Anaesthetic management of a neonate with nesidioblastosis

Nesidioblastosis is a disorder which is characterized by autonomous insulin secretion that is not affected by decreases in blood glucose. Patients usually present during the neonatal or infantile period with hypoglycaemia associated with hyperinsulinaemia. Most cases of nesidioblastosis are sporadic; however, familial forms appears to be inherited in an autosomal recessive manner. We discuss the anaesthetic management of a patient with nesidioblastosis who presented for near total pancreatectomy and review the literature pertinent to the anaesthesiologist.