The following information may help to address your question:
What is Caroli disease?
Caroli disease is a condition characterized by an abnormal widening of the intrahepatic bile ducts (the ducts that carry bile from the liver) and renal cysts. People affected by this condition experience recurrent episodes of
cholestasis, stone development in the bile ducts, and bacterial
cholangitis. In addition to the symptoms of Caroli disease, people affected by Caroli
syndrome may also experience
liver fibrosis and
portal hypertension (
high blood pressure of the portal vein).
[1][2][3] Although the underlying cause of Caroli disease and Caroli syndrome are poorly understood, they are thought to be genetic conditions. Caroli disease generally occurs sporadically in people with no
family history of the condition; however, rare reports exist of
autosomal dominant inheritance in association with
autosomal dominant polycystic kidney disease.
[4][1] Caroli syndrome is generally
inherited in an
autosomal recessive manner and is frequently seen in association with
autosomal recessive polycystic kidney disease. Treatment is based on the signs and symptoms present in each person.
[1][2]
Last updated: 7/19/2017
How might Caroli disease be treated?
The management of Caroli disease depends on the clinical presentation, localization, and stage of the disease.
[5] 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.
[5][6]
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
What is the long-term outlook for people with Caroli disease?
The long-term outlook for people with Caroli disease and Caroli syndrome is variable and determined by the frequency and severity of the episodes of
cholangitis; the presence of associated diseases; and the increased risk of bile duct
cancer.
[1]
Last updated: 5/19/2011
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GARD Information Specialist
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