Diseases & Conditions


A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

Caroli Disease


Synonyms of Caroli Disease
  • Congenital Dilatation of Intrahepatic Bile Duct

Disorder Subdivisions



    General Discussion
    Caroli disease is a rare inherited disorder characterized by abnormal widening (dilatation) of the ducts that carry bile from the liver (intrahepatic bile ducts). According to the medical literature, there are two forms of Caroli disease. In most cases, the isolated or simple form is characterized by widening of the bile ducts (dilatation or ectasia). A second, more complex form is often called Caroli syndrome. The complex form or syndrome is associated with the presence of bands of fibrous tissue in the liver (congenital hepatic fibrosis) and high blood pressure in the portal artery (portal hypertension. This form of Caroli disease is also often associated, in ways that are not well understood, with polycystic kidney disease, and, in severe cases, liver failure.

    The genetics of Caroli disease are complex as well. The isolated or simple form is transmitted as an autosomal dominant trait, while the complex form associated with polycystic kidney disease is transmitted as an autosomal recessive trait.

    Organizations related to Caroli Disease
    • ARPKD/CHF Alliance
      PO Box 70
      Kirkwood PA 17536
      Phone #: 717-529-5555
      800 #: 800-708-8892
      e-mail: info@arpkdchf.org
      Home page: http://www.arpkdchf.org
    • American Liver Foundation
      75 Maiden Lane
      New York NY 10038
      Phone #: 212-668-1000
      800 #: 800-465-4837
      e-mail: info@liverfoundation.org
      Home page: http://www.liverfoundation.org
    • Madisons Foundation
      PO Box 241956
      Los Angeles CA 90024
      Phone #: 310-264-0826
      800 #: N/A
      e-mail: getinfo@madisonsfoundation.org
      Home page: http://www.madisonsfoundation.org
    • NIH/National Digestive Diseases Information Clearinghouse
      2 Information Way
      Bethesda MD 20892-3570
      Phone #: 301-654-3810
      800 #: 800-891-5389
      e-mail: nddic@info.niddk.nih.gov
      Home page: http://www.niddk.nih.gov



    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html