Caroli's Disease is one of the congenital cystic bile duct (CCBD) disorders.
Mechanism: ductal plate malformation of the major intrahepatic ducts during early bile duct embryogenesis
Features:
(1) congenital cystic dilatation of one or more intrahepatic bile ducts with formation of choledochal cysts
(2) variable congenital hepatic fibrosis and/or renal cystic disease
Pattern
Type
Autosomal Inheritance
CCBD alone
simple
dominant
with hepatic fibrosis or renal cystic disease
complex (Caroli's Syndrome)
recessive
Complications:
(1) cholangitis
(2) intrahepatic biliary stones
(3) adenocarcinoma
(4) portal hypertension with bleeding varices (with hepatic fibrosis)
(5) sepsis
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Specialty: Gastroenterology, Genetics
