Extra-hepatic portal vein obstruction

Extra-hepatic portal vein obstruction is the most common cause of noncirrhotic portal hypertension in children and young adults in developing countries. It may or may not extend into intrahepatic portal veins.

It usually occurs in children and young adults, presenting as unexplained gastrointestinal bleeding.

Other features include:

  • obstructive jaundice may be seen due to extrahepatic compression of the bile duct by cavernous collaterals
  • splenomegaly
  • ascites

Cavernous transformation of portal vein usually occurs to maintain the flow to the liver.

  • idiopathic (50% cases)
  • children
    • neonatal infections
    • umbilical catheterization
    • developmental anomalies
    • trauma/surgery
  • young adults
    • infections
    • neoplastic diseases
    • pancreatitis
    • myeloproliferative disorders
    • hypercoagulable states

Features include:

  • normal liver echotexture
  • splenomegaly
  • splenic hilar collaterals
  • cavernous transformation of the portal vein
  • ascites

See: portal vein thrombosis

Options include:

  • endoscopic variceal band ligation 
  • endoscopic sclerotherapy
  • Rex shunt: mesenterico-left portal vein shunt
    • Rex segment (umbilical segment of left portal vein) must be patent for this
      • retrograde portography may be used to identify its patency, if non-invasive imaging fails
  • spleno-renal shunt (distal or proximal)
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Article information

rID: 32082
Tag: cases
Synonyms or Alternate Spellings:
  • Extra-hepatic portal vein obstruction (EHPVO)

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