Vascular liver disease

Abstract

Vascular liver disease may affect the portal vein, the sinusoids, or the hepatic veins, and may be congenital or develop later during childhood. Clinical presentations vary markedly, depending on the location of the disease in the hepatic vascular anatomy, the nature of the pathology, the degree of hemodynamic change, and the acuity of onset of any vascular obstruction. Affected children may develop clinical manifestations of portal hypertension (e.g., ascites, gastrointestinal bleeding), portosystemic shunting (e.g., encephalopathy, hepatopulmonary syndrome), liver failure, liver masses, or high-output heart failure. Accurate diagnosis depends mostly on imaging. Treatments are directed at specific pathologies and hemodynamic abnormalities, and include medical therapies (e.g., nonspecific beta-blockers, defibrotide), interventional radiology procedures, and surgery. We describe the current approach to children with portal vein thrombosis, Budd-Chiari syndrome, sinusoidal obstruction syndrome, idiopathic non-cirrhotic portal hypertension, congenital vascular malformations, and liver vascular tumors.