The following information may help to address your question:
What is Caroli disease?
is a rare disorder characterized by abnormal widening of the large intrahepatic bile ducts.
It may present with recurrent bacterial cholangitis
, biliary stones causing biliary pain or episodes of pancreatitis
. Age of onset is variable and mainly females are affected. The more common variant of this disease, Caroli syndrome, is characterized by dilations of the large bile duct in association with congenital hepatic fibrosis. Patients with Caroli syndrome may present with signs and symptoms of portal hypertension
, bacterial cholangitis
, esophageal varices
, and gastrointestinal hemorrhage. Caroli syndrome may be found in association with autosomal recessive polycystic kidney disease
, autosomal dominant polycystic kidney disease
, medullary sponge kidney
, and medullary cystic disease
Caroli disease may be sporadic or autosomal dominant
, whereas Caroli syndrome is generally transmitted in an autosomal recessive
Last updated: 5/19/2011
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 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.
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 prognosis for individuals with Caroli disease?
Prognosis for individuals with Caroli disease 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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