title: Liver transplantation in a 7-month-old girl with Caroli's disease.
abstract: Caroli's disease (including Caroli's syndrome) is a rare autosomal recessive disorder of the liver characterized by diffuse cystic dilatation of the intrahepatic bile ducts. The disease may present at any age and is characterized by recurrent episodes of biliary obstruction, cholangitis, hepaticolithiasis, and liver abscesses. Caroli's syndrome is further associated with congenital hepatic fibrosis and portal hypertension. Patients with recurrent complications or cirrhosis may die because of recurrent infection, portal hypertension, liver failure, or cholangiocarcinoma. Liver transplantation is the treatment of choice for these complicated patients. Here we describe the youngest reported patient with Caroli's syndrome treated successfully using liver transplantation and review the recent literature.
authors.affiliation: Department of Surgery, Section of Transplantation and Hepatobiliary Surgery, University of Utah School of Medicine, Salt Lake City, UT 84132, USA. robin.kim@hsc.utah.edu
authors.forename: Robin D
authors.initials: RD
authors.lastname: Kim
authors.forename: Linda
authors.initials: L
authors.lastname: Book
authors.forename: Allah
authors.initials: A
authors.lastname: Haafiz
authors.forename: Jason J
authors.initials: JJ
authors.lastname: Schwartz
authors.forename: John B
authors.initials: JB
authors.lastname: Sorensen
authors.forename: Regino P
authors.initials: RP
authors.lastname: Gonzalez-Peralta
mesh_terms: D016767:Caroli Disease / Q000175:diagnosis / Q000601:surgery*; D002648:Child; D005260:Female; D006801:Humans; D016031:Liver Transplantation