KEY CLINICAL UPDATES IN NONCIRRHOTIC PORTAL HYPERTENSION
Contrast-enhanced CT or magnetic resonance angiography of the portal system can assess extension of thrombus into the mesenteric veins and exclude tumor thrombus in patients with cirrhosis.
The decision to give an anticoagulant to a patient with cirrhosis and portal vein thrombosis depends on the presence of ascites, the patient's fall risk, and the patient's candidacy for liver transplantation.
Moreover, partial portal vein thrombosis may resolve in 30–50% of cases.
Data on the use of direct-acting oral anticoagulants in patients with cirrhosis and portal vein thrombosis are lacking.
Causes of noncirrhotic portal hypertension 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, and arterial-portal vein fistula. Idiopathic noncirrhotic portal hypertension is common in India and has been attributed to chronic infections, exposure to medications or toxins, prothrombotic disorders, immunologic disorders, and genetic disorders that result in obliterative vascular lesions in the liver. It is rare in Western countries, where increased mortality is attributable to associated disorders and older age; the term portosinusoidal vascular disease has been proposed.
Portal vein thrombosis may occur in 10–25% of patients with cirrhosis, is associated with the severity of the liver disease and related in part to acquired protein C deficiency and splenorenal shunts (resulting in stagnant portal venous blood flow), and may be associated with hepatocellular carcinoma but not with increased mortality. Other risk factors are oral contraceptive use, pregnancy, chronic inflammatory diseases (including pancreatitis), injury to the portal venous system (including surgery), other malignancies, and treatment of thrombocytopenia with eltrombopag. 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; or type 3, involving the trunk and branches of the portal vein. Additional descriptors are occlusive or nonocclusive, recent or chronic, and extension (into the mesenteric vein) as well as the nature of any underlying liver disease. Splenic vein thrombosis may complicate pancreatitis or pancreatic cancer. Pylephlebitis (septic thrombophlebitis of the portal vein) may complicate intra-abdominal inflammatory disorders such as appendicitis or diverticulitis, particularly when anaerobic organisms (especially Bacteroides species) are involved. Nodular regenerative hyperplasia results from altered hepatic perfusion and can be associated with collagen vascular diseases; myeloproliferative disorders; and drugs, including azathioprine, 5-fluorouracil, oxaliplatin, and thioguanine; an association with a mutation in the telomerase gene has been reported. In patients infected with HIV, long-term use of didanosine and use of a combination of didanosine and stavudine have been reported to account for some cases of noncirrhotic portal hypertension often due to nodular regenerative hyperplasia; genetic factors may play a role. The term “obliterative portal venopathy” ...