Liver & intrahepatic bile ducts
Vascular disorders
Hepatoportal sclerosis

Topic Completed: 1 December 2017

Minor changes: 13 February 2020

Copyright: 2003-2020,, Inc.

PubMed Search: Hepatoportal sclerosis [title]

Raul S. Gonzalez, M.D.
Page views in 2019: 1,865
Page views in 2020 to date: 1,269
Cite this page: Gonzalez R. Hepatoportal sclerosis. website. Accessed August 6th, 2020.
Definition / general
  • Portal vein abnormalities and bridging fibrosis, causing portal hypertension
Essential features
  • Portal hypertension without overt liver failure
  • Variety of portal vein changes may be seen histologically
  • Also called obliterative portal venopathy, noncirrhotic portal fibrosis, idiopathic portal hypertension (Semin Liver Dis 2002;22:59)
  • Sometimes considered synonymous with incomplete septal cirrhosis, although overall this finding is more favored to indicate regressed cirrhosis
  • Can be idiopathic
  • Many cases likely due to extrahepatic or intrahepatic portal venous obstruction, mainly portal vein thrombosis
  • Also linked to exposure to arsenic or vinyl chloride, didanosine in AIDS patients (Curr Opin Infect Dis 2011;24:12) and other medications
Clinical features
  • Tissue generally required for diagnosis; on biopsy, findings are usually only "suggestive of" or "consistent with" the diagnosis, due to patchy nature of disease
Radiology description
Case reports
  • Patients may require anticoagulation if disease is secondary to portal vein thrombosis
Microscopic (histologic) description
  • Portal vein abnormalities, including fibrous intimal thickening, vein loss, herniation beyond portal tract borders into hepatic parenchyma and replacement by numerous small vein radicles
  • Collagen deposition into space of Disse and periportal areas
  • Bridging fibrosis between portal areas without background cirrhosis, although hepatocyte regeneration (nodular regenerative hyperplasia) may be present, as may parenchymal atrophy secondary to portal malperfusion
  • Obliteration of portal veins has been specifically linked to didanosine (AIDS 2010;24:1171)
Microscopic (histologic) images

Images hosted on PathOut server:

Images contributed by Raul S. Gonzalez, M.D.
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Portal vein radicles

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Portal vein herniation

Images hosted on other servers:
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Portal tract abnormalities 1

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Portal tract abnormalities 2

Board review style question #1
    A 60 year old man in the United States with no known history of liver disease develops ascites and is discovered to have portal hypertension. He undergoes biopsy, which shows portal tract abnormalities but no cirrhosis. Additional workup determines he has Factor V Leiden. What is the most likely cause of the patient’s portal hypertension?

  1. Cirrhosis missed on biopsy
  2. Granulomatous hepatitis
  3. Hepatoportal sclerosis
  4. Schistosomiasis
  5. Sinusoidal obstruction syndrome
Board review answer #1
C. Hepatoportal sclerosis
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