Hypoglycemic syndrome in infancy and childhood. A surgeon's perspective.

Persistent, severe hypoglycemia due to inappropriate or excessive insulin secretion is an infrequent but devastating metabolic emergency in infancy and childhood. The primary therapeutic goal is to normalize blood glucose levels and to prevent the catastrophic sequelae of permanent brain damage and mental retardation due to refractory hypoglycemia. Overall, the use of diazoxide, the mainstay of medical treatment, has proved disappointing and has led to the reevaluation of aggressive, early surgical intervention. Currently, subtotal (80% to 90%) pancreatectomy is the most widely used operative procedure in the treatment of hyperinsulinism. It is, however, still associated with a relatively high failure rate, particularly in neonates or during early infancy when either nesidioblastosis or islet cell hyperplasia-both diffuse proliferative beta-cell disorders-is the most common cause of inappropriate insulin secretion. In these cases, "total" or "near-total" pancreatectomy may be necessary to control the hypoglycemic crisis.