Anomalous Junction of the Pancreaticobiliary Ductal System With a Long Common Channel in a Patient With Chromosome 14 Translocation Causing Recurrent Acute and Chronic Pancreatitis

Abstract

The pancreatic duct (PD) and common bile duct (CBD) normally join with a short (4-5mm) common channel into the duodenum. Rarely, anomalous junctions can occur outside the duodenum where they are no longer influenced by the sphincter of Boyden. One such anomaly is the ‘long common channel' in which the PD inserts into the CBD outside the duodenum and a common channel over 15mm enters the duodenum. These cases are associated with higher incidence of chronic pancreatitis as both bile and pancreatic juices are allowed to reflux into the opposing channel. We present the case of a two year and 3 month old toddler with a past medical history of chromosome 14 translocation (Turner Syndrome variant), agenesis of the corpus callosum, possible optic nerve atrophy, s/p Nissen procedure x2 and gastric tube dependence who presented to the hospital with abdominal pain. Of note, she had recurrent bouts of biliary pancreatitis as well as pancreatitis of unclear etiology. Her symptoms began at 20 months of age when she was jaundiced and underwent MRCP demonstrating gallbladder sludge, and biliary stenosis. She subsequently underwent laparoscopic cholecystectomy but continued to develop recurrent bouts of pancreatitis. Endoscopic cholangiopancreatography (ERCP) demonstrated diffuse irregularity with dilatation (6mm) and beading of the main ventral pancreatic duct in the head of the pancreas and tortuosity with clubbing of the side branches. Also noted was a long 20mm common channel with the pancreatic duct entering the common bile duct (CBD) 2cm above the ampulla. At ERCP, filling of the pancreatic duct was noted upon injecting contrast in the distal CBD. This anatomic variation with an anomalous high entry of the pancreatic duct into the CBD with clear bile reflux into the pancreatic duct was the likely etiology of the patient's recurrent acute on chronic pancreatitis. A 5 french/5cm pigtail stent was endoscopically placed into the pancreatic duct, preventing reflux of bile into the pancreatic duct. This was subsequently changed 2 weeks later. Post-stenting, no recurrent bouts of pancreatitis were noted and the patient did not require hospital readmission. She subsequently underwent laparoscopic choledochoduodenostomy without incident. She remains thriving at this time without further episodes of pancreatitis since surgery. This is the first reported case of an anomalous long common channel in a toddler with a chromosome 14 translocation. Further, it is the first case to successfully treat a long common channel endoscopically with a pancreatic stent, and surgically with choledochoduodenostomy. The development of severe chronic pancreatitis in this case in an unfortunate sequela of the persistent bile reflux through this anomaly.