
• Doppler- ultrasound
• UGI endoscopy for varieces
• Specific investigation for underlying etiology
• Viral markers
• Coagulation profile
• CECT
• MRI
• Venography
• Liver biopsy

16. PORTAL PRESSURE MEASUREMENT
• Hepatic venous pressure gradient = wedged hepatic venous pressure
– free hepatic venous pressure
• The normal HVPG is 5-6 mmHg and >10 mmHg represent clinically
significant portal hypertension.
• HVPG is related to survival and also to prognosis in patient with
bleding oesophageal varices
• It may use to monitor thrapy for instance the effect of beta blockers
such as propranolol.

17. Management of acute variceal bleeding
• MEDICAL MANAGEMENT:
• Fluid resuscitation initially in the form of crystalloid infusion followed
by replacement of red blood cells.
• Care should be taken to overtransfusing children as it increase portal
pressure.
• coagulopathy correction by vitamin k or platelet or FFP .
• Nasogastric tube should be placed to document presence of blood
within the stomach and monitor for ongoing bleeding.
• H2 blocker or PPI i.v.
• i.v. antibiotic as there is higher chances of infection.

18. • Pharmacological therapy :
• VASOPRESSIN AND TERLIPRESSIN : Acts by increasing splancnic
vascular tone and thus decrease portal blood flow.
• S/E: it causes vasoconstriction which can impair cardiac function and
perfusion of heart,bowel and kidney.
• SOMATOSTATIN ANALOG OCTREOTIDE : it decreases splancnic blood
flow with fewer side effect.

19. • SURGICAL MANAGEMENT:
• After failure of medical management endoscopy with variceal band
ligation or variceal sclerotherapy is preffered.
• Sclerotherapy treatment may be associated with
bleeding,bacteremia,esophageal ulceration, stricture formation.
• If patient continue to bleed despite of these treatment ,SENGSTAKEN-
BLAKEMORE TUBE may be placed to decrease hemorrhage by
mechanically compressing esophageal and gastric varices.

20. • PORTOCAVAL SHUNT : it diverts nearly all of the portal blood flow into
the subhepatic inferior right vena cava.