Surgery for nesidioblastosis--indications, treatment and results.

Nesidioblastosis is a life-threatening form of hypoglycemia that starts during the neonatal period in most cases and is caused by hyperinsulinism. Its diagnostic criteria are an extremely high demand for carbohydrates (more than 15 g/kg/day), an inadequately high plasma insulin level, and an inhibited production of ketone bodies. This acute, life-threatening hypoglycemia requires immediate intensive-care treatment. The most important aim of continuous therapy is the prevention of irreversible brain damage. This cannot be reliably avoided by conservative treatment (increased carbohydrate supply, diazoxide administration). Therefore, surgical treatment consisting in subtotal pancreatectomy is becoming increasingly important. The reduction of hormone-producing tissue resolves hyperinsulinism and apparently enables the onset of physiological regulatory mechanisms. Surgical strategy and results in 12 children who underwent surgery for nesidioblastosis are described.