Pancreatic resection was required in 11 pediatric patients over a 14 year period for control of persistent hypoglycemia. A standard surgical approach based on pathologic considerations was utilized. A subtotal distal pancreatectomy, liver biopsy, and removal of additional adenomatous tissue when found was performed in each patient. This approach precluded the need for routine preoperative visceral angiography, which is currently reserved for patients who require a second exploration. Surgery was curative in patients with adenomatous disease, although patients with diffuse pancreatic lesions usually required continued pharmacologic therapy postoperatively. Two patients thus far have had a total pancreatectomy performed at a second laparatomy due to persistent intractable disease. Seizure disorders, mental retardation, and behavioral problems frequently were unresponsive to improved control of hypoglycemia. An aggressive approach to management of this disorder is required if permanent neurologic damage is to be minimized.
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