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World J Gastroenterol. Apr 7, 2007; 13(13): 1906-1911
Published online Apr 7, 2007. doi: 10.3748/wjg.v13.i13.1906
Clinical characteristics of idiopathic portal hypertension
Ozgur Harmanci, Yusuf Bayraktar
Ozgur Harmanci, Yusuf Bayraktar, Hacettepe University Faculty of Medicine, Department of Gastroenterology, Sihhiye 06100, Ankara, Turkey
Author contributions: All authors contributed equally to the work.
Correspondence to: Ozgur Harmanci, MD, Hacettepe University Faculty of Medicine, Department of Gastroenterology, Sihhiye 06100, Ankara, Turkey. ozgurmd@hacettepe.edu.tr
Telephone: + 90-312-4439428 Fax: +90-312-4429429
Received: October 10, 2006
Revised: October 15, 2006
Accepted: October 25, 2007
Published online: April 7, 2007
Abstract

Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that “not all varices mean cirrhosis”.

Keywords: Idiopathic portal hypertension; Non-cirrhotic portal fibrosis; Hepatoportal sclerosis; Portal vein thrombosis