Guideline 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). 1 2 3 3 3 Definition Extrahepatic portal vein obstruction is a vascular liver disorder characterized by variceal dilation, ascites, and portal hypertension. 2 Epidemiology Extrahepatic portal vein obstruction is caused by portal vein thrombosis and portal cavernoma. 3 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 Prognosis and risk of recurrence https://web.pathway.md/diseases/recp0fBHlBM0I2wwd 1/3 6/29/23, 4:21 AM Extrahepatic portal vein obstruction Pathway Prognosis and risk of recurrence The 1-year mortality rate for liver transplantation patients with portal vein thrombosis is 15%. 3 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. A Indications for screening: consider screening for extrahepatic portal vein obstruction in patients with myeloproliferative disease and in patients with antiphospholipid syndrome. C 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. A Evaluation for liver disease: exclude underlying cirrhosis or obliterative portal venopathy in patients with abnormal liver tests, dysmorphic appearance of the liver on imaging, or abnormal liver elastometry results. A 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. C Management of prothrombotic conditions: manage any underlying prothrombotic condition(s) according to established guidelines. B Management of portal hypertension: treat portal hypertension according to established guidelines for cirrhosis. B Clinical findings Symptoms Past medical history Abdominal pain Antiphospholipid syndrome Diarrhea Gastroesophageal varices Fever Hyperhomocysteinemia Liver cirrhosis Medication history Myeloproliferative disease Hormonal contraceptives Past obstetric history https://web.pathway.md/diseases/recp0fBHlBM0I2wwd 2/3 6/29/23, 4:21 AM Extrahepatic portal vein obstruction Pathway Pregnancy Abdominal exam Rectal exam Abdominal distension Ascites Rectal blood Hepatomegaly Splenomegaly Hematological findings Lab findings Pancytopenia Lactic acidosis liver enzymes Imaging findings Intestinal congestion References 1. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016 Jan;64 1 179 202. Open 2. Zeeshan A. Wani, Riyaz A. Bhat, Ajeet S. Bhadoria et al. Extrahepatic Portal Vein Obstruction and Portal Vein Thrombosis in Special Situations: Need for a New Classification. Saudi J Gastroenterol. 2015 May-Jun; 21 3 129 138. Open 3. A Kumar, P Sharma, A Arora. Review article: portal vein obstruction epidemiology, pathogenesis, natural history, prognosis and treatment. Aliment Pharmacol Ther. 2015 Feb;41 3 276 92. Open 4. Italian Association for the Study of the Liver AISF , Italian Association for the Study of the Liver AISF. AISF position paper on liver disease and pregnancy. Dig Liver Dis. 2016 Feb;48 2 120 37. Open https://web.pathway.md/diseases/recp0fBHlBM0I2wwd 3/3