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 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.