An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.
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.