Polycystic Liver Disease

Polycystic liver disease (PLD) is an inherited disorder estimated to affect around 1 in 100,000 people. It is characterized by the progressive growth of cysts of various sizes scattered throughout the liver. People affected by this condition tend to have more and larger cysts as they age and usually start to have symptoms around age 50, although symptoms can begin to occur earlier. However, many affected individuals do not have symptoms. Enlargement of the liver (hepatomegaly) can cause abdominal pain and discomfort, shortness of breath (dyspnea), early satiety and gastro-esophageal reflux. Rare complications are hepatic cyst hemorrhage, infection or rupture. Surgical and medical treatment is available to manage the symptoms, but the only definitive treatment for this condition is liver transplant. Most cases are inherited in an autosomal dominant pattern, but some cases seem to occur with no apparent cause (sporadically). Sometimes, cysts are found in the liver in association with the presence of autosomal dominant polycystic kidney disease (ADPKD). In fact, most people who have ADPKD have liver cysts.