Dominant dorsal duct syndrome: a rare cause of acute recurrent pancreatitis in children revisited

Acute recurrent pancreatitis in children can be caused by anomalies of fusion of pancreatic ducts such as the dominant dorsal duct syndrome wherein a dominant dorsal pancreatic duct is associated with stenosis of the minor papilla. Clinical presentations and management of 2 patients are discussed. An infant presented with severe acute pancreatitis with pseudocyst formation due to an underlying ductal disruption. Surgical treatment was offered on account of failure of medical therapy and endoscopic stenting. A dominant dorsal duct with minor papilla stenosis was encountered. Sphincteroplasty of the minor papilla and lateral pancreaticojejunostomy were performed with good result. A 14-year-old boy with a type 1 choledochal cyst was troubled by recurrent acute pancreatitis. At operation, a dilated dorsal pancreatic duct opening into a stenosed minor papilla was found in addition to the choledochal cyst. Choledochal cyst excision, choledochoduodenostomy, and sphincteroplasty of the minor papilla stenosis were performed. Dominant dorsal duct syndrome is a rare cause of acute pancreatitis in children. A high index of suspicion is necessary to establish a precise diagnosis. Sphincteroplasty of the minor papilla may affect adequate pancreatic drainage and prevent recurrent pancreatitis.