Clinical characteristics of idiopathic portal hypertension

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".

Figure 1

Our proposed theory about etiopathogenesis of idiopathic portal hypertension.

Figure 2

The histological examination of the portal vein shows periportal thickening (marked by asterisks) found in an IPH patient.

Figure 3

Splenopo-rtography shows two major sites of thrombosis in portal venous system (shown by asterisks). Note the dilated portal vein at hepatic hilum despite portal vein thrombosis.

Figure 4

The computed tomography of the same patient in figure-3. Note the thrombosis in the portal vein (Marked by arrow).

Figure 5

Splenoportography shows patent portal vein with severe dilation and collateral circulation. Note the small caliber of intrahepatic portal vein branches compared to extrahepatic portal vein.