An acquired syndrome with idiopathic portal hypertension and splenic anemia.
Banti-Senator Disease; Senator Syndrome; Spleen-Liver Syndrome; Chronic Congestive Splenomegaly; Noncirrhotic Idiopathic Portal Hypertension. Idiopathic Congestive Splenomegaly; Idiopathic Presinusoidal Portal Hypertension; Hepatolienal Fibrosis; Hepatoportal Sclerosis; Noncirrhotic Portal Fibrosis. Some authors prefer to distinguish between Banti Disease and Banti Syndrome, with the latter then referring to Banti disease caused by a chronic infection (eg, malaria, syphilis) or intoxication, ie, a known cause, although the lines between the two appear somewhat blurry.
First described in 1882 by the Italian pathologist Guido Banti (1852–1925).
Incidence and genetic inheritance
The incidence is unknown, but the disease seems more common in India and Japan. In India, it accounts for up to 30% of all patients with portal hypertension. There is no known genetic predisposition to this disorder. Some studies found a male:female ratio of about 1:1.5 and the age at presentation in a study from India ranged from 10 to 59 years (with a mean of 30 years).
This disorder is characterized by portal hypertension with increased splenic and portal venous pressures in the absence of any obvious etiology (eg, cirrhosis, blood disease, parasites (eg, schistosomiasis), occlusion of the hepatic and/or portal veins). Possible causes of portal hypertension may include toxins (eg, arsenic, copper sulfate, vinyl chloride), infections (eg, syphilis, malaria) and immunologic or genetic factors. In Japan, idiopathic portal hypertension is frequently associated with autoimmune disorders (eg, systemic lupus erythematosus, progressive systemic sclerosis, thyroiditis or mixed connective tissue disease). Portal hypertension is accompanied by dilatation and wall thickening of the portal vein, esophageal varices, and significant splenomegaly (often >10 cm below the left costal margin). Cytopenia of at least one blood cell line (ie, anemia, leuco- or thrombocytopenia) accompany hypersplenism, which is present in almost half of the patients. A liver biopsy is performed to exclude a primary hepatic cause and may demonstrate intact lobular architecture, intimal fibroelastosis of the medium-sized portal veins, sclerosis with obliteration of the small-sized portal vein branches, portal fibrosis of variable extent, and subcapsular scarring. Extrahepatic portal vein obstruction must also be excluded. The hepatic veins are patent, but show elevated hepatic venous wedge pressure, a patent portal vein with increased pressure and presence of multiple collaterals (the left gastric vein, short gastric vein, and superior mesenteric vein). Imaging of the abdominal vessels reveals spontaneous shunts (splenorenal and umbilical vein) in more than 15% of patients. These spontaneous shunts reduce the risk of esophageal variceal bleeding. The classical imaging findings are selective dilatation of the left branch of the portal vein with an abrupt narrowing of the major intrahepatic branches, paucity of medium-sized portal branches, and an avascular subcapsular zone. Visible abdominal veins (caput medusae or palm-tree sign) are found in almost 20% of patients. Liver function tests are ...