Liver and intrahepatic bile ducts-nontumor
Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 9 April 2012, last major update April 2012-IN PROGRESS
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● Also called communicating cavernous biliary ectasia
● Autosomal recessive disorder, mildly associated with autosomal dominant and autosomal recessive polycystic kidney disease (Wikipedia)
● Due to arrest of remodeling of ductal plate of larger intrahepatic bile ducts
● Usually presents during childhood and early adulthood (Curr Gastroenterol Rep 2007;9:151)
● Larger ducts of intrahepatic biliary tree are segmentally dilated and may contain inspissated bile; when progressive, leads to intrahepatic stones, recurrent cholangitis, portal hypertension, cholangiocarcinoma, and liver failure
● Usually associated with congenital hepatic fibrosis; 7-14% develop dysplasia and cholangiocarcinoma
● Often gallstones, ulcer, hyperplasia
● Poor prognosis, with death due to sepsis or liver failure
● Caroliís syndrome: Caroliís disease plus congenital hepatic fibrosis
● Jaundice, right upper abdominal pain, fever; due to associated complications of hepatolithiasis or bacterial cholangitis
● 1-4 cm cysts separated by normal bile ducts
Dilated bile ducts
● Dilated ducts lined by cuboidal or columnar epithelium with fibrotic duct wall
Dilated portal bile ducts (trichrome stain), with inspissated bile, with congenital hepatic fibrosis
● Primary sclerosing cholangitis: autoimmune disorder associated with inflammatory bowel disease; not inherited; fibrosing cholangitis with onion skinning around affected ducts
End of Liver and intrahepatic bile ducts-nontumor > Developmental anomalies/cysts > Caroli's disease
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