Pancreas divisum in a family with hereditary pancreatitis.

Hereditary pancreatitis is characterized by an autosomal-dominant mode of inheritance with incomplete penetrance, onset of symptoms in childhood or early adolescence (mean age of onset approximately 13 years), and an approximately equal sex incidence. Pancreas divisum is a congenital variant of pancreatic ductal anatomy in which the ventral and dorsal pancreatic ductal systems fail to fuse, so that two functional papillae drain the exocrine secretions of the pancreas. In recent years, several reports of pancreatitis associated with pancreas divisum in children have appeared. We now report a family in which the mother, son, and daughter all had presented with recurrent pancreatitis from an early age. Both the mother and son have endoscopic retrograde cholangiopancreatography-documented pancreas divisum, whereas the daughter has a stricture in her distal pancreatic duct. To our knowledge, this is the first such report of "familial" pancreas divisum. The implications of these findings in the setting of hereditary pancreatitis highlight the controversial issues of the clinical significance of pancreas divisum and the appropriateness of surgical therapy.