Noninsulinoma pancreatogenous hypoglycemia syndrome: An update in 10 surgically treated patients

Background. Neuroglycopenia from endogenous hyperinsulinism usually is caus ed by insulinomas in adults. We recently reported a novel hypoglycemic diso rder in 5 patients (patients 1 to 5) with postprandial neuroglycopenia, neg ative 72-hour fasts, negative perioperative imaging studies, but positive c alcium stimulation tests and islet hypertrophy and nesidioblastosis in the gradient-guided resected pancreata. Methods. In this report we compare our experience with 5 additional patient s (patients 6 to 10) with this syndrome to that in the original report. Results. The clinical features of patients 6 to 10 were similar to those of patients 1 to 5. Each had positive calcium stimulation testing that guided the extent of the distal pancreatectomy and histologic evidence of islet c ell hypertrophy or nesidioblastosis. All 10 patients are alive from 9 to 50 months after operation, 1 of whom had no amelioration of neuroglycopenia. Minor perioperative complications occurred in 3 patients. One patient has e xperienced repeated bouts of acute pancreatitis, pseudocyst formation, and exocrine insufficiency. Conclusions. We have identified adult patients with severe, postprandial hy perinsulinemic hypoglycemia from diffuse islet cell disease, 80% of whom ha ve been well palliated with surgery. The results in 7 men have been better than those in the 3 women for reasons that are not obvious.