Idiopathic portal hypertension

Dr Owen Kang and Dr Matt A. Morgan et al.

Idiopathic portal hypertension (noncirrhotic portal hypertension or Banti syndrome) is a term that has been given to portal hypertension occurring without hepatic cirrhosis, parasitic infection, or portal venous thrombosis.

Rare condition. More common in India and Japan.

Essentially a form of pre-sinusoidal portal hypertension, a mechanism for this non-cirrhotic portal hypertension is unclear. Some have suggested an unknown infectious agent or immunologic event that leads to portal venous system fibrosis. Some documented cases may represent a failure to diagnose a more common pre-sinsoidal aetiology. 

The condition was originally thought to arise from splenic congestion and increased splenic vein blood flow ("congestive splenomegaly"), but is now thought to be related to increased portal system resistance.

  • splenomegaly
  • may present with upper gastrointestinal bleeding from varices
  • may present with anaemia, leukopaenia, moderate thrombocytopaenia
General
  • sequelae of portal hypertension, including
    • splenomegaly
    • oesophageal and/or gastric varices
    • elevated hepatic wedge pressures
  • liver
    • no evidence of cirrhosis
    • subcapsular parenchymal atrophy

Thought to have a more favourable outcome than cirrhotic portal hypertension, but treatment strategies currently attempt to control the varices rather than treat the portal hypertension.

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Article information

rID: 33857
Section: Pathology
Synonyms or Alternate Spellings:
  • Banti syndrome
  • Noncirrhotic portal hypertension
  • Noncirrhotic portal fibrosis
  • Banti's syndrome

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Cases and figures

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    Dilated portal vein
    Case 1: on ultrasound
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    Case 1: on CT
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