[Anesthetic management of a patient with 21-hydroxylase deficiency]

21-hydroxylase deficiency was described as a fatal disease in infancy manifested by severe adrenal insufficiency with lack of differentiation of the external genitalia in males. Patients are in adrenal insufficiency or receiving long term corticosteroid therapy and are unable to respond normally to surgery or other forms of stress. Therefore, supplementation with corticosteroids and various regimens are required for surgery and anesthetic management. A 3 year 10 months correction of 6] old phenotypic female [correction of male] with 21-hydroxylase deficiency and congenital adrenal hyperplasia was scheduled for critroplasty. Anesthesia was induced with sevoflurane, nitrous oxide and oxygen. After endotracheal intubation, a caudal epidural catheter was placed. At the induction of anesthesia, the patient received a bolus of hydrocortisone 30 mg i.v. followed by a continuous infusion of hydrocortisone. The patient's general condition was good during surgery without any major complications. Caudal epidural anesthesia is considered to be effective for anesthetic management of these patients.