A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.
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.