Polycystic liver disease

Polycystic liver disease (PLD) is a condition in which multiple cysts are scattered throughout otherwise normal liver tissue. It is commonly seen together with autosomal dominant polycystic kidney disease, which has a prevalence of about 1 in 400 to 1000, while a much rarer autosomal dominant form affects the liver alone and progresses without kidney involvement. Associations with the genes PRKCSH and SEC63 have been described. Most people have no symptoms, and the cysts are often found by chance on imaging. When symptoms do appear they usually come from the size and number of the cysts pressing on nearby organs, and include abdominal distension, discomfort in the upper right abdomen, early satiety, heartburn and shortness of breath. Severe cases can lead to portal hypertension, bile duct obstruction with jaundice, or compression of the inferior vena cava, and cyst bleeding, infection or rupture are rare. Diagnosis rests on imaging, usually CT or MRI, with liver function tests that are often normal. Treatment is reserved for people with pain or complications, aims to shrink the cysts while protecting working liver tissue, and can involve surgical removal or aspiration sclerotherapy.