Fertility and body composition after laparoscopic bilateral adrenalectomy in a 30-year-old female with congenital adrenal hyperplasia

Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is caused b y an inborn defect in the 21-hydroxylase gene (CYP21), leading to virilizat ion of female patients and causing ambiguous genitals in the majority of fe male infants. Adult women may suffer from loss of libido, irregular or abse nt cycles, and reduced fertility, despite intensive medical treatment. Thes e problems have stimulated the search for alternative treatment modalities. We present an adult female patient, who was difficult to treat medically a nd whose clinical situation markedly improved after laparoscopic bilateral adrenalectomy. The procedure was well tolerated and without side effects. P ostoperatively the elevated serum progesterone and 17-hydroxyprogesterone l evels, as well as the undetectable LH levels, normalized. The procedure res ulted in marked clinical improvement. Within 12 months after surgery she lo st 11 kg in weight. This weight loss consisted mainly of adipose tissue. Ac ne disappeared, and she had a regular 4-week menstrual cycle, with progeste rone levels that are compatible with a luteal phase. The introduction of la paroscopic techniques may give an impulse to the application of surgical th erapy at a larger scale in patients with 21-hydroxylase deficiency who are difficult to treat with adrenal suppression therapy.