Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 16-17: Noncirrhotic Portal Hypertension + Key Features Download Section PDF Listen +++ +++ 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 nonocclusive, acute or chronic, extent (eg, into the mesenteric vein), and 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 Download Section PDF Listen +++ +++ 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 Download Section PDF Listen +++ +++ 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 ++ Endoscopy shows esophageal or gastric varices A low liver stiffness measurement by elastography may help distinguish noncirrhotic portal hypertension from cirrhosis Needle biopsy of the liver May be indicated to diagnose schistosomiasis, nodular regenerative hyperplasia, and noncirrhotic portal fibrosis May demonstrate sinusoidal dilatation + Treatment Download Section PDF Listen +++ +++ Medications ++ Anticoagulation (particularly with low-molecular-weight or unfractionated heparin) or thrombolytic therapy May be indicated for isolated acute portal vein thrombosis (leads to at least partial recanalization in up to 75% of cases when started within 6 months of thrombosis) and possibly for acute splenic vein thrombosis An oral anticoagulant may be continued long-term if a hypercoagulable disorder is identified or if an acute portal vein thrombosis extends into the mesenteric veins A possible role for enoxaparin to prevent portal vein thrombosis and hepatic decompensation in patients with cirrhosis has shown promise +++ Surgery ++ Splenectomy is curative if splenic vein thrombosis is the cause of variceal bleeding Liver transplantation is required for progressive liver dysfunction (rare) +++ Therapeutic Procedures ++ Band ligation followed by β-blockers to reduce portal pressure is initiated for variceal bleeding Portosystemic shunting (including TIPS) is reserved for when endoscopic therapy fails + Outcome Download Section PDF Listen +++ +++ When to Refer ++ Refer all patients with noncirrhotic portal hypertension to a hepatologist + References Download Section PDF Listen +++ + +De Gottardi A et al; VALDIG Group. Porto-sinusoidal vascular disease: proposal and description of a novel entity. Lancet Gastroenterol Hepatol. 2019 May;4(5):399–411. [PubMed: 30957754] + +Hernández-Gea V et al. Idiopathic portal hypertension. Hepatology. 2018 Dec;68(6):2413–23. [PubMed: 30066417] + +Hoolwerf EW et al. Direct oral anticoagulants in patients with liver cirrhosis: a systematic review. Thromb Res. 2018 Oct;170:102–8. [PubMed: 30153564] + +Intagliata NM et al. Diagnosis, development, and treatment of portal vein thrombosis in patients with and without cirrhosis. Gastroenterology. 2019 May;156(6):1582–99. [PubMed: 30771355] + +Khanna R et al. Noncirrhotic portal hypertension: current and emerging perspectives. Clin Liver Dis. 2019 Nov;23(4):781–807. [PubMed: 31563222] + +Loffredo L et al. Effects of anticoagulants in patients with cirrhosis and portal vein thrombosis: a systematic review and meta-analysis. Gastroenterology. 2017 Aug;153(2):480–7. [PubMed: 28479379] + +Pettinari I et al; BO-LIVES (BOlogna LIVEr vascular Studies). Clinical impact and safety of anticoagulants for portal vein thrombosis in cirrhosis. Am J Gastroenterol. 2019 Feb;114(2):258–66. [PubMed: 30538290] + +Stine JG et al. Management of non-tumoral portal vein thrombosis in patients with cirrhosis. Dig Dis Sci. 2019 Mar;64(3):619–26. [PubMed: 30560339] + +Young K et al. Evaluation and management of acute and chronic portal vein thrombosis in patients with cirrhosis. Clin Liver Dis. 2017 Dec;10(6):152–6.