Idiopathic Hereditary Pancreatitis

Abstract

† Idiopathic hereditary pancreatitis is a rare form of primary chronic pancreatitis transmitted as an autosomal dominant trait with a variable clinical penetrance. We studied a Hispanic kindred of 23, seven of whom had the disease. In each of the seven cases, abdominal pain had started when the patient was a teenager. The disease was confirmed surgically in three patients, biochemically in three, and roentgenographically in one. No causes were determined. The three patients who had surgery each had a ten- to 30-year history of recurrent severe abdominal pain requiring multiple hospital admissions. In each the pancreatic duct had a "chain-of-lakes" appearance on endoscopic retrograde cholangiopancreatographic examination and was drained by longitudinal pancreatojejunostomy. There was no recurrence of symptoms after surgery. Retrograde drainage of the pancreatic duct reliably relieves the symptoms of idiopathic hereditary pancreatitis. (Arch Surg 1982;117:408-412)