Non-cirrhotic portal hypertension - The point of view of the pathologis

Abstract

Non-cirrhotic portal hypertension - The point of view of the pathologist Abstract. Non-cirrhotic portal hypertension (NCPH) is a heterogeneous group of liver disorders leading to portal hypertension. Many disorders are associated with NCPH. Based on the site of resistance to blood flow, they are classified as pre-hepatic, hepatic, and post-hepatic. Hepatic causes are further subdivided into pre-sinusoidal, sinusoidal and post-sinusoidal. The diagnosis of idiopathic non-cirrhotic portal hypertension (INCPH) can be made if all these disorders have been excluded and consequently no clear liver disease has been identified. INCPH is clinically characterized by features of PHT, moderate to massive splenomegaly, with or without hypersplenism, and preserved liver functions. This review is focused on pathological features of INCPH, which is thought to be caused largely by parenchymal vascular obstruction. INCPH must be considered in every patient presenting with clinical signs of portal hypertension where cirrhosis appears to be absent and the cause of portal hypertension is not clear. In most instances, patients present with bleeding esophageal varices. In order to exclude severe fibrosis or cirrhosis, liver histology remains essential in the diagnosis of INCPH.