A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.
A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.