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Non-Cirrhotic portal fibrosis

  1. Non cirrhotic Hypertension → can be due to non cirrhotic portal fibrosis (NCPF) or Extra hepatic portal venous obstruction (EHPVO)
  2. It is also called: Idiopathic portal hypertension
  3. Its incidence is very high in India accounts for 15% to 18% of all patients with portal hypertension.
  4. Aetiology:
    1. Infection → E. coli can cause NCPF
    2. Exposure to chemicals → Chronic ingestion of (1) Arsenic (2) Vinyl Chloride toxicity (3) Copper

Extra Edge (Arsenic also causes skins Ca, lung Ca, hepatic angiosarcoma)

Clinical features:
  1. Massive hematemesis. (M/C presentation)
  2. Rarely patient develop encephalopathy.
  3. Ascites is uncommon
  4. Splenomegaly is present
  5. Jaundice and signs of liver cell failure are uncommon.
  1. Normal liver function test
    1. Normal PT
    2. S. Proteins normal
  2. Portography:
    1. Weeping willow appearance – sudden cut off of peripheral portal vein branches.
    2. Tree in winter appearance – filling of a large number of collaterals with gross distortion of intrahepatic pattern.
    3. Splenic vein is dilated and tortuous.
Treatment of choice is endoscopic sclero therapy.
DD is EHPO (Extra hepatic portal venous obstruction) 
Comparison between NCPF and EHPVO
  1. Similarities between the two conditions
    1. Massive variceal bleeding
    2. Moderate to massive splenomegaly
    3. Normal liver on histology
    4. Absence of Ascites
    5. Normal liver function
  2. Difference between these two conditions
    1. Age of presentation
      1. EHPVO → Children
      2. NCPF → Young adult
    2. Site of obstruction in the portal venous system
      1. EHPVO → Main and 1st order branches of portal vein
      2. NCPF → Smaller 3rd and 4th order branches of portal vein are involved.
    3. Splenomegaly – more common in NCPF than EHPVO

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