Liver and intrahepatic bile ducts - nontumor
Vascular disorders
Hepatoportal sclerosis

Author: Raul S. Gonzalez, M.D. (see Authors page)

Revised: 6 January 2018, last major update December 2017

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Hepatoportal sclerosis [title]
Cite this page: Gonzalez, R.S. Hepatoportal sclerosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverhepatoportalsclerosis.html. Accessed July 21st, 2018.
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
Terminology
  • 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
Epidemiology
Etiology
  • 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
Diagnosis
  • 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
Treatment
  • 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.
Missing Image

Portal vein radicles

Missing Image

Portal vein herniation



Images hosted on other servers:
Missing Image

Portal tract abnormalities 1

Missing Image

Portal tract abnormalities 2

Board review 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