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 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 ).