Home

Loading...

Table of contents

Extrahepatic portal vein obstruction

Background

Overview

Definition
Extrahepatic portal vein obstruction is a vascular liver disorder characterized by variceal dilation, ascites, and portal hypertension.
1
2
Pathophysiology
Extrahepatic portal vein obstruction is caused by portal vein thrombosis and portal cavernoma.
3
4
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).
3
4
Prognosis and risk of recurrence
The 1-year mortality rate for liver transplantation patients with portal vein thrombosis is 15%.
3
4

Guidelines

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). ...
Show more

Screening and diagnosis

Clinical presentation: as per EASL 2016 guidelines, assess for extrahepatic portal vein obstruction in any patient presenting with features of portal hypertension, hypersplenism or abdominal pain, or biliary tract disease.
A
Create free account

More topics in this section

  • Indications for screening

Diagnostic investigations

Diagnostic imaging: as per EASL 2016 guidelines, obtain Doppler ultrasound as first line investigation for the diagnosis of extrahepatic portal vein obstruction. Obtain CT for diagnostic confirmation and extension assessment.
A

More topics in this section

  • Evaluation for liver disease

Medical management

Anticoagulant therapy: as per EASL 2016 guidelines, consider permanent anticoagulation in patients with a strong prothrombotic condition, past history suggesting intestinal ischemia, or recurrent thrombosis on follow-up.
C

More topics in this section

  • Management of prothrombotic conditions

  • Management of portal hypertension