Intrahepatic biliary cysts in congenital biliary atresia. 1989

J S Fain, and K J Lewin
Department of Pathology, UCLA Medical Center 90024-1732.

A case of pronounced cystic dilatation of the intrahepatic bile ducts with biliary atresia is presented. Intrahepatic biliary cysts have been described in biliary atresia, although, as far as we are aware, none as extensive as in this case. The cysts represent end-stage obstruction with irreversible liver injury; thus, drainage of these cysts does not alleviate the condition. The differential diagnosis includes Caroli's disease, congenital hepatic fibrosis, and polycystic liver disease.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008105 Liver Cirrhosis, Biliary FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes. Biliary Cirrhosis,Biliary Cirrhosis, Primary,Biliary Cirrhosis, Secondary,Cholangitis, Chronic Nonsuppurative Destructive,Liver Cirrhosis, Obstructive,Primary Biliary Cholangitis,Biliary Cirrhosis, Primary, 1,Primary Biliary Cirrhosis,Secondary Biliary Cholangitis,Secondary Biliary Cirrhosis,Biliary Cholangitides, Primary,Biliary Cholangitis, Primary,Biliary Cholangitis, Secondary,Cholangitides, Primary Biliary,Cholangitis, Primary Biliary,Cholangitis, Secondary Biliary,Cirrhosis, Biliary,Cirrhosis, Secondary Biliary,Liver Cirrhoses, Biliary,Obstructive Liver Cirrhosis,Primary Biliary Cholangitides,Secondary Biliary Cholangitides
D008297 Male Males
D002780 Cholestasis, Intrahepatic Impairment of bile flow due to injury to the HEPATOCYTES; BILE CANALICULI; or the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). Bile Duct Obstruction, Intrahepatic,Biliary Stasis, Intrahepatic,Intrahepatic Cholestasis,Biliary Stases, Intrahepatic,Cholestases, Intrahepatic,Intrahepatic Biliary Stases,Intrahepatic Biliary Stasis,Intrahepatic Cholestases
D003560 Cysts Any fluid-filled closed cavity or sac that is lined by an EPITHELIUM. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues. Cyst
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001656 Biliary Atresia Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE. Atresia, Biliary,Biliary Atresia, Extrahepatic,Biliary Atresia, Intrahepatic,Extrahepatic Biliary Atresia,Familial Extrahepatic Biliary Atresia,Idiopathic Extrahepatic Biliary Atresia,Intrahepatic Biliary Atresia,Atresia, Extrahepatic Biliary,Atresia, Intrahepatic Biliary
D001660 Biliary Tract Diseases Diseases in any part of the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER. Biliary Tract Disease,Disease, Biliary Tract,Diseases, Biliary Tract,Tract Disease, Biliary,Tract Diseases, Biliary

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