title: Anaesthetic management of a case of nesidioblastosis for subtotal pancreatectomy.
abstract: Hyperinsulinism is a rare cause of severe persistent hypoglycaemia in the neonatal period. It is associated with a high incidence of brain damage and mental retardation as a consequence of repeated episodes of hypoglycaemia. Subtotal to near total pancreatectomy is indicated as a matter of urgency to decrease the amount of circulating insulin. The perioperative management of a 45-day-old, 5 kg male infant with hyperinsulinaemia (nesidioblastosis) is described. He had a history of generalized tonic clonic seizures 4 h after birth. The blood sugar at that time was 0.66 mmol x l(-1) (12 mg x dl(-1)) and serum calcium was 2.4 mmol x l(-1) (9.82 mg x dl(-1)). The insulin : glucose ratio was 1.6 (normal < 0.4). Occasional episodes of hypoglycaemia persisted in spite of medical line of management with intravenous dextrose 12%, 2 h gastric tube feeds, hydrocortisone (5 mg x kg(-1) x day(-1) i.v.) and oral diazoxide 10 mg x kg(-1), 8 h for 3 weeks. A CT scan and USG did not reveal any abnormality of the pancreas. However, the EEG varied from one of abnormally low amplitude to an isoelectric record. Renal, liver function tests and coagulation profile were normal. The patient was scheduled for elective subtotal pancreatectomy. The anaesthetic management with emphasis on glucose homeostasis and fluid balance is discussed.
authors.affiliation: Bai Jerbai Wadia Hospital For Children, Acharya Donde Marg, Parel, Mumbai, India.
authors.forename: Madhusudhan
authors.initials: M
authors.lastname: Mali
authors.forename: Hema
authors.initials: H
authors.lastname: Bagry
authors.forename: Lakshmi
authors.initials: L
authors.lastname: Vas
mesh_terms: D000768:Anesthesia, General; D001786:Blood Glucose / Q000378:metabolism; D006706:Homeostasis; D006801:Humans; D006946:Hyperinsulinism / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010180:Pancreatectomy; D010182:Pancreatic Diseases / Q000601:surgery*; D014882:Water-Electrolyte Balance
chemical_list: D001786:Blood Glucose