A 6-week-old child presents with generalized seizures, a serum glucose of 30 mg/dL and concurrent hyperinsulinemia. This child’s first priority is which of the following?

a. Permanent central venous access and glucose infusion

Most cases of hyperinsulinemia in the first 2 years of life are due to nesidioblastosis, a condition associated with excessive and diffuse formation of neoislets from primitive pancreatic ductal cells. Hyperinsulinemia secondary to islet cell adenoma or carcinoma or islet cell hyperplasia is more common in the older child. Infants with nesidioblastosis, such as the one described here, typically present with symptomatic hypoglycemia, seizures and hyperinsulinemia. An insulin-to-glucose ratio (insulin in IU/ml divided by glucose in mg/dL) that is greater than 0.5 with fasting is highly suggestive.

Infants with nesidioblastosis are managed initially medically with maintenance of blood glucose levels above 40 mg/dL. This is best carried out by the infusion of hypertonic glucose solutions through a permanent central venous catheter. In addition, diazoxide, cortisone and adrenocorticotropic hormone and streptozocin have been used to treat the hypoglycemia. Definitive management of nesidioblastosis may require pancreatic resection. This requires a pancreatectomy usually estimated at approximately 95% to 99% with splenic and duodenal preservation. Following 90% to 95% pancreatectomy, over 90% of infants with nesidioblastosis are rendered permanently euglycemic. A CT scan of the abdomen to search for an adenoma is an appropriate diagnostic maneuver, but it is not the first priority.