Noncirrhotic Portal Fibrosis

Noncirrhotic portal hypertension describes a cohort of patients with features of portal hypertension (esophageal and gastric varices and splenomegaly) in the absence of injury to the hepatic parenchyma. Multiple hypotheses are proposed to explain the development of non-cirrhotic portal fibrosis in an individual-underlying prothrombotic disease, endothelial-to-mesenchymal transformation, and endotoxin-induced portal hypertension. The disease course is predominantly characterized by recurrent episodes of variceal bleeding with transient, mild, self-resolving ascites. Treatment algorithms focus on the prevention of variceal bleeding. Recent advances in management include endovascular interventions, transjugular intrahepatic portosystemic shunts for recurrent variceal hemorrhage. Liver transplantation may be considered for patients with features of poor hepatic function.