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Diseases

Genetic and Rare Diseases Information Center (GARD)

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.

Treatment

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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

References
  1. 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.
  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. http://www.ncbi.nlm.nih.gov/pubmed/21571652. Accessed 1/27/2012.


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

    Finding Treatment Information
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.