Type I Choledochal Cyst with Abnormal Union of the Pancreaticobiliary Junction Presenting with Ruptured Gallbladder Carcinoma

Abstract

A 43-year-old female presented with nausea, vomiting, fever, hypotension, tachycardia and right upper quadrant abdominal pain. Laboratory values revealed leukocytosis and cholestasis. Transabdominal ultrasound showed a thickened gallbladder wall (2 cm) without surrounding fluid as well as a markedly dilated (2 cm) extrahepatic biliary system. Abdominal CT revealed a dilated extrahepatic biliary duct and additionally, a large poorly defined hypodensity in liver segment V suggestive of gallbladder rupture or focal abscess. ERCP was performed to further characterize the patient's anatomy and process prior to surgical intervention. ERCP demonstrated fusiform dilation of the extrahepatic biliary system of up to 2.8 cm suggestive of a type 1A choledochal cyst. Contrast injection of the gallbladder revealed an irregular wall with contained contrast extravasation into the hepatic parenchyma suspicious for gallbladder cancer with associated rupture. Cholangiogram of the distal biliary system revealed a smoothly tapering common bile duct proximal to the junction with the pancreatic duct without any stricture. Aside from mild tortuosity in the head, the pancreatogram was normal. A borderline abnormal pancreaticobiliary junction with a 10 mm long common channel was appreciated distally. A double pigtail biliary stent was placed across the papilla and into the proximal dilated portion of the bile duct. As a result of the cholangiogram, CT scan and intra-operative findings, the patient underwent an extended left hepatic lobectomy with en bloc cholecystectomy, excision of the choledochal cyst and hepaticojejunostomy reconstruction. Pathology revealed a poorly differentiated adenosquamous carcinoma of the gall bladder and fibrosis and chronic inflammation of the bile duct wall suggestive of a type 1A choledochal cyst. Primary gallbladder carcinoma is an uncommon but highly malignant tumor with a poor 5-year survival rate. Anomalous union of the pancreaticobiliary ductal junction without associated choledochal cyst is a rare congenital anomaly and a known risk factor for development of gallbladder cancer. Here we present a case of advanced gallbladder carcinoma in the setting of abnormal pancreaticobiliary junction with an associated choledochal cyst. Radiographic images (including CT, MRCP and ERCP images) as well as both gross and microscopic pathology images will be displayed.