Idiopathic hereditary pancreatitis: experience with surgical treatment
R. A. Williams, B. F. Caldwell and S. E. Wilson
Idiopathic hereditary pancreatitis is a rate 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 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.
