Intrahepatic Portal Venopathy and Related Disorders of the Liver

Yogesh Chawla; Radha Krishan Dhiman

doi:10.1055/s-0028-1085095

Seminars in Liver Disease, Table of Contents

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

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

Abstract

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

Full Text

References

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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