Current concepts in the management of non-cirrhotic non-malignant portal vein thrombosis

Figure 1 Cross-sectional images of recent portal vein thrombosis and subsequent cavernoma formation. A: Axial computed tomography demonstrating acute portal vein thrombosis (Orange arrow) with altered hepatic parenchymal attenuation secondary to ischaemia; there is an incidental large splenic cyst; B: Coronal computed tomography demonstrating acute portal vein thrombosis (Orange arrow) with altered hepatic parenchymal attenuation secondary to ischaemia; there is an incidental large splenic cyst; C: Axial computed tomography 6 months later in the same patient, demonstrating formation of portal vein cavernoma (Orange arrow); D: Coronal computed tomography 6 months later in the same patient, demonstrating formation of portal vein cavernoma (Orange arrow).

Figure 2 Suggested management algorithm for recent non-cirrhotic non-malignant portal vein thrombosis. ¹Major risk factors include: Myeloproliferative neoplasms, antiphospholipid syndrome, paroxysmal nocturnal haemoglobinuria, homozygous or compound heterozygous factor V Leiden or prothrombin G20210A gene mutations, personal or first-degree familial history of unprovoked deep vein thrombosis. ²Consider measuring D-dimer. If D-dimer > 500 ng/mL at 1 month following anticoagulation discontinuation consider long-term anticoagulation. NCPVT: Non-cirrhotic non-malignant portal vein thrombosis.

Figure 3 Suggested management algorithm for chronic non-cirrhotic non-malignant portal vein thrombosis. ¹Major risk factors include: Myeloproliferative neoplasms, antiphospholipid syndrome, paroxysmal nocturnal haemoglobinuria, homozygous or compound heterozygous factor V Leiden or prothrombin G20210A gene mutations, personal or first-degree familial history of unprovoked deep vein thrombosis. ²Consider long-term anticoagulation on a case-by-case basis. Where a decision is made to discontinue existing anticoagulation consider measuring D-dimer. If D-dimer > 500 ng/mL at 1 month following anticoagulation discontinuation consider long-term anticoagulation. NCPVT: Non-cirrhotic non-malignant portal vein thrombosis; PVR: Portal vein recanalization; TIPS: Transjugular intrahepatic portosystemic shunt.