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Genetic and Rare Diseases Information Center (GARD)

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Congenital extrahepatic portosystemic shunt

Other Names for this Disease
  • Abernethy malformation
  • CEPS
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What are the symptoms of congenital extrahepatic portosystemic shunt?

The symptoms of congenital extrahepatic portosystemic shunt (CEPS) vary and are caused by blood not moving through the liver.  The liver removes certain toxic chemicals from the blood; if a CEPS exists, these chemicals stay in the blood and can cause signs of liver disease or problems in the brain (hepatic encephalopathy).  Hepatic encephalopathy occurs more often in older people with CEPS.[1]
Last updated: 2/1/2012

  1. Alonso-Gamarra E, Parrón M, Pérez A, Prieto C, Hierro L, López-Santamaría M. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics. 2011; 31:707-722. Accessed 1/27/2012.