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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
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Congenital extrahepatic portosystemic shunt (CEPS), also known as Abernethy malformation, occurs when blood vessels near the liver do not form correctly during fetal development.  The hepatic portal vein delivers blood to the liver; in CEPS, the portal vein may not develop or another blood vessel may attach to the portal vein, redirecting (shunting) blood away from the liver rather than through the liver. CEPS is divided into two types based on if blood can flow through the liver. In type 1, certain blood vessels in the liver are missing. In type 2, these blood vessels are present but they have abnormal connections.  Both types can be associated with other complications, such congenital heart disease, liver disease, nodules on the liver, and hepatic encephalopathy.[1][2]
Last updated: 6/20/2012

References

  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. http://www.ncbi.nlm.nih.gov/pubmed/21571652. Accessed 1/27/2012.
  2. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatric Radiology. 2003; 33:614-620. http://www.ncbi.nlm.nih.gov/pubmed/12879313. Accessed 1/27/2012.
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Other Names for this Disease
  • Abernethy malformation
  • CEPS
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.