The following information may help to address your question:
What is 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).
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
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.
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.
Conservative treatment may include supportive care with antibiotics
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.
Additional information regarding treatment of Caroli disease can be accessed through the following emedicine links: http://emedicine.medscape.com/article/927248-treatment#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.
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
Last updated: 5/19/2011
We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please
GARD Information Specialist
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