Pancreaticobiliary maljunction without choledochal cysts in infants and children: clinical features and surgical therapy.

Pancreaticobiliary maljunction (PBM) usually is associated with choledochal cyst. PBM without dilatation of the common bile duct is rare in infants and children. This rare type of the anomaly may lead to the development of malignancy of the bile duct in later life. The authors report the clinical presentation and surgical treatment of seven pediatric patients with PBM. Symptoms and signs included repeated episodes of abdominal pain (7 of 7), nausea and vomiting (6 of 7), intermittent jaundice (3 of 7), and acholic stools (2 of 7). Some patients presented with high levels of serum and urinary amylase. These symptoms and signs might have been related to the temporary obstruction of bile flow in the common channel, where endoscopic retrograde cholangiopancreaticography (ERCP) disclosed a protein plug in four of the patients. The common bile duct proximal to the junction of the main pancreatic duct was excised, and a Roux-en-Y hepaticojejunostomy reconstruction was performed. To prevent iatrogenic injury of the main pancreatic duct, repeat cholangiography was performed with the aid of radiopaque silver clips placed on the line of dissection to evaluate the distance between the site of dissection and the main pancreatic duct. All patients have been free of symptoms since the surgery. PBM without dilatation of the common bile duct can be detected more frequently if ERCP is performed on every patient who has repeated episodes of abdominal pain refractory to conventional therapy. Complete excision of the common bile duct and gallbladder followed by hepaticojejunostomy is recommended for PBM, while the goals of decreasing the high risk of carcinoma of the bile duct and preventing recurrent symptoms.