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Semin Liver Dis 2008; 28(3): 270-281
DOI: 10.1055/s-0028-1085095
© Thieme Medical Publishers

Intrahepatic Portal Venopathy and Related Disorders of the Liver

Yogesh Chawla1 , Radha Krishan Dhiman1
  • 1Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Further Information

Publication History

Publication Date:
23 September 2008 (online)

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ABSTRACT

Intrahepatic portal venopathy leads to various entities that are important causes of portal hypertension. Noncirrhotic portal fibrosis (NCPF) occurs in the Indian subcontinent, whereas idiopathic portal hypertension (IPH) occurs in Japan although the pathogenesis and presentation of both are similar. NCPF presents mainly with upper gastrointestinal bleeding; IPH presents with massive splenomegaly. The liver functions are preserved. Wedged hepatic venous pressure is normal, but portal venous pressure is high indicating a presinusoidal block. Patients are best managed with endoscopic therapy or surgery, with better results than in patients with cirrhosis. Nodular regenerative hyperplasia is a histological diagnosis characterized by development of nodules in the liver due to uneven perfusion of the portal venous blood. These patients may develop portal hypertension and if they bleed would require treatment as in NCPF/IPH. Schistosomiasis produces portal hypertension by the development of fibrous tissue around the portal veins as a response to schistosome eggs. Gratifying results have been reported with praziquantel therapy. Rarely sarcoidosis and chronic biliary obstruction may also produce portal venopathy.

KEYWORDS

Hepatoportal sclerosis - schistosomiasis - idiopathic portal hypertension - noncirrhotic portal fibrosis - nodular regenerative hyperplasia - incomplete septal cirrhosis - noncirrhotic portal hypertension

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Yogesh ChawlaM.D. 

Professor and Head, Department of Hepatology, Postgraduate Institute of Medical Education and Research

Chandigarh 160 012, India

Email: ykchawla@gmail.com

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