Last update:
25-Nov-2018

Portal vein thrombosis and Budd Chiari syndrome are the most widely known vascular diseases of the liver. Over time, however, a number of rarer vascular diseases with significant morbidity and mortality have been identified, which form a heterogeneous group with variability in pathophysiology, clinical presentation, prognosis and indicated treatment. Idiopathic portal hypertension is a rare disease of unknown origin, leading to clinically significant portal hypertension. Liver function is usually preserved, its natural course is benign, and the treatment is primarily symptomatic. Obstruction of the hepatic artery is manifest as ischemic cholangiopathy syndrome, which ranges from an asymptomatic disorder to a life-threatening condition that may require liver transplantation in its final stages. Sinusoidal obstruction syndrome is caused mainly by intensive chemotherapeutic regimens administered in preparation for stem cell transplantation. Use of more conventional dosage and the avoidance of hepatotoxic agents reduce its incidence. Hepatic peliosis is characterized by the presence of diffuse blood-filled cystic spaces in the parenchymal organs, including the liver. It has been associated with toxic, immunological and infectious factors, and the most appropriate therapeutic approach is identification and treatment of the underlying cause. Ηereditary hemorrhagic telangiectasia is associated with hepatic involvement in 8–31% of patients, in whom it gives rise to vascular malformations of varying size. In the case of advanced liver involvement, transplantation is the only definitive treatment. In the future, new forms of treatment may improve the outcome of these conditions.

Key words: Hepatic peliosis, Hereditary hemorrhagic telangiectasia, Hyperplasia, Nodular regenerative non-cirrhotic portal hypertension, Portal hypertension, Sinusoidal obstruction syndrome, Vascular liver diseases.