Polycystic liver disease

Polycystic liver disease encompasses different entities characterized by the presence in the liver of several fluid collections, at least 10, of serous type, limited by a unicellular base of cuboid or cylindrical cells, similar to biliary epithelium but not communicating with the bile duct. The architecture of the biliary tree is thus preserved. Von Meyenburg biliary or complex hamartomas correspond to a congenital malformation of the ductal plate. The three other pathologies are genetic diseases involving the middle-size biliary ducts: autosomal recessive polycystic liver and kidney disease, which is a pediatric pathology, and two pathologies found in adults: autosomal dominant polycystic liver and kidney disease and isolated polycystic liver disease. This review, mainly dedicated to the two genetic polycystic diseases in adults, is based on the recent recommendations of the European Association for the Study of the Liver [1].