Three generations of hereditary chronic pancreatitis

The patient was a 22 year-old male. Hereditary chronic pancreatitis was sus pected as a diagnosis since his mother's uncle had been operated on for chr onic pancreatitis 14 years previously at the age of 64 years and his mother had been operated on for chronic pancreatitis with calculi 5 years previou sly at the age of 40 years. Surgery was needed, since: 1) he had experience d abdominal pain for 8 years; 2) endoscopic retrograde cholangiopancreatogr aphy(ERCP) revealed a marked irregular dilatation in the main pancreatic du ct and a marked irregular dilatation and protein plugs in the ductule of th e tail of the pancreas; and, 3) pancreatic functional diagnostic (PFD) test examination showed a 75% decrease in exocrine function. If a surgical proc edure had not been performed, the patient would likely have experienced cal culi formation in the pancreas and a further decrease in exocrine function. Since the patient was very young and had many protein plugs in the dilated ductule of the tail of the pancreas, we decided to perform a spleen-preser ving Puestow's procedure with removal of the tail of the pancreas. Clinical and pathological findings of hereditary pancreatitis are reviewed.