Nesidioblastosis involves beta-cell hyperplasia within the pancreatic islets, resulting in hyperinsulinemia and hypoglycemia. A patient with nesidioblastosis may need to undergo surgery, especially a partial or total pancreatectomy.
Concern
Management
hypoglycemia
Infuse glucose at a rate sufficient to avoid hypoglycemia (see below). Blood glucose is monitored periodically.
seizures
Avoid hypoglycemia. Medication to control seizures should be readily available. A patient with a serious seizure disorder should receive anticonvulsants during surgery.
oxygen desaturation
Monitor oxygenation with a pulse oximeter.
fluid overload
Monitor fluid input and output.
hypothermia, especially if an infant
Monitor body temperature.
electrolyte imbalances
Monitor serum electrolytes, adjusting as needed.
The daily amount of glucose required to avoid hypoglycemia is determined prior to surgery. This amount is used as a guide for glucose infusion during surgery.
In addition to glucose infusions the patient may receive glucagon or diazoxide to control insulin secretion.
If a pancreatectomy is performed, then the serum insulin will drop and the need for a constant infusion of glucose decreases. With a total pancreatectomy exogenous insulin and pancreatic enzymes will be a lifetime requirement.
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