1373 A Rare Case of Biliary Obstruction in Autosomal Dominant Polycystic Kidney Disease

Abstract

INTRODUCTION: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common genetic disease in adults. We present a rare case of biliary obstruction due to polycystic disease affecting the kidney and liver. CASE DESCRIPTION/METHODS: A 18 year old female patient with polycystic disease of the liver and kidney diagnosed in childhood, presented with new onset jaundice and abdominal pain radiating to the back. Initial labs suggested a predominantly cholestatic patter of liver injury with total bilirubin 3.1 mg/dl with a direct bilirubin of 2.3 mg/dl, aspartate aminotransferase 96 U/L, alanine aminotransferase 177 U/L, and alkaline phosphatase 237 international U/L. Lipase was noted to be 443 U/L. The patient did not exhibit signs of cholangitis and was started on intravenous fluids at 2 ml/kg/hr after a bolus of 2 L. The platelets were noted to be 123 with an INR of 1.3. A Magnetic Resonance Cholangiopancreatography showed near-complete replacement of hepatic, bilateral renal and pancreatic parenchyma with greater than 100 simple cysts. Also noted was dilatation of the common bile duct measuring up to 16 mm (Figure1). No internal filling defect was noted. An ERCP was performed. During endoscopy, multiple luminal “bulges” representing extrinsic compression of cysts were noted in the stomach and duodenal. A native ampulla was identified in the second portion and was displaced laterally by extrinsic luminal compression (Figure 2). Cholangiogram revealed dilated CBD and CHD with cystic dilations of the intrahepatic ducts more pronounced in the left lobe of the liver. There appears to be contrast seen in several liver cysts indicating possible communication of biliary tree with the cysts (Figure 3). We entertained the possibility of a choledochal cyst (4a). External compression of the intraduodenal CBD was the etiology of the obstruction and a 10 Fr × 10 cm plastic biliary stent was placed resulting in large volume of bile evacuating from the biliary tree. The placement of the biliary stent resulted in the normalization of total bilirubin to 0.5 mg/dl with, AST 23 U/L, ALT 24 U/L, and ALP 109 international U/L. We recognized the risk of cholangitis due to the stasis of contrast and provided Ceftriaxone 1 g IV intra procedure and Cephalexin once daily for 7 days. DISCUSSION: This patient with large number of hepatic cysts replacing the hepatic parenchyma and large renal cysts resulted in distal biliary compression in the intraduodenal portion. This patient is being considered for a liver kidney transplantation.