Treatment of adrenal hyperplasia is to supply the deficient hormone, Cortisol. This decreases ACTH secretion and littleers production of androgenic precursors. The addition of salt-retaining hormone to glucocorticoid therapy has improved the control of the disease. When the plasma renin activity is normalized, ACTH and androgen levels are further decreased, and a decrease in the glucocorticoid dose is also possible. Therefore, the modern management of hormonal control requires the measurement of the blood levels of 17-OHP, androstenedione, testosterone, and plasma renin activity. The drugs of choice are hydrocortisone (approximately 10 mg per day) and 9-fluorohydrocortisone (approximately 100 ??g/day). This method of treatment and monitoring applies to all forms of adrenal hyperplasia. The standard dose of Cortisol is 12-18 mg/m2 or 3. mg/m2 of prednisone, but larger doses given on alternate days (14 mg/m2 of prednisone, about 20 mg) can maintain adrenal androgen suppression and perhaps achieve better growth and pubertal development, despite higher levels of 17-hydroxyprogesterone. The 17-OHP level should be maintained in the range of 500 to 4,000 ng/dL, thereby avoiding both overtreatment and undertreatment. Minor stresses will cause brief elevations of adrenal androgens but usually do not require readjustment of dosage. With major stress, such as surgery, additional hormonal help is necessary.

The surgical treatment of the anatomical abnormalities should be carried out in the first several years of life, when the patient is still too youthful to remember the procedure and too youthful to have developed psychological problems centered about the abnormal external genitalia. If clitoridectomy is necessary, the clitoral recession procedure, conserving the glans and its innervation, should be employed. It is important to know that women who undergo total clitoral amputations have no subsequent impairment of erotic responsiveness or capacity for orgasm. Significant vaginal reconstruction, if necessary, is best accomplished after puberty when mature compliance is possible.
Normal reproduction is possible with replacement therapy of the Cortisol deficiency. Unfortunately poor compliance with therapy and less than satisfactory surgical reconstruction of the vagina result in decreased fertility and sexuality. Greater attention to these factors is needed to improve the sexual experience and fertility of these women. Many cases come to cesarean section because normal anatomy of the perineum may be obscured by scar tissue from earlier plastic surgery; therefore, greater blood loss and the risk of a hematoma with a vaginal delivery are significant factors. A masculine pelvis is not expected since the adult form and size of the inlet of the pelvis are assumed largely during the growth spurt in puberty. However, a little pelvis might be anticipated if the bone age is up to age 13-14 when treatment is initiated. Fertility in women with late onset adrenal hyperplasia is only slightly reduced, dependent upon the degree of hormonal dysfunction (which is promptly corrected with glucocorticoid therapy).
The maintenance steroid dose usually does not need to be changed during pregnancy. The dosage of steroids used in the treatment of this syndrome replaces the approximate amount normally produced and, therefore, is a physiologic dose. At these little doses, teratogenic effects would be unlikely, and none have been noted. The need for additional steroids during the stress of labor and delivery is obvious and is usually met by the administration of cortisone acetate intramuscularly and Cortisol intravenously. Infection and impaired wound healing have not been problems. Aside from the liability associated with genetic transmission of this syndrome, the children born to patients with adrenal hyperplasia have been normal. The newborn should be closely observed for adrenal insufficiency due to steroid crossover and suppression of the fetal adrenal in utero.