Background: Nesidioblastosis, also known as noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS), is a rare functional disorder of pancreatic beta cells characterized by inappropriate insulin secretion in the presence of hypoglycemia. Although idiopathic nesidioblastosis is diagnosed in infancy, there is now considerable interest in an acquired form in adults, especially after bariatric surgery1,2. Anesthesia providers must take special precautions in caring for these patients in the perioperative setting.
Case Description: A 42-year-old man with cervical spinal stenosis, cerebral palsy, nesidioblastosis, myasthenia gravis, and narcolepsy with cataplexy presented for an elective anterior cervical discectomy and fusion.
Preoperatively: The patient was diagnosed with nesidioblastosis after frequent episodes of random hyperinsulinemia and hypoglycemia which were complicated by hypoglycemic infarcts seen on brain MRI. He reported occasional nighttime hypoglycemic episodes so he is now taking glucagon 1 mg subcutaneously at bedtime. He is followed by his endocrinologist, who recommended continuous glucose monitoring and strict avoidance of insulin during surgery.
Intraoperatively: After induction, an arterial line was placed for close hemodynamic and glucose monitoring, and general anesthesia was maintained using propofol, remifentanil, and sevoflurane. A blood glucose level measured 35 minutes after induction was 88 mg/dL; however, 10 minutes later it was 55 mg/dL so 10 g of 50% dextrose and 1 mg of IV glucagon were given. It improved to 82 mg/dL in 3 minutes and levels were subsequently measured every 10 minutes. Although a continuous dextrose infusion was considered, it was never initiated since levels remained stable between 93-180 mg/dL for the remainder of the case.
Postoperatively: The patient was successfully extubated and transferred to the ICU for close glycemic and respiratory monitoring. Glucose levels remained between 105-133 mg/dL over the next 24 hours and the patient was discharged home the next day.
Discussion: Intraoperative glycemic control in the setting of hyperinsulinemic hypoglycemia presents a unique challenge for the anesthesia provider; surgical stress causes perturbations in metabolism, and most neuroglycopenic symptoms of hypoglycemia are undetectable under general anesthesia. To the author’s knowledge, this is the first case report of intraoperative management of hypoglycemia in a patient with nesidioblastosis, outside of patients undergoing pancreatectomy3. Mainstays of management include using a continuous infusion of glucose-containing solutions, frequent blood glucose monitoring, and emergent treatment of hypoglycemia with dextrose and glucagon. This case highlights successful intraoperative management techniques that can be utilized by anesthesiologists who may encounter an adult patient with nesidioblastosis.
References:
- M.V. Davi, A. Pia, V. Guarnotta, G. Pizza, A. Colao, A. Faggiano. The treatment of hyperinsulinemic hypoglycaemia in adults: an update. J Endocrinol Invest. 2017; 40: 9-20.
- P Ritz, C Vaurs, M Barigou, H Hanaire. Hypoglycemia after gastric bypass: mechanisms and treatment. Diabetes Obesity and Metabolism. 2016; 18: 217-223.
- O Hardy, RS Litman. Review article: Congenital hyperinsulinism – a review of the disorder and a discussion of the anesthesia management. Pediatric Anes. 2007; 17: 616-621.
