Rare variant of pancreaticobiliary maljunction associated with pancreas divisum in a child diagnosed and treated by endoscopic retrograde cholangiopancreatography: A case report

Abstract

Background: Pancreaticobiliary maljunction (PBM) is an uncommon congenital anomaly of the pancreatic and biliary ductal system, defined as a union of the pancreatic and biliary ducts located outside the duodenal wall. According to the Komi classification of PBM, the common bile duct (CBD) directly fuses with the ventral pancreatic duct in all types. Pancreas divisum (PD) occurs when the ventral and dorsal ducts of the embryonic pancreas fail to fuse during the second month of fetal development. The coexistence of PBM and PD is an infrequent condition. Here, we report an unusual variant of PBM associated with PD in a pediatric patient, in whom an anomalous communication existed between the CBD and dorsal pancreatic duct.

Case summary: A boy aged 4 years and 2 mo was hospitalized for abdominal pain with nausea and jaundice for 5 d. Abdominal ultrasound showed cholecystitis with cholestasis in the gallbladder, dilated middle-upper CBD, and a strong echo in the lower CBD, indicating biliary stones. The diagnosis was extrahepatic biliary obstruction caused by biliary stones, which is an indication for endoscopic retrograde cholangiopancreatography (ERCP). ERCP was performed to remove biliary stones. During the ERCP, we found a rare communication between the CBD and dorsal pancreatic duct. After clearing the CBD with a balloon, an 8.5 Fr 4-cm pigtail plastic pancreatic stent was placed in the biliary duct through the major papilla. Six months later, his biliary stent was removed after he had no symptoms and normal laboratory tests. In the following 4-year period, the child grew up normally with no more attacks of abdominal pain.

Conclusion: We consider that ERCP is effective and safe in pediatric patients with PBM combined with PD, and can be the initial therapy to manage such cases, especially when it is combined with aberrant communication between the CBD and dorsal pancreatic duct.

Keywords: Case report; Children; Communication; Endoscopic retrograde cholangiopancreatography; Pancreas divisum; Pancreaticobiliary maljunction; Variant.

Figure 1

Results of endoscopic retrograde cholangiopancreatography. A: During endoscopic retrograde cholangiopancreatography, a hemispheric papilla with a villus-like opening resembling the major papilla was seen. B-E: Successful cannulation of the papilla and a dilated common bile duct (CBD) detected by X-ray after injection of a contrast agent (B). Multiple small biliary stones were discharged from the papilla after minor endoscopic sphincterotomy (D). Beneath the papilla, the real major papilla was detected (E). After cannulation of the major papilla, the CBD was observed again, which was dilated with multiple filling defects (C), and at the level of the middle–lower CBD, narrowing was observed (arrow); F: During removal of the biliary stones with an endoscopic balloon, unexpectedly, the dorsal pancreatic duct was revealed at the level of the middle–lower part of the CBD; G: Schematic representation of the image of F; H: After clearance of the CBD with the balloon, an 8.5 Fr 4-cm pigtail plastic pancreatic stent was placed in the biliary duct through the major papilla (white arrow); the minor papilla was cannulated again; and the guidewire was advanced into the CBD (black arrow).

Figure 2

Schematic representation of the pancreaticobiliary system. This child had three anomalies: Pancreaticobiliary maljunction, pancreas divisum (arrowheads indicating dorsal pancreatic duct), and abnormal communication between the common bile duct and dorsal pancreatic duct (arrow).