Congenital hepatic fibrosis with dilation of intrahepatic bile ducts. A therapeutic approach.
A case of congenital hepatic fibrosis with dilation of the intrahepatic ducts is presented. External drainage and lavage of the biliary tract through a prosthetic "Y" tube and internal drainage accomplished by a Roux en-Y cholangiojejunostomy resulted in prevention of the patient's recurrent bouts of cholangitis and sepsis. Surgical therapy for recurrent cholangitis in this disease is reviewed.
D001653:Bile Ducts, Intrahepatic / Q000601:surgery; D001660:Biliary Tract Diseases / Q000601:surgery; D002758:Cholangiography; D002761:Cholangitis / Q000601:surgery; D002769:Cholelithiasis / Q000601:surgery; D004108:Dilatation, Pathologic / Q000601:surgery; D004322:Drainage; D005260:Female; D042882:Gallstones / Q000601:surgery; D006801:Humans; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000151:congenital* / Q000601:surgery; D008111:Liver Function Tests; D008875:Middle Aged; D007507:Therapeutic Irrigation