Preoperative Detection of Malignant Transformation in a Choledochal Cyst

Abstract

1 A female patient presented with jaundice and upper abdominal pain. Physical examination revealed cleral icterus and right upper quadrant abdominal tenderness. aboratory investigations were significant for total bilirubin of .7 mg/dL (normal, 0.2–1.3 mg/dL) and alkaline phosphatase of 89 U/L (normal, 38 –126 U/L). A computed tomogram of the bdomen revealed a dilated common bile duct (CBD) of 6 cm ith distal bile duct narrowing (Figure A). An endosopic ultraound examination to rule out underlying malignancy revealed CBD of 7 cm with large amount of sludge and no mass lesions Figure B). Endoscopic retrograde cholangiopancreatography as then undertaken for evaluating the bile duct wall layers by sing intraductal ultrasound and to decompress the CBD. At ndoscopic retrograde cholangiopancreatography, the patient as diagnosed to have a type I choledochal cyst (Figure C), and here was no evidence of anomalous pancreaticobiliary juncion. Intraductal ultrasound revealed papillary projections in he bile duct wall consistent with malignant transformation Figure D). A biliary sphincterotomy was undertaken with rainage of black bile. The patient underwent bile duct resection (Figure E) and epaticojejunostomy. Histopathology revealed flat and papilary biliary intraepithelial neoplasia with multiple foci of highrade dysplasia but without deeper invasion (Figure F). Because he proximal left bile duct margin revealed focal epithelial typia, the patient subsequently underwent left hepatic lobecomy with intrahepatic biliary-enteric reconstruction. The paient had an uneventful postoperative course and was disharged home 10 days later. Choledochal cysts are rare congenital anomalies occurring in in 100,000 –150,000 live births in the United States.1 There is