Massive polycystic liver disease in autosomal-dominant polycystic kidney disease

Polycystic liver diseases (PLDs) are a rare group of genetic disorders, in which multiple cysts occur in the liver. This could occur on a background of cystic kidneys, known as autosomal-dominant polycystic kidney disease (ADPKD) or without the presence of cysts in any other organ of the body, known as autosomal-dominant PLD. Irrespective of the type of the polycystic liver, the natural course of these disorders is similar. Both genders are affected, but the female gender has a higher prevalence. Majority of patients with PLD are asymptomatic and can be managed conservatively, but a minority could develop massive hepatomegaly and attendant symptoms from compression of the surrounding organs. We present a patient with massive polycystic liver on a background of ADPKD.