Abstract
Splanchnic vein thrombosis includes thrombosis of the hepatic venous system (Budd–Chiari
syndrome) and thrombosis of the portal venous system. Both conditions share uncommon
prothrombotic disorders as causal factors, among which myeloproliferative neoplasms
rank first. Budd–Chiari syndrome presents with acute or chronic, asymptomatic or severe
liver disease. Diagnosis depends on noninvasive imaging of the obstructed hepatic
venous outflow tract. A spontaneously fatal course can be prevented by a stepwise
approach: (1) anticoagulation therapy, specific therapy for underlying disease, and
medical or endoscopic management of liver-related complications, (2) angioplasty/stenting
in a second step, and (3) eventually the insertion of transjugular intrahepatic stent
shunt or liver transplantation. Recent portal vein thrombosis mostly jeopardizes the
gut. Early anticoagulation prevents thrombus extension but is incompletely successful
in achieving recanalization. Chronic portal vein thrombosis is complicated by bleeding
related to portal hypertension, which can be prevented by usual pharmacological and
endoscopic means. The prevention of recurrent thrombosis is achieved by anticoagulation
therapy the impact of which on the risk of bleeding remains unclear. Portal vein thrombosis
in patients with cirrhosis is likely neither a direct consequence of nor a direct
cause for liver disease progression. Therefore, the indications and effects of anticoagulation
therapy for portal vein thrombosis in patients with cirrhosis remain uncertain.
Keywords hepatic vein thrombosis - Budd–Chiari syndrome - portal vein thrombosis - cavernoma
- TIPS