Introduction: To recognize clinical manifestations of type II Mirizzi syndrome (MS) in a patient with porcelain gallbladder. Case Report: A 64-year-old female presented with 3 weeks of abdominal discomfort, jaundice, clay colored stools, pruritus, dark urine, and weight loss. Abdomen was soft and non-tender. Laboratory studies were significant for AST 83 U/L, ALT 26 U/L, alk phos 468 U/L, and bilirubin 19.2 mg/dL. CT abdomen showed dilatation of intrahepatic ducts and common bile duct (CBD) with abrupt transition to normal. No stones were visualized but a calcified cystic lesion adjacent to the left lobe of the liver was seen (Figure 1). An ERCP done showed a 16-mm stone in the middle of CBD. Cystic duct was small but a fistula connecting the gallbladder to extrahepatic ducts at the bifurcation of common hepatic duct was identified (Figure 2). Initial attempts to perform mechanical lithotripsy using a basket were unsuccessful as the basket broke. Stent was placed for decompression and ERCP with EHL was repeated in 4 weeks. Complete stone removal was accomplished with clinical and chemical resolution of biliary obstruction.Figure 1: CT of the abdomen showing a red arrow marking the classic image of a porcelain gallbladder.Figure 2: ERCP image showing CBD stone and a type II Mirizzi syndrome.Discussion: Type II MS is characterized by obstructive picture and a fistulous connection between the gallbladder and the biliary tree involving less than one-third of the circumference of the duct wall. Patient can be managed endoscopically. Our patient was also found to have a porcelain gallbladder, which is rare complication of chronic cholecystitis and is associated with cholangiocarcinoma (2%; incomplete type > complete type). Cholecystectomy is advocated by most.
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