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Outcome of bilateral adrenalectomy in congenital adrenal hyperplasia: one unit’s experience

¹ Departments of Endocrinology, ² Clinical Biochemistry and ³ Surgery, University College London Hospitals, London, UK

(Correspondence should be addressed to G Conway, Department of Endocrinology, UCLH Foundation Trust, 250 Easton Rd, London NW1 2PQ, UK; Email: g.conway{at}ucl.ac.uk)

Objective: The use of bilateral adrenalectomy in the management of congenital adrenal hyperplasia (CAH) is controversial. We set out to review the outcome of 5 cases of CAH who have undergone adrenalectomy in our unit.

Design: A retrospective case note review and subject interview of the experience of adrenalectomy in the setting of a tertiary adult CAH clinic.

Methods: Subjects who had undergone adrenalectomy were reviewed at a routine clinic visit with particular reference to clinical and biochemical outcomes after adrenalectomy.

Results: Two subjects underwent surgery for the sole indication of desire for fertility with successful outcome and without subsequent adrenal crises. Three women suffered from the common clinical management problem of unsuppressible hyperandrogenism and worsening obesity. Whilst the outcome of improved appearance and weight loss was achieved in these subjects, all three experienced significant complications including pigmentation and acute episodes of adrenal insufficiency.

Conclusion: We present a mean follow-up of 4.2 patient years and conclude that this procedure may be suitable for selected women with CAH. Outcomes for those pursuing fertility were positive; however, complications were noted in those women for whom the procedure was performed for symptom control. Accepting that the number of subjects is small, it is clear that more data are required before widely recommending this procedure.