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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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How might congenital extrahepatic portosystemic shunt (CEPS) be treated?

Because congenital extrahepatic portosystemic shunts (CEPS) are rare, there are no guidelines for standard treatment of this condition.[1]  Treatment is determined on an individual basis and depends on the type of CEPS.  In type I CEPS, liver transplantation is thought to be the only treatment.  Type II CEPS can be treated with surgery to close the connection between the hepatic portal vein and the hepatic vein, which would redirect bloodflow through the normal blood vessels within the liver.[2]
Last updated: 2/1/2012

  1. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatric Radiology. 2003; 33:614-620. Accessed 1/27/2012.
  2. 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.