title: [Curative partial hepatectomy in adult Caroli's disease].
abstract: Caroli's disease consists of a congenital malformation of the intrahepatic bile ducts characterized by saccular, segmental, cystic dilatations giving rise to recurrent cholangitis. The inheritance pattern is unclear and the disease may be associated with other hereditary diseases such as adult polycystic kidney disease. The most effective therapeutic option in symptomatic unilobar Caroli's disease is resection of the affected lobe. In bilobar involvement, liver transplantation should be considered. Due to their rarity, we report three cases of Caroli's disease successfully resolved through lobectomy of the affected lobe: two patients with recurrent cholangitis and one patient with associated adult polycystic kidney disease.
authors.affiliation: Sección de HBP-Oncológica, Servicio de Cirugía General y Digestiva, Hospital de la Santa Creu i Sant Pau, UAB, Barcelona, España. rmedrano@santpau.es
authors.forename: Rodrigo
authors.initials: R
authors.lastname: Medrano-Caviedes
authors.forename: Vicente
authors.initials: V
authors.lastname: Artigas
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: Sancho
authors.forename: Guillermo
authors.initials: G
authors.lastname: Marín-Hargreaves
authors.forename: Manuel
authors.initials: M
authors.lastname: Rodríguez
authors.forename: Manuel
authors.initials: M
authors.lastname: Trías
mesh_terms: D000368:Aged; D016767:Caroli Disease / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006498:Hepatectomy / Q000379:methods*; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome