Association of extrahepatic bile duct duplication with pancreaticobiliary maljunction and congenital biliary dilatation in children: a case report and literature review

Abstract

We herein report a case of cystic-type congenital biliary dilatation (CBD) in whom an extremely rare anomalous duplication of the common bile duct and pancreaticobiliary maljunction were diagnosed intraoperatively by meticulous surgical manipulations via conventional open surgery. By performing a dissection at the outer epicholedochal layer of the cyst, a thin cord-like structure shown to be the distal part of the common bile duct was identified. A further exploration revealed that the most distal (extra- and intrapancreatic) part of the common bile duct was duplicated, and each branch of the duct was connected to the main and accessory pancreatic ducts. The experience with our case and a literature review showed that extrahepatic bile duct duplication is generally associated with pancreaticobiliary maljunction and CBD. We conclude that an extremely careful exploration with delicate and meticulous surgical manipulation is essential to identify these morphological anomalies and prevent intraoperative and postoperative complications of CBD, such as pancreatic duct injury or pancreatitis.

Anatomical variations of liver blood supply in patients with pancreaticobiliary maljunction

“…Several authors have reported cases of annular pancreas (AP), pancreas divisum (PD), and double common bile duct (DCBD) accompanied by PBM [34][35][36][37][38][39]. Our cohort included one patient with PBM and PD together without vascular variants.…”

An extremely rare combination of choledochocele and bile duct duplication escalating severe acute pancreatitis and cholangitis: A case report

“…There are few reports of combined case of congenital choledocho-dilatation (choledochal cyst) with bile duct duplication [ 7 ]. In their report, all cases of choledoco-dilatation were cystic or fusiform type (Type I).…”

Common bile duct duplication: The more the murkier

“…Boyden proposed that the development of type I DCBD was due to double vacuolization during the solid stage during the development of extrahepatic bile ducts, whereas chance elongation and early subdivision of the primitive hepatic furrow may be responsible for the other types of DCBD. [ 10 11 ]…”

“…[ 9 ] It is more commonly seen in children as it presents early in life due to abdominal pain and other associated symptoms. [ 10 ] Patients with ABPJ may invariably require surgery in the form of separation of the biliary and pancreatic systems to prevent the reflux of pancreatic juice as well as excision of the extrahepatic ducts, which are at risk of malignancy. It is important to know whether ABPJ is associated with the double common CBD.…”