The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.
Keywords: BCS, Budd-Chiari syndrome; CALR, calreticulin; Cavernoma; DOACs, direct-acting oral anticoagulants; Direct oral anticoagulants; EHPVO, extrahepatic portal vein obstruction; GFR, glomerular filtration rate; JAK2, Janus kinase 2; LMWH, low-molecular-weight heparin; MPN, myeloproliferative neoplasm; MTHFR, methylene-tetrahydrofolate reductase; PNH, paroxysmal nocturnal hemoglobinuria; PVT, portal vein thrombosis; Portal biliopathy; Portal vein recanalisation; SVT, splanchnic vein thrombosis; TIPS, transjugular intrahepatic portosystemic shunt; VKAs, vitamin K antagonists; Vascular liver diseases.
© 2022 The Author(s).