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For further information, see CMDT Part 16-17: Noncirrhotic Portal Hypertension

Key Features

Essentials of Diagnosis

  • Upper gastrointestinal bleeding from esophageal or gastric varices in patients without liver disease

  • Splenomegaly

  • Portal vein thrombosis complicating cirrhosis

General Considerations

  • Causes include

    • Extrahepatic portal vein obstruction (portal vein thrombosis often with cavernous transformation [portal cavernoma])

    • Splenic vein obstruction (presenting as gastric varices without esophageal varices)

    • Schistosomiasis

    • Nodular regenerative hyperplasia

    • Arterial-portal vein fistula

  • Risk factors for portal vein thrombosis

    • Oral contraceptive use

    • Pregnancy

    • Chronic inflammatory diseases (including pancreatitis)

    • Injury to the portal venous system (including surgery)

    • Hepatocellular carcinoma and other malignancies

    • Treatment of thrombocytopenia with eltrombopag

  • Idiopathic noncirrhotic portal hypertension

    • Common in India

    • Rare in Western countries

    • Has been attributed to

      • Chronic infections

      • Exposure to medications or toxins

      • Prothrombotic disorders

      • Immunologic disorders

      • Genetic disorders that result in obliterative vascular lesions in the liver

  • Portal vein thrombosis

    • May be classified as

      • Type 1: involving the main portal vein

      • Type 2: involving one (2a) or both (2b) branches of the portal vein

      • Type 3: involving the trunk and branches of the portal vein

      • Additional descriptors are occlusive or nonocclusive, acute or chronic, extent (eg, into the mesenteric vein), and nature of any underlying liver disease

    • May occur in 10–25% of patients with cirrhosis

      • Associated with the severity of the liver disease and related in part to acquired protein C deficiency and splenorenal shunts (resulting in stagnant portal venous blood flow)

      • May be associated with hepatocellular carcinoma but not with increased mortality

  • "Obliterative portal venopathy" is used to describe primary occlusion of intrahepatic portal veins in the absence of cirrhosis, inflammation, or hepatic neoplasia

  • Cases of noncirrhotic portal hypertension due to nodular regenerative hyperplasia have been reported in HIV-infected patients treated with didanosine or with a combination of didanosine and stavudine or didanosine and tenofovir

Clinical Findings

Symptoms and Signs

  • Acute portal vein thrombosis usually causes abdominal pain

  • Aside from splenomegaly, physical examination is unremarkable

  • Hepatic decompensation can follow severe gastrointestinal bleeding and intestinal infarction may occur when portal vein thrombosis is associated with mesenteric venous thrombosis

  • Ascites may occur in 25% of persons with noncirrhotic portal hypertension

  • Low-grade hepatic encephalopathy is common in patients with noncirrhotic portal vein thrombosis


Laboratory Tests

  • Complete blood count may reveal thrombocytopenia and other findings of hypersplenism

  • Liver chemistries are usually normal

  • An underlying hypercoagulable state is found in many noncirrhotic patients with portal vein thrombosis in the absence of an obvious provoking factor

  • Malignant vascular invasion is suggested by a serum alpha-fetoprotein level > 1000 ng/dL (10 mcg/L), venous expansion enhancement of the thrombus, neovascularity, and proximity to a primary hepatocellular carcinoma

Imaging Studies

  • Color Doppler ultrasonography is the initial diagnostic test for ...

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