Key result
Perioperative management of a 14-year-old girl with 11-beta-hydroxylase deficiency CAH was complicated by severe intraoperative hypotension, successfully treated with intravenous fluids and an epinephrine infusion.
Case Report (n=1)
No
Patients with 11-beta-hydroxylase deficiency CAH may experience severe perioperative hemodynamic instability due to chronic antihypertensive therapy (like ARBs) combined with neuraxial anesthesia.
Anticipate hemodynamic instability in CAH patients on ARBs with neuraxial anesthesia; case report leaves open optimal perioperative protocols.
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. 11?-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 11?-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. J Med Cases. 2015;6(5):221-225 doi: http://dx.doi.org/10.14740/jmc2125w
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Whitaker et al. (2015) conducted a case report in Congenital adrenal hyperplasia (11-beta-hydroxylase deficiency) (n=1). Perioperative anesthetic and medical management was evaluated. Perioperative management of a 14-year-old girl with 11-beta-hydroxylase deficiency CAH was complicated by severe intraoperative hypotension, successfully treated with intravenous fluids and an epinephrine infusion.
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