Idiopathic non-cirrhotic portal hypertension

Idiopathic portal hypertension ("Noncirrhotic portal hypertension" or "Banti syndrome") is a term that has been given to portal hypertension occurring without hepatic cirrhosis, parasitic infection, or portal venous thrombus.

Epidemiology

Rare condition. More common in India and Japan.

Pathology

Essentially a form of pre-sinusoidal portal hypertension, a mechanism for the noncirrhotic portal hypertension is unclear. Some have suggested an unknown infectious agent or immunologic event that leads to portal venous system fibrosis. Some documented cases may represent a failure to diagnose a more common pre-sinsoidal etiology. 

The condition was originally thought to arise from splenic congestion and increased splenic vein blood flow ("congestive splenomegaly"), but is now thought to be related to increased portal system resistance

Epidemiology

Rare condition. More common in India and Japan.

Clinical presentation

• splenomegaly
• may present with UGI bleeding from varices
• may present with anemia, leukopenia, moderate thrombocytopenia

Radiographic features

• sequalae of portal hypertension, including
  • splenomegaly
  • esophageal and/or gastric varices
  • elevated hepatic wedge pressures
• liver
  • no evidence of cirrhosis
  • subcapsular parenchymal atrophy

Treatment and prognosis

Thought to have a more favorable outcome than cirrhotic portal hypertension, but treatment strategies currently attempt to control the varices rather than treat the portal hypertension.