title: Adult presentation of Caroli's syndrome treated with orthotopic liver transplantation.
abstract: When Caroli's disease, defined as a congenital dilation and ectasia of segmental intrahepatic bile ducts in the absence of other histological abnormalities, is associated with periportal fibrosis, it is termed Caroli's syndrome. We describe the case of a 35-yr-old woman with Caroli's syndrome without clinical manifestation of portal hypertension despite diffuse involvement of the liver who was successfully treated with orthotopic liver transplantation after recurrent nearly fatal episodes of cholangitis.
authors.affiliation: Department of Medicine, Mount Sinai Medical Center, New York, New York, USA.
authors.forename: T D
authors.initials: TD
authors.lastname: Schiano
authors.forename: M I
authors.initials: MI
authors.lastname: Fiel
authors.forename: C M
authors.initials: CM
authors.lastname: Miller
authors.forename: H C
authors.initials: HC
authors.lastname: Bodenheimer
authors.forename: A D
authors.initials: AD
authors.lastname: Min
mesh_terms: D000328:Adult; D016767:Caroli Disease / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation