2365 A Rare Case of Severe Primary Polycystic Liver Disease

Abstract

INTRODUCTION: Polycystic Liver Disease (PLD) is found mainly in association with autosomal dominant polycystic kidney disease (ADPKD). Isolated PLD is rare with a prevalence of less than 0.01%. Massive hepatomegaly causing symptoms associated with PLD is even rarer. We present a patient with severe PLD requiring surgical intervention. CASE DESCRIPTION/METHODS: A 55-year-old man had an incidental finding of multiple liver cysts on an ultrasound done for investigation of a superficial abdominal wall mass. He reported left upper quadrant pressure. Two siblings had hepatic cysts but no family history of kidney cysts. He had been in South Korea and central America in the past. Physical exam showed hepatomegaly, splenomegaly and a bulge in the right upper quadrant (Figure 1). Hepatic synthetic, excretory function and aminotransferases were normal. CT abdomen revealed too numerous to count hepatic cysts, some measuring up to 11cm in diameter, renal cysts and a 1.4 cm splenic cyst. MRI showed numerous intrahepatic cysts, measuring up to 14 cm, a small number of bilateral renal cysts and splenic cyst (Figure 2). AFP, Echinococcus IgG and chronic hepatitis panel were negative. He did not met criteria for ADPKD as the renal his cysts were simple and age-related according to nephrology. He reported worsening symptoms abdominal pressure and postprandial pain. He underwent laparoscopic fenestration of liver cysts and specimens were consistent with simple hepatic cysts. DISCUSSION: PLD is a rare condition that is often asymptomatic. At least one-third of PLD may also have a few kidney cysts that do not meet criteria for ADPKD. Gigot’s classification is based on imaging findings and can used to help determine management choice based on total liver involvement, cyst size and number (Table 1). Asymptomatic PLD patients do not require treatment. Symptomatic patients may be treated medically with somatostatin analogues or surgically including fenestration +/- hepatic resection or liver transplantation. Severe PLD causing bulging on the abdomen is rare. Our patient benefited from fenestration.