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The Role of Bilateral Adrenalectomy in the Treatment of Congenital Adrenal Hyperplasia

Division of Pediatric Endocrinology (J.J.V.W.), The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599; and the Division of Pediatric Endocrinology (E.M.R.), Department of Woman and Child Health, Karolinska Institute, S171-76 Stockholm, Sweden

Address all correspondence and requests for reprints to: Judson J. Van Wyk, M.D., Division of Pediatric Endocrinology, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599. E-mail: judvw{at}med.unc.edu.

This report summarizes follow-up studies in 18 patients who underwent bilateral adrenalectomy for congenital adrenal hyperplasia. Three of these patients were young children with null/null mutations of CYP21, and the other 15 were adrenalectomized because of difficulties in their management on conventional therapy. The average duration of follow-up was 59 months and represents an aggregate of 90 postoperative years.

The adrenals were removed laparoscopically in 13 patients and by open flank incisions in five. Adrenal crises associated with severe illnesses occurred in five patients at times when their glucocorticoid substitution was suboptimal. All were responsive to appropriate therapy. Two of these patients were young children who had hypoglycemia during gastroenteritis or febrile illness associated with poor food intake or vomiting. Significant elevations of adrenal steroid precursors, presumably from ectopic adrenal rests, were observed postoperatively in eight of the patients.

Patients and parents were nearly unanimous in their enthusiasm for adrenalectomy. In most, signs of androgen excess have decreased, and obesity has become less of a problem with lowering the dose of glucocorticoid. We conclude that adrenalectomy is a safe and efficacious method of managing congenital adrenal hyperplasia in selected patients. Prophylactic adrenalectomy in young children with double null mutations remains experimental.

Part of these studies were supported by grants to E.M.R. by the Swedish Research Council and the Karolinska Institute.

Abbreviations: CAH, Congenital adrenal hyperplasia; HC, hydrocortisone.

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