A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.
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.