Ann Hepatobiliary Pancreat Surg.  2023 May;27(2):220-225. 10.14701/ahbps.22-118.

Duplicated extrahepatic bile duct (type Vb): An important rare anomaly

• Varshney VK
• Hussain S
• Vignesh N
• Selvakumar B
• Agarwal L
• Yadav T

• KMID: 2542588
• DOI: http://doi.org/10.14701/ahbps.22-118

Abstract

Congenital duplication of the extrahepatic bile duct (DEBD) is an unusual anomaly of the biliary system. It occurs due to inability of the embryological duplex biliary system to regress. DEBD has various subtypes depending on the morphology and opening of the aberrant common bile duct. It can have distinct complications. We encountered a 38-year-old lady who experienced pain in the right upper abdomen along with a low-grade fever. Magnetic resonance cholangiopancreatography revealed DEBD with multiple calculi in the right hepatic duct (ductolithiasis) and joining of the right hepatic duct with the left hepatic duct in the intrapancreatic region. Endoscopic retrograde cholangiography failed to clear the calculi from the right duct. They were then managed by common bile duct exploration and roux-en-Y right hepaticojejunostomy for biliary drainage. Her postoperative period was uneventful. She is currently doing well after three months of follow-up. Hence, a proper preoperative delineation of such rare anomalies is essential. It could avoid inadvertent injury to the bile duct and operative complications.

Keyword

Rare diseases; Extrahepatic bile ducts; Magnetic resonance cholangiopancreatography; Congenital abnormalities; Gallstones

Figure

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  Fig. 1 Magnetic resonance cholangiopancreatography (MRCP) images. (A, B) Axial T2-weighted fat saturated images showing calculus in the cystic duct (white arrow in A) which is draining in right duct and cholelithiasis (arrowhead in B); (C) Unmarked and (D) marked MRCP images depicting ductolithiasis in the right duct as filling defects (black arrows). Proximal communication between both ducts (red arrow) with distal joining (yellow arrow) and associated narrowing of the right duct in the lower part is shown. A single distal common bile duct (green arrow) is seen with a normal pancreatic duct.

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  Fig. 2 Endoscopic retrograde cholangiography. (A) Contrast injected into the left duct (yellow arrow) along with visualization of the right duct via proximal. (B) Both right and left extrahepatic bile duct (EBD) visualized with communication between the two in the proximal part (red arrow) and multiple calculi in the right EBD (yellow arrow). (C) Biliary stents were placed, one in the left EBD (black arrow) and the other in the right anterior sectoral duct (red arrow), via the cranial communication between the two ducts.

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  Fig. 3 Intraoperative images. (A) Gall bladder (GB) dissected by fundus-first approach with visualization of the cystic duct (star), right (yellow arrow) and left (blue arrow) extrahepatic bile duct (EBD); (B) right EBD explored with the removal of all calculi (yellow arrow) along with closed cystic duct stump (blue arrow); (C) completed right hepaticojejunostomy (blue arrow); (D) schematic diagram of type Vb EBD with ductolithiasis in the right EBD and intercommunicating duct (red arrow).

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  Fig. 4 Choledochoscopy images. (A, B) Visualization of the proximal communication from the right duct (arrow) and its strictured lower end (arrow); (C) intraoperative cholangiogram: contrast was injected into the right duct depicting proximal right duct (red arrow) with contrast passed into the duodenum via the proximal communication between the left duct. Two biliary stents could be visualized in the left ductal system (black arrows).

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  Fig. 5 Modified classification system of the duplicated extrahepatic bile duct.

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