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

Diagnosis

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 ...