Management of hepatic vascular diseases.
Primary damage to hepatic vessels is rare. (i) Hepatic arterial disorders, related mostly to iatrogenic injury and occasionally to systemic diseases, lead to ischemic cholangiopathy. (ii) Hepatic vein or inferior vena cava thrombosis, causing primary Budd-Chiari syndrome, is related typically to a combination of underlying prothrombotic conditions, particularly myeloproliferative neoplasms, factor V Leiden, and oral contraceptive use. The outcome of Budd-Chiari syndrome has markedly improved with anticoagulation therapy and, when needed, angioplasty, stenting, TIPS, or liver transplantation. (iii) Extrahepatic portal vein thrombosis is related to local causes (advanced cirrhosis, surgery, malignant or inflammatory conditions), or general prothrombotic conditions (mostly myeloproliferative neoplasms or factor II gene mutation), often in combination. Anticoagulation at the early stage prevents thrombus extension and, in 40% of the cases, allows for recanalization. At the late stage, gastrointestinal bleeding related to portal hypertension can be prevented in the same way as in cirrhosis. (iv) Sinusoidal obstruction syndrome (or venoocclusive disease), caused by agents toxic to bone marrow progenitors and to sinusoidal endothelial cells, induces portal hypertension and liver dysfunction. Decreasing the intensity of myeloablative regimens reduces the incidence of sinusoidal toxicity. (v) Obstruction of intrahepatic portal veins (obliterative portal venopathy) can be associated with autoimmune diseases, prothrombotic conditions, or HIV infection. The disease can eventually be complicated with end-stage liver disease. Extrahepatic portal vein obstruction is common. Anticoagulation should be considered. (vi) Nodular regenerative hyperplasia is induced by the uneven perfusion due to obstructed sinusoids, or portal or hepatic venules. It causes pure portal hypertension.
Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, Hôpital Beaujon, AP-HP, Clichy, France.
Aurélie
A
Plessier
Pierre-Emmanuel
PE
Rautou
Dominique-Charles
DC
Valla
D001652:Bile Ducts / Q000098:blood supply*; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000628:therapy; D006504:Hepatic Veno-Occlusive Disease / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D007511:Ischemia / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D008107:Liver Diseases / Q000175:diagnosis* / Q000628:therapy*; D011169:Portal Vein; D013927:Thrombosis / Q000175:diagnosis / Q000628:therapy*