PORTAL HYPERTENSION

CLINICAL FEATURES
• Patients with extrahepatic portal venous obstruction (EHPVO) presents with
□ upper GI bleeding
□ sleenomegaly (10-15%)
• Hematemesis and melena – esophageal or gastric variceal bleeding.
• Patients with cirrhosis – jaundice, ascites, hepatosplenomegaly and less often GI bleeding.
• Budd-Chiari syndrome – Ascites and hepatomegaly. Tortuous prominent back vein with inf.
Vena cava block.
 INVESTIGATIONS

• USG and doppler

• Endoscopy
• Colonoscopy
• Selective CT and MRI
• Liver function test
USG and doppler – Block in the vein.
Size of portal vein.
Size of liver and spleen.
Collaterals and ascites.
• Endoscopy - Varices in esophagus or stomach.
• Colonoscopy - lower GI bleeding and presence of rectal varices
• Selective CT or MRI portovenography for delineation of vascular anatomy.
• Liver function test - deranged in patients with cirrhosis.
○ Hemogram – Anemia, leukopenia, thrombocytopenia -"hypersplenism“
 COMPLICATIONS

• Most common – GI bleeding secondary to esophageal varices.

• Hyperslenism, enlarged spleen infarcts and accidental rupture with trauma.
• Others – Ascites, hepatic encephalopathy in children with cirrhosis.
• Hepatopulmonary syndrome – chronic liver disease or portal hypertension
impaired oxygenation, intrapulmonary vascular dilatation.
Symptoms – dyspnea, platypnea, orthodeoxia.
Signs – Clubbing, cyanosis.
Investigation – Contrast echocardiography for intrapulmonary shunting.
• Portopulmonary syndrome – Pulmonary arterial hypertension with severe
portal hypertension.
Symptoms – dyspnea, syncope.
Investigations – echocardiography
Management : GOALS – i) Management of complications.
ii) Treatment of underlying cause.

• Extrahepatic portal venous obstruction – commonest cause of portal HTN in

children in India.
▪ Characterized by obstruction of main portal vein (with or without
mesenteric vein or splenic vein) which gets replaced by cavernoma that shunts
blood across the obstruction along with portosystemic collaterals.
 ▪ Etiology – Umbilical sepsis, pyelophlebhitis, procoagulant disorders.
▪ Presentation – Splenomegaly, hematemesis (variceal bleed).
▪ Complications – poor growth, hypersplenism, portal hypertensive
gastropathy/ colopathy/ enteropathy, rectal varices, ectopic varices, portal biliopathy
And minimal hepatic encephalopathy.
▪ Meso Rex bypass can restore portal flow and alleviate all complications.

• Budd-Chiari Syndrome – Occlusion of hepatic veins and/or suprahepatic inferior

vena cava.
Primary – obstruction of hepatic venous outflow endoluminal venous lesion.
Secondary – obstruction originates from lesion outside the venous system.
- Majority of the patients are primary with chronic course.
- Acute disorder presentation – Abdominal pain, ascites, hepatomegaly, rapid progressive
hepatic failure.
- Chronic form presentation – hepatomegaly, Abdominal distension, portal hypertension.

- Investigation – Doppler ultrasound and venography confirm the diagnosis.

Presence of hypercoagulable state.
- Treatment – Restoring the patency of hepatic vein/inf. Vena cava by radiological means
or surgery (mesoatrial shunt, mesocaval shunt).
Orthotopic liver transplant is reserved for patients with end stage liver
disease or fulminant failure.
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