J Pathol Transl Med.  2016 Jan;50(1):17-25. 10.4132/jptm.2015.09.23.

Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

• Lee H
• Rehman AU
• Fiel MI

• KMID: 2211397
• DOI: http://doi.org/10.4132/jptm.2015.09.23

Abstract

Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity.

Keyword

Idiopathic; Noncirrhotic; Hypertension, portal; Liver; Histopathology

MeSH Terms

Diagnosis
Ethnic Groups
Fibrosis
Humans
Hypertension, Portal*
Liver
Risk Factors

Figure

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  Fig. 1. Gross cut surface of a liver with idiopathic noncirrhotic portal hypertension demonstrates vague nodularity without cirrhosis.

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  Fig. 2. Phlebosclerosis with narrowed venous lumen.

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  Fig. 3. Portal sclerosis associated with increased number of vascular channels.

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  Fig. 4. Abnormally dilated portal venous branches associated with herniation of the vein into the hepatic lobule (shunt vessel).

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  Fig. 5. (A) Regenerative nodules of the lobule without cirrhosis. (B) Reticulin stain of the corresponding area highlights regenerative nodules.

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  Fig. 6. Dilated sinusoids (also known as megasinusoids).

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  Fig. 7. Abnormally dilated veins in the lobule.

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  Fig. 8. Distorted hepatic architecture in idiopathic noncirrhotic portal hypertension.

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  Fig. 9. Hypoplastic/rudimentary portal tract.

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