Analysis of multiple invasive and non invasive therapeutic options for polycystic livers. Call for a Yielding Single Treatment Strategy.

This thesis describes studies on the treatment of polycystic livers. Polycystic livers are characterized by the presence of numerous cysts scattered throughout the whole liver parenchyma. This phenotype occurs in the setting of two inherited diseases: either in combination with polycystic kidneys: autosomal dominant polycystic kidney disease (ADPKD); or in an isolated form: autosomal dominant polycystic liver disease (PCLD). The main aim of this thesis is to evaluate the available therapeutic options for polycystic livers in terms of effect of treatment and best treatment strategy. Furthermore, identification of new non-invasive treatment was studied. The phenotype of PCLD is described by the characterization of the largest cohort in literature of patients with PCLD. The main aim of treatment is reduce total liver volume. Various invasive therapeutic options are described: Aspiration-sclerotherapy, which is the less invasive therapeutic option, and is performed in patients with large liver cysts; Laparoscopic fenestration is a more invasive therapeutic option and patients with more large (>5cm) cysts are suitable for this therapeutic option; Liver transplantation is the only curative treatment, but should be only performed in the most severe courses. The first studies upon non-invasive therapeutic options are described. Treatment with lanreotide, a somatostatin analogue, resulted in a liver volume reduction of 2.9% and kidney volume reduction of 1.5%, instead of an increase of liver volume of 1.6% and an increase of kidney volume of 3.6%. In addition, patients treated with lanreotide had an increase of current health perception, which did not apply to patients on placebo. Treatment with octreotide resulted in a volume reduction as well. Finally, all invasive therapeutic options are reviewed and the current knowledge on non-invasive therapeutic options are described. In addition, we give a flow chart for the various treatment options