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Diseases

Genetic and Rare Diseases Information Center (GARD)

Caroli disease


Other Names for this Disease
  • Congenital polycystic dilatation of intrahepatic bile ducts
  • Caroli disease isolated
  • Cystic dilatation of the intrahepatic biliary tree
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 Caroli disease be treated?

The management of Caroli disease depends on the clinical presentation, localization, and stage of the disease.[1] Conservative treatment may include supportive care with antibiotics for cholangitis and ursodeoxycholic acid for gallstones. Surgical resection has been used successfully in patients with monolobar disease. For patients with diffuse involvement, the treatment of choice is liver transplantation.[1][2]

Additional information regarding treatment of Caroli disease can be accessed through the following emedicine links: http://emedicine.medscape.com/article/927248-treatment#showall
http://emedicine.medscape.com/article/927248-medication#showall 

Medical journal articles that discuss the treatment of Caroli disease can be found through PubMed, a searchable database of biomedical journal articles. Although not all of the articles are available for free online, most articles listed in PubMed have a summary available. To obtain the full article, contact a medical/university library or your local library for interlibrary loan. You can also order articles online through the publisher’s Web site. Using "Caroli disease AND treatment" as your search term should help you locate articles. Use the advanced search feature to narrow your search results. Click here to view a search.
http://www.ncbi.nlm.nih.gov/PubMed

The National Library of Medicine (NLM) Web site has a page for locating libraries in your area that can provide direct access to these journals (print or online). The Web page also describes how you can get these articles through interlibrary loan and Loansome Doc (an NLM document-ordering service). You can access this page at the following link http://nnlm.gov/members/. You can also contact the NLM toll-free at 888-346-3656 to locate libraries in your area.
Last updated: 6/9/2011

References
  1. Caroli disease. Orphanet. October 2006; http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=10715&Disease_Disease_Search_diseaseGroup=Caroli-disease&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Caroli-disease&title=Caroli-disease&search=Disease_. Accessed 5/18/2011.
  2. Ananthakrishnan AN, Saeian K. Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 2007; http://www.ncbi.nlm.nih.gov/pubmed/17418061. Accessed 5/19/2011.


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

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Caroli disease. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".
Other Names for this Disease
  • Congenital polycystic dilatation of intrahepatic bile ducts
  • Caroli disease isolated
  • Cystic dilatation of the intrahepatic biliary tree
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.