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 child hood or early adolescence (mean age of onset similar to 13 years), and an approximately equal sex incidence. Pancreas divisum is a congenita l variant of pancreatic ductal anatomy in which the ventral and dorsal pancreatic ductal systems fail to fuse, so that two functional papill ae drain the exocrine secretions of the pancreas. In recent years, sev eral reports of pancreatitis associated with pancreas divisum in child ren have appeared. We now report a family in which the mother, son, an d daughter all had presented with recurrent pancreatitis from an early age. Both the mother and son have endoscopic retrograde cholangiopanc reatography-documented pancreas divisum, whereas the daughter has a st ricture in her distal pancreatic duct. To our knowledge, this is the f irst such report of ''familial'' pancreas divisum. The implications of these findings in the setting of hereditary pancreatitis highlight th e controversial issues of the clinical significance of pancreas divisu m and the appropriateness of surgical therapy.