A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.
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.