Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ Key Features ++ Essentials of Diagnosis ++ Upper gastrointestinal bleeding from esophageal or gastric varices in patients without liver disease Splenomegaly Portal vein thrombosis complicating cirrhosis ++ General Considerations ++ Causes include Extrahepatic portal vein obstruction (portal vein thrombosis often with cavernous transformation [portal cavernoma]) Splenic vein obstruction (presenting as gastric varices without esophageal varices) Schistosomiasis Nodular regenerative hyperplasia Arterial-portal vein fistula Risk factors Oral contraceptive use Pregnancy Chronic inflammatory diseases (including pancreatitis) Injury to the portal venous system (including surgery) Hepatocellular carcinoma and other malignancies Treatment of thrombocytopenia with eltrombopag Idiopathic noncirrhotic portal hypertension Common in India Rare in Western countries Has been attributed to Chronic infections Exposure to medications or toxins Prothrombotic disorders Immunologic disorders Genetic disorders that result in obliterative vascular lesions in the liver Portal vein thrombosis May be classified as Type 1: involving the main portal vein Type 2: involving one (2a) or both (2b) branches of the portal vein Type 3: involving the trunk and branches of the portal vein Additional descriptors are occlusive or nonocculsive, acute or chronic, the extent (eg, into the mesenteric vein), and the nature of any underlying liver disease May occur in 10–25% of patients with cirrhosis Associated with the severity of the liver disease May be associated with hepatocellular carcinoma but not with increased mortality "Obliterative portal venopathy" is used to describe primary occlusion of intrahepatic portal veins in the absence of cirrhosis, inflammation, or hepatic neoplasia Cases of noncirrhotic portal hypertension due to nodular regenerative hyperplasia have been reported in HIV-infected patients treated with didanosine or with a combination of didanosine and stavudine or didanosine and tenofovir ++ Clinical Findings ++ Symptoms and Signs ++ Acute portal vein thrombosis usually causes abdominal pain Aside from splenomegaly, physical examination is unremarkable Hepatic decompensation can follow severe gastrointestinal bleeding or a concurrent hepatic disorder, and intestinal infarction may occur when portal vein thrombosis is associated with mesenteric venous thrombosis Ascites may occur in 25% of persons with noncirrhotic portal hypertension Low-grade hepatic encephalopathy is common in patients with noncirrhotic portal vein thrombosis ++ Diagnosis ++ Laboratory Tests ++ Complete blood count may reveal thrombocytopenia and other findings of hypersplenism Liver chemistries are usually normal An underlying hypercoagulable state is found in many patients with portal vein thrombosis ++ Imaging Studies ++ Color Doppler ultrasonography and contrast-enhanced CT are usually the initial diagnostic tests for portal vein thrombosis Magnetic resonance angiography (MRA) of the portal system is generally confirmatory Endoscopic ultrasonography may be helpful in some cases In patients with jaundice, magnetic resonance cholangiography may demonstrate compression of the bile duct by a large portal cavernoma (portal biliopathy) In patients with pyelephlebitis, CT may demonstrate An intra-abdominal source of infection Thrombosis or gas in the portal venous system Hepatic abscess ++ Diagnostic Studies... GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription GET ACCESS TO THIS RESOURCE Subscription Options Pay Per View Timed Access to all of AccessMedicine 24 Hour $34.95 (USD) Buy Now 48 Hour $54.95 (USD) Buy Now Best Value AccessMedicine Full Site: One-Year Individual Subscription $995 USD Buy Now View All Subscription Options