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Extrahepatic portal vein obstruction

Definition
Extrahepatic portal vein obstruction is a vascular liver disorder characterized by variceal dilation, ascites, and portal hypertension.
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Pathophysiology
Extrahepatic portal vein obstruction is caused by portal vein thrombosis and portal cavernoma.
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Disease course
Acute manifestations include abdominal pain, fever, nausea, postprandial fullness, anorexia, general malaise, splenomegaly, and ascites. Progression may lead to variceal bleeding, bowel infarction, perforation, peritonitis, sepsis, metabolic acidosis, renal and respiratory failure, shock, and death due to multiorgan failure. Chronic manifestations range from asymptomatic presentation to complications of portal hypertension such as variceal bleeding, thrombocytopenia, splenomegaly, jaundice. Other symptoms include transient ascites, early satiety, abdominal discomfort, intestinal ischemia, hepatic encephalopathy, and portal biliopathy (jaundice, coagulation disturbances, cholangitis, gall stones, hemobilia, and secondary biliary cirrhosis).
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Prognosis and risk of recurrence
The 1-year mortality rate for liver transplantation patients with portal vein thrombosis is 15%.
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Key sources
The following summarized guidelines for the evaluation and management of extrahepatic portal vein obstruction are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2016).
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Guidelines

1.Screening and diagnosis

Clinical presentation: assess for extrahepatic portal vein obstruction in any patient presenting with features of portal hypertension, hypersplenism or abdominal pain, or biliary tract disease.
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  • Indications for screening

2.Diagnostic investigations

Diagnostic imaging: obtain Doppler ultrasound as first line investigation for the diagnosis of extrahepatic portal vein obstruction. Obtain CT for diagnostic confirmation and extension assessment.
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  • Evaluation for liver disease

3.Medical management

Anticoagulant therapy: consider permanent anticoagulation in patients with a strong prothrombotic condition, past history suggesting intestinal ischemia, or recurrent thrombosis on follow-up.
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  • Management of prothrombotic conditions

  • Management of portal hypertension