Polycystic Liver Disease

Polycystic liver disease (PCLD) is characterized by the presence of numerous (>20) hepatic cysts similar in structure to simple cysts, but usually larger. It can occur alone (best prognosis; usually asymptomatic) or coexist with polycystic kidney disease—both with the autosomal dominant form (the most common form of PCLD; see Autosomal Dominant Polycystic Kidney Disease (ADPKD)) and the autosomal recessive form (the least common form).

Treatment should be considered in the case of symptomatic cysts (percutaneous aspiration, surgical aspiration). Benefits of using somatostatin analogues and mTOR inhibitors (eg, sirolimus) have been reported. In patients with severe symptoms and a large number of cysts, liver transplant may be considered, sometimes simultaneously with kidney transplant.