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Genetic and Rare Diseases Information Center (GARD)


Other names people use for this condition
  • CEPS
  • Abernethy malformation
Home > Rare Diseases and Related Terms > Congenital extrahepatic portosystemic shunt



Congenital extrahepatic portosystemic shunt
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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]


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. Available at: http://www.ncbi.nlm.nih.gov/pubmed/21571652. January 27, 2012.
  2. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatric Radiology. 2003;33:614-620. Available at: http://www.ncbi.nlm.nih.gov/pubmed/12879313. January 27, 2012.
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    • PubMed is a searchable database of medical literature and lists journal articles that discuss Congenital extrahepatic portosystemic shunt. Click on the link to view a sample search on this topic.
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    • DNAandU.org is a Web site and blog that collects firsthand stories from people facing issues, making tough decisions, and using genomic (DNA) information in their own healthcare.  Click on the link to learn from their experiences and/or submit your own story.

    • Madisons Foundation
      P.O. Box 241956
      Los Angeles, CA 90024
      Telephone: 310-264-0826
      Fax: 310-264-4766
      E-mail: getinfo@madisonsfoundation.org  
      Web site: http://www.madisonsfoundation.org/  
    • RareShare is an online social hub dedicated to patients, families and healthcare professionals affected by rare medical disorders.
 
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    • RDCRN 3rd Conference on Clinical Research for Rare Diseases, October 2, 2012
      Location: Hilton Washington DC/Rockville Hotel & Executive Meeting Center, Rockville, MD
      Description: Clinical research in rare diseases presents a number of challenges and unique issues that are not usually considered in the training of clinical investigators through existing training programs. Goals of the conference include: direct instruction of trainees and new investigators in rare disease research methodology; development of a reusable curriculum/syllabus on rare disease research methodology; and stimulation of ideas regarding the unique issues facing investigators engaged in the study of rare diseases.
 
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