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 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.