3.5 Idiopathic non-cirrhotic portal hypertension

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This condition is rare and has different names in the literature (hepatoportal sclerosis, non-cirrhotic portal fibrosis, incomplete septal cirrhosis, nodular regenerative hyperplasia).

As a result of phlebosclerosis of the portal vein, there is a perfusion disorder of the liver. It is usually associated with immunological and systemic diseases, use of medication such as azathioprine, HIV medication and thrombophilic diseases. Non-cirrhotic portal hypertension is frequently found in cystic fibrosis (as a result of precipitation of bile in the small bile ducts).

It gives portal hypertension which usually manifests itself in the form of a greatly enlarged spleen and esophageal varices. On an ultrasound there are signs reminiscent of cirrhosis such as noularity of the liver parenchyma, but an elastography measurement gives values ​​< 10 kPa.

An increased incidence of portal vein thrombosis is found. The short-term prognosis is good (normal liver synthesis capacity), but it can evolve into chronic liver failure and the need for liver transplantation.

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