Perioperative Care of an Adolescent With 11-Beta-Hydroxylase Deficiency

Abstract

Congenital adrenal hyperplasia (CAH) is the result of an autosomal recessive disorder affecting one of the several steps required for the synthesis of cortisol from cholesterol by the adrenal cortex. 11β-hydroxylase deficiency accounts for 5-8% of all cases of CAH with an incidence estimated at 1 in 100,000 -200,000 newborns. We present a 14-year-old girl with CAH due to 11β-hydroxylase deficiency who presented for surgical treatment of virilization. The perioperative care of patients with CAH should address issues related to the deficient and excessive production of specific mineralocorticoids and corticosteroids by the adrenal cortex. The authors discuss the perioperative implications of the disorder and review previous reports of anesthetic care for such patients.