[Gastrointestinal surgery and gastroenterology. XV. Polycystic liver disease]

Polycystic liver disease is a congenital disorder with an autosomal dominant pattern of inheritance. There are two variants. In one, liver cysts occur as an extra-renal phenomenon in autosomal dominant polycystic kidney disease. In the other, the disease is not associated with renal pathology. The cysts have to reach a certain diameter before becoming clinically manifest and therefore are generally only detected many years after birth. Polycystic liver disease causes symptoms in 20% of the patients. These symptoms are the consequence of the weight and size of the liver or of complications such as abscess formation, obstructive jaundice or the Budd-Chiari syndrome. Most symptoms can be treated at least temporarily by fenestration of the larger cysts and resection of parts of the liver, which are affected by large groups of small cysts. A permanent cure of the disease is only obtained by liver transplantation.