Portal vein obstruction results from thrombosis (blood clot) or narrowing of the portal vein, which brings blood to the liver from the intestines.
Because the portal vein is narrowed or blocked, pressure in the portal vein increases. This increased pressure (called portal hypertension) causes the spleen to enlarge (splenomegaly). It also results in dilated, twisted (varicose) veins in the esophagus (esophageal varices) and often in the stomach (portal hypertensive gastropathy). These veins can bleed profusely. Fluid accumulation in the abdomen (called ascites) is not common but may develop when the blockage of the portal vein is accompanied by liver congestion or damage or when large amounts of fluids are given intravenously to treat major bleeding from ruptured varices in the esophagus or stomach. Portal vein thrombosis that develops in people with cirrhosis causes their condition to deteriorate.
About 25% of adults with cirrhosis have portal vein thrombosis, usually due to sluggish blood flow. Portal vein thrombosis also can be caused by any condition that makes blood more likely to clot. Common causes differ by age group:
Often, several conditions work together to cause the blockage. The cause is unknown in about one third of people.
Most people do not have any symptoms. In some people, problems gradually develop, resulting from portal hypertension. If varicose veins develop in the esophagus or stomach, they may rupture and bleed, sometimes profusely. People then vomit blood. The blood may also pass through the digestive tract, making stools black, tarry, and foul-smelling (called melena). Another vascular complication of portal hypertension is the development of abnormal small veins and capillaries in the stomach (portal hypertensive gastropathy), which may result in gastrointestinal bleeding.
Doctors suspect portal vein thrombosis in people who have some combination of the following:
Blood tests to evaluate the liver often are normal.
Doppler ultrasonography usually confirms the diagnosis. It shows that blood flow through the portal vein is reduced or absent. In some people, magnetic resonance imaging (MRI) or computed tomography (CT) is necessary.
Angiography is done if a procedure to create an alternate route for blood flow is planned. For angiography, x-rays of the veins are taken after a radiopaque dye (which is visible on x-rays) is injected into the portal vein.
If a blood clot suddenly blocks the vein, a drug that dissolves clots (such as tissue plasminogen activator) is sometimes used. The effectiveness of this treatment (called thrombolysis) is unclear.
If the disorder develops gradually, an anticoagulant, such as heparin, is sometimes used long term to help prevent clots from recurring or enlarging. Anticoagulants do not dissolve existing clots.
In newborns and children, the cause (usually an infected umbilical cord or acute appendicitis) is treated.
Problems caused by portal hypertension are also treated. Bleeding from varicose veins in the esophagus can be stopped using several techniques (see see Treatment):
Occasionally, when these treatments are ineffective, a procedure to create an alternate route for blood flow, bypassing the liver, may be done. Here, the intent is to reduce pressure in the portal vein by creating a connection (shunt) to the inferior vena cava. Creating a shunt when the portal vein is blocked is difficult. Also, shunts tend to become blocked.
For some people, liver transplantation is necessary.
Last full review/revision December 2007 by Eldon A. Shaffer, MD