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.
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.