3.1 Portal vein thrombosis (without cirrhosis)

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Presents as acute abdominal pain or upper gastrointestinal bleeding without underlying cirrhosis.

Venous thrombosis generally occurs due to blood stasis (by local factors) and prothrombotic disorders.

Local risk factors for portal vein thrombosis without cirrhosis are perinatal umbilical infections and pancreatitis. In the latter situation it leads to a thrombosis of the vena lienalis. It can also be provoked by a splenectomy.

The prothrombotic abnormalities can be acquired, among other things as a result of an underlying myeloproliferative disorder (detectable via JAK2 mutations). They can also be caused by congenital deficiencies, such as protein S deficiency, protein C deficiency, factor V Leiden and mutations in the prothrombin gene. An additional risk factor is oral contraception. In 50% of cases there is more than one risk factor. The clinic of an isolated portosplanchnic venous thrombosis is a coincidental finding, in a third of cases abdominal pain (due to venous congestion) and signs of systemic inflammation and esophageal varices bleeding. Because the liver is also supplied by the hepatic artery, no ischemia of the liver occurs.

The diagnosis is made using a Doppler examination of the liver. An old thrombosis gives a cavernous transformation of the portal vein. Rarely, a portal cholangiopathy develops due to compression of the collaterals on the choledochus.

Since the systematic anticoagulation of these patients, the five-year survival rate has increased to over 90%. Mortality can occur due to an intestinal infarction, especially when the mesenteric vein is also thrombosed.

Screening for esophageal varices should be performed.

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