Neonatal hyperinsulinism--surgical and pathologic considerations.

Hyperinsulinism in infancy is most often associated with a diffuse pancreatic lesion designated islet-cell dysmaturation syndrome. This disease is commonly associated with persistent hypoglycemia which usually results from inappropriate secretion of insulin. Urgent medical therapy consisting of hypertonic glucose infusion, frequent feeding, and diazoxide is mandatory in order to prevent central nervous system damage. Where medical means of therapy are not effective, an early 85% subtotal pancreatectomy with preservation of the spleen, is indicated. Nine infants who suffered from hyperinsulinism are reported. In two, medical measures were sufficient to control the disease. One of these patients in whom treatment was started late, remained slightly mentally retarded. Seven patients underwent 85% pancreatectomy. In one of these, an additional 7.5% of the pancreas had to be removed and in a second patient total pancreatectomy was performed in order to control the disease. One patient died on the eighth postoperative day after acute gastric perforation. There was no evidence of residual brain damage in the patients who underwent subtotal pancreatectomy.