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Fertility and Body Composition after Laparoscopic Bilateral Adrenalectomy in a 30-Year-Old Female with Congenital Adrenal Hyperplasia

Departments of Medicine (H.B., A.H.B., J.W.K.) and Surgery (J.B.), University Hospital Rotterdam, 3000 CA Rotterdam; and Department of Medicine (F.H.d.J., S.W.J.L.), Erasmus University Rotterdam, 3000 DR Rotterdam, The Netherlands

Address correspondence and requests for reprints to: Dr. Atte H. Bootsma, Department of Medicine, Room D430, University Hospital Rotterdam, P.O. Box 2040, 3000 CA Rotterdam, The Netherlands. E-mail: bootsma{at}inw3.azr.nl

Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is caused by an inborn defect in the 21-hydroxylase gene (CYP21), leading to virilization of female patients and causing ambiguous genitals in the majority of female infants. Adult women may suffer from loss of libido, irregular or absent cycles, and reduced fertility, despite intensive medical treatment. These problems have stimulated the search for alternative treatment modalities. We present an adult female patient, who was difficult to treat medically and whose clinical situation markedly improved after laparoscopic bilateral adrenalectomy. The procedure was well tolerated and without side effects. Postoperatively the elevated serum progesterone and 17-hydroxyprogesterone levels, as well as the undetectable LH levels, normalized. The procedure resulted in marked clinical improvement. Within 12 months after surgery she lost 11 kg in weight. This weight loss consisted mainly of adipose tissue. Acne disappeared, and she had a regular 4-week menstrual cycle, with progesterone levels that are compatible with a luteal phase. The introduction of laparoscopic techniques may give an impulse to the application of surgical therapy at a larger scale in patients with 21-hydroxylase deficiency who are difficult to treat with adrenal suppression therapy.

This article has been cited by other articles:

				J. J. Van Wyk and E. M. Ritzen The Role of Bilateral Adrenalectomy in the Treatment of Congenital Adrenal Hyperplasia J. Clin. Endocrinol. Metab., July 1, 2003; 88(7): 2993 - 2998. [Abstract] [Full Text] [PDF]