Extrahepatic portal vein obstruction

Last revised by Dr Jeremy Jones on 20 Sep 2021

Extrahepatic portal vein obstruction is the most common cause of non-cirrhotic portal hypertension in children and young adults in developing countries. It may or may not extend into the intrahepatic portal vein.

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

Other features include:

  • idiopathic (50% cases)
  • children
  • young adults
    • infections
    • neoplastic diseases
    • pancreatitis
    • myeloproliferative disorders
    • hypercoagulable states

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

Features include:

Options include:

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

ADVERTISEMENT: Supporters see fewer/no ads

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.