Hepatoportal Sclerosis

 Form of noncirrhotic portal hypertension






Etiology/Pathogenesis




• Associated with conditions that cause increased vascular resistance at presinusoidal level
image Genetic predisposition

image Chronic exposure to toxins and medications

image Prothrombotic states

image Autoimmune disorders

image Many cases idiopathic


Clinical Issues




• Up to 40% of patients with portal hypertension in India and Japan
image Increasingly recognized in Western countries

• Most common in young and middle-aged adults

• Better prognosis than cirrhotic portal hypertension because of preserved liver function

• Typically presents with signs of portal hypertension
image Usually normal or near normal liver function tests


Macroscopic




• Normal, enlarged, or shrunken liver with irregular, wrinkled capsular surface

• Dilatation and mural thickening of large portal veins


Microscopic




• Abnormal hepatic architecture with lack of consistent relationship between portal tracts and central veins

• Portal fibrosis

• Narrowing or obliteration of portal veins (phlebosclerosis)

• Concomitant nodular regenerative hyperplasia may be present

image
Macroscopic Features by MR Imaging
Abdominal MR shows an irregular hepatic surface with a shrunken appearance image and heterogeneous hepatic parenchyma due to portal fibrosis. Note the presence of splenomegaly image .


image
Abnormal Hepatic Architecture
Trichrome stain highlights abnormal approximation of portal tracts seen in a needle biopsy from a patient with portal hypertension. Portal fibrosis and narrowed portal veins image are evident. No central veins are seen between portal tracts in this field. Note the absence of cirrhosis.

image
Portal Fibrosis
This portal tract is expanded by fibrosis, with marked narrowing of the portal vein image. Note the presence of normal-caliber hepatic artery image and bile duct image. There are no significant inflammatory cell infiltrates in the portal tract.

image
Portal and Perisinusoidal Fibrosis
This portal tract shows marked fibrosis, minimal inflammatory cell infiltrates image, and multiple slit-like spaces image representing narrowed portal veins. Focal perisinusoidal collagen deposition image is also noted in this biopsy.


TERMINOLOGY


Abbreviations




• Hepatoportal sclerosis (HPS)

Tags:
Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Hepatoportal Sclerosis

Full access? Get Clinical Tree
Get Clinical Tree app for offline access