Bilateral laparoscopic adrenalectomy as a treatment for classic congenital adrenal hyperplasia attributable to 21-hydroxylase deficiency.

Abstract

Current medical therapy for congenital adrenal hyperplasia (CAH) attributable to a complete 21-hydroxylase deficiency is not optimal. Difficulties in adequate adrenal androgen suppression are common, causing short adult stature, infertility, and hyperandrogenism. We report the use of laparoscopic bilateral adrenalectomy as a definitive therapy for this condition and argue that it is superior to conventional medical therapy in selected patients. Participants were 2 adult females with classic, salt-wasting CAH and a history of poor adrenal control were selected for adrenalectomy: case 1 was a 22-year-old woman with mild hirsutism and primary amenorrhea; case 2 was a 28-year-old woman with severe hirsutism, acne, and amenorrhea. Preoperative and postoperative hormonal profiles were performed. Both underwent laparoscopic bilateral adrenalectomy with a mean follow-up of 37 months. Bilateral laparoscopic adrenalectomy was performed in both patients with no complications and an uneventful recovery. Maintenance medications of glucocorticoid and mineralocorticoid replacement were reduced compared with preoperative doses. Three years postoperatively, however, rising adrenal steroid precursor levels in case 1, presumably caused by adrenal rests, prompted an increase in replacement therapy dose. Hirsutism and acne improved in both patients, and regular menstruation began 5 months (case 1) and 2 months (case 2) postoperatively. Pregnancy 3 years postoperatively was successful in case 2, who delivered a unaffected infant, full-term via Cesarian section. Surgical adrenalectomy should be considered in females with classic CAH attributable to 21-hydroxylase deficiency and a history of poor hormonal control. Adrenalectomy may prove to be superior to current medical therapy for these patients.