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.
A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).