title: The place of liver transplantation in Caroli's disease and syndrome.
abstract: Caroli's disease (CD) or syndrome (CS) are rare inherited disorders which may cause severe, life-threatening, cholangitis or which may lead to hepatobiliary degeneration. The typical cystic biliary anomalies are often associated to congenital hepatic fibrosis (CHF) and, less frequently, to cystic renal disease especially autosomic recessive polycystic kidney disease (ARPKD). The place of liver transplantation (LT) in the treatment of CD or CS is evaluated based on our own experience of three successfully transplanted patients, the literature review of 19 patients and the European experience with 110 patients collected in the European Liver Transplant Registry. LT should be proposed as a definitive therapeutic option once severe cholangitis or (suspicion of) malignant transformation is present. The frequently used radiological, endoscopical or surgical biliary drainage procedures carry a high morbidity and mortality rate. In case of concomitant symptomatic CHF and renal failure, combined or sequential hepatorenal transplantation should be carried out, dependent on the evolution of the hepatic and renal disease. In case of associated ARPKD, renal transplantation is often indicated early on because of the more rapid progression of the renal component of the disease.
authors.affiliation: Abdominal Transplantation Unit, Université Catholique de Louvain, UCL, Brussels, Belgium.
authors.forename: Laurent
authors.initials: L
authors.lastname: De Kerckhove
authors.forename: Martine
authors.initials: M
authors.lastname: De Meyer
authors.forename: Catherine
authors.initials: C
authors.lastname: Verbaandert
authors.forename: Michel
authors.initials: M
authors.lastname: Mourad
authors.forename: Etienne
authors.initials: E
authors.lastname: Sokal
authors.forename: Pierre
authors.initials: P
authors.lastname: Goffette
authors.forename: Andre
authors.initials: A
authors.lastname: Geubel
authors.forename: Vincent
authors.initials: V
authors.lastname: Karam
authors.forename: Rene
authors.initials: R
authors.lastname: Adam
authors.forename: Jan
authors.initials: J
authors.lastname: Lerut
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016767:Caroli Disease / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007674:Kidney Diseases / Q000628:therapy; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000628:therapy; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged