Proposal for an algorithm for liver transplantation in Caroli's disease and syndrome: putting an uncommon effort into a common task

Abstract

Liver transplantation (LT) represents an uncommon indication for Caroli's disease (CD) or syndrome (CS). Excellent results of LT have been reported as shown by recent multicentric European and American registry reports. Clear therapeutic flowcharts to adopt in these diseases are still lacking. This review aims at analyzing highlighting recent transplant experiences in this field and also at focusing on the role of LT in case-specific comorbidities such as development of cholangiocellular cancer or renal failure are present.

Keywords: Caroli's disease; Caroli's syndrome; autosomal recessive polycystic kidney disease; cholangiocellular cancer; combined kidney-liver transplantation; congenital hepatic fibrosis; liver transplantation.