Table 1.

Criteria for Diagnosing Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH).

Essential criteria14 •Presence of portal hypertension with splenomegaly •Patent portal vein and hepatic venous outflow tract •Normal or near normal liver function •Absence of parenchymal disease and cirrhosis/advanced fibrosis on liver biopsy •No obvious etiology of chronic liver disease (e.g. alcohol, Hepatitis viruses) Other etiologies with similar histological findings are ruled out (e.g. congenital hepatic fibrosis, primary biliary cirrhosis, sarcoidosis, schistosomiasis) Variants–supplementary (non-essential) criteria. Nodular regenerative hyperplasia9: With distinct contrast between nodular and internodular tissue in most area nodules less than 3 mm. Hepatoportal sclerosis11 (i) Thrombosis/sclerosis of small portal vein branches; and/or (ii) intrahepatic aberrant abnormal portal venules, defined as absent or clearly reduced in caliber with sclerosis of vessels. Obliterative portal venopathy15 (i) Liver biopsy longer than 1 cm and containing more than 5 complete portal tracts; more than 2/3 (66%) of the complete portal tracts harboring thickening of the vein's smooth muscle wall. Incomplete septal cirrhosis10 (i) Parenchymal nodularity; (ii) thin incomplete septa.