Perioperative management of a child with congenital nephrogenic diabetes insipidus

The key point in perioperative management of a patient with congenital nephrogenic diabetes insipidus is fluid and electrolytes management. Since the urine of these patients consists mainly of solute free water, replacement fluids should be fluids which provide free water. A 2-year-old girl with congenital nephrogenic diabetes insipidus was scheduled for dental extraction. Her daily fluid intake was 10 liter. She had a history of recurrent fever, polyuria and polydipsia since 2 months of age. Her previous perioperative course for gastric volvulus at another hospital was complicated with postoperative hyponatremia and convulsion. A venous line was secured the day before surgery and 5% dextrose in water was infused at a rate of 12 ml.kg-1.hr-1. Intraoperative infusion was mainly with 5% dextrose in water combined with maintenance fluid. Five hours after surgery oral intake was started. Her intraoperative electrolytes levels were low (Na 133 mEq.l-1, K 2.8 mEq.l-1), but otherwise her perioperative course was uneventful.