Surgical therapy and long-term follow-up of childhood hereditary pancreatitis.

Treatment and a 15-year follow-up survey of 42 patients with hereditary pancreatitis (HP) were compared with 28 patients with idiopathic recurrent pancreatitis (RP) of childhood. There was no difference between the two groups except for pancreatic ductal dilatation and stones in patients with HP. Longitudinal pancreaticojejunostomy (20) or resection and drainage procedures (7) were more commonly required in patients with HP than RP (55% v 14%). There was no surgical mortality. Postoperatively, immediate and complete relief of symptoms was obtained in 43% of patients with HP and 25% of patients with RP. In the remainder, recurrent attacks of pancreatitis abated over 2 years such that 81% of the surgical patients were in good or excellent health. Occasional symptoms persisted in 52% of HP patients and 25% of RP patients. Of the 20 patients with HP or RP undergoing longitudinal pancreaticojejunostomy extending from the head to the tail, 75% were symptom-free on follow-up. However, 3 of 6 patients with poor results had also undergone this procedure. At long-term follow-up of patients who did not undergo operation, 75% of HP patients and 90% of RP patients reported excellent or good health despite the persistent symptoms in 68% and 42%, respectively. Surgery for childhood HP is dependent on the complications present. Longitudinal pancreaticojejunostomy is beneficial for ductal dilatation and associated pseudocysts or pancreatic ascites. The performance of this procedure in the absence of consistent pancreatic duct dilatation will give poor results. Patients without ductal dilatation and the majority of patients with RP may eventually lead near normal lives without resorting to surgery.(ABSTRACT TRUNCATED AT 250 WORDS)