Progress in the clinical diagnosis of idiopathic noncirrhotic portal hypertension

Abstract

Idiopathic noncirrhotic portal hypertension (INCPH) is an uncommon disease of intrahepatic portal hypertension with associated clinical manifestations such as esophageal varices, hypersplenism and ascites. This diagnosis is rendered following the exclusion of other causes of portal hypertension, including cirrhosis, other presinusoidal, sinusoidal and post sinusoidal causes of portal hypertension and splanchnic venous thrombosis. INCPH is relatively uncommon in the western world, and the terminology and diagnostic criteria of INCPH remain controversial. Histological features commonly observed in INCPH are nodular regeneration of the hepatocytes, phlebosclerosis, increased number of portal vessels, sinusoidal dilatation, periportal shunting vessels, perisinusoidal fibrosis and rudimentary portal tracts. It’s urge to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, the pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. Key words: Portal hypertension; Idiopathic; Etiology; Diagnosis