Polycystic liver disease presenting as pruritus
A 52-year-old female presented with generalized grade three pruritus of six months duration. There was no history of any skin lesions, fever, abdominal pain, jaundice or fatigue. Neither did she have any history of leg swelling, facial puffiness, decreased urine output or gastrointestinal bleed. She was detected to be hypertensive one year back for which Amlodipine was prescribed. Examination revealed a 5 cm hepatomegaly more in the epigastric region along with separate masses in both lumbar regions which were bimanually palpable and ballotable. Liver function test revealed normal bilirubin and aminotransferases but a more than three times elevation of serum alkaline phosphatase and γ-glutamyl transferase. Renal function was normal and there was no proteinuria.\nInitial ultrasound scan was followed by CT scan of abdomen () which showed multiple noncommunicating cysts of varying size in both lobes of liver. There was mild dilation of intrahepatic biliary radicles in both lobes. Common hepatic duct and common bile duct (CBD) appeared normal. Both kidneys were bulky with multiple noncommunicating cysts.\nMRI abdomen () with MRCP () showed multiple T2WI high signal parenchymal cysts mostly in the left lobe of liver, the largest measuring 9.8 × 6.7 cm. Mild dilation of intrahepatic bile ducts was seen. A few small cysts in communication with the dilated bile radicles were also noted. The proximal CBD was not visualized due to compression by the cysts at the porta hepatis. The distal CBD was normal in caliber. Spleen and pancreas were normal. Both kidneys were markedly enlarged with parenchyma totally replaced by innumerable cortical cysts, the largest measuring 7×5.6 cm. The large cysts in the left lobe of liver and porta hepatis compressing the bile ducts were not communicating with the biliary system. A diagnosis of polycystic liver disease with Caroli’s disease and biliary obstruction was made. The patient also had polycystic kidney disease. Hence our patient presented for the first time with longstanding pruritus as the only manifestation of polycystic liver disease.\nThe patient was planned for laparoscopic deroofing of liver cysts. However she was lost to follow up.