Idiopathic hypoglycemia in infancy. Surgical management.

Abstract

Resection of 75% to 90% of the pancreas is an important measure in the treatment of at least three different β-cell disorders that produce severe persistent hypoglycemia of infancy. The treatment is associated with low mortality and morbidity and should be undertaken early to obviate the not infrequent occurrence of mental retardation. Of three infants described herein who underwent subtotal pancreatectomy for idiopathic hypoglycemia, two maintained normal blood glucose levels postoperatively without further therapy. The third required cortisone administration for four years, after which the blood glucose level was maintained innormal range without therapy. The resected pancreas in two patients showed islet cell hyperplasia; in one of these, an unusual budding of islet cells (nesidioblastosis) was found. In one patient, the resected pancreas was normal histologically. Two patients have no apparent brain damage, and one has mild retardation.