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Hepatology International
Published in: Hepatology International 1/2018

01-02-2018 | Special Issue - Portal Hypertension

Idiopathic portal hypertension and extrahepatic portal venous obstruction

Authors: Rajeev Khanna, Shiv Kumar Sarin

Published in: Hepatology International | Special Issue 1/2018

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Abstract

Background

Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations.

Aim

The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO.

Results

IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions. While the level of block in IPH is presinusoidal, in EHPVO it is at prehepatic level. Infections, autoimmunity, drugs, immunodeficiency and prothrombotic states are possible etiological agents in IPH. Contrastingly in EHPVO, prothrombotic disorders and local factors around the portal vein are the incriminating factors. Diagnosis is often clinical, supported by simple radiological tools. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority of patients in both the disorders. Surgical shunts revert the complications secondary to PHT. Meso-Rex shunt has become the standard surgery in children with EHPVO.

Conclusion

This review gives a detailed summary of these two vascular conditions of liver—IPH and EHPVO. Further research is needed to understand the pathogenesis and natural history of these disorders.
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Metadata
Title
Idiopathic portal hypertension and extrahepatic portal venous obstruction
Authors
Rajeev Khanna
Shiv Kumar Sarin
Publication date
01-02-2018
Publisher
Springer India
Published in
Hepatology International / Issue Special Issue 1/2018
Print ISSN: 1936-0533
Electronic ISSN: 1936-0541
DOI
https://doi.org/10.1007/s12072-018-9844-3

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