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Old 03-31-2011
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Liver choledochal cyst and caroli syndrome

what is the difference between Caroli syndrome and Choledochal cyst type 5 according to uw?!
UW said both of them has intrahepatic biliary ducts dilation!!!
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Old 04-01-2011
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According to UpToDate, the terminology of "choledochal cysts" was changed to "biliary cysts" in 1977 to acknowledge the occurence of intrahepatic cysts in addition to the cysts of the extrahepatic bile ducts. At that time, they also changed the classification system to include Type V (intrahepatic cysts).

Type V cysts account for 20 percent of biliary cysts. They are characterized by one or more cystic dilations of the intrahepatic ducts, without extrahepatic duct disease. The presence of multiple saccular or cystic dilations of the intrahepatic ducts is known as Caroli's disease.

Caroli's disease is a congenital disorder characterized by multifocal, segmental dilatation of large intrahepatic bile ducts. The condition is usually associated with renal cystic disease of varying severity. Caroli initially described two variants, which has led to some confusion in terminology.

Caroli's disease is the less common form and is characterized by bile ductular ectasia without other apparent hepatic abnormalities.

The more common variant is Caroli's syndrome, in which bile duct dilatation is associated with congenital hepatic fibrosis.

Caroli's disease and syndrome have been described in the same family. Most cases are transmitted in an autosomal recessive fashion and are associated with autosomal recessive polycystic kidney disease (ARPKD).

(Taken from UpToDate)

Does that help?
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Old 04-01-2011
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thank you so much healer2b!you are so kind
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