21-hydroxylase deficiency surgery

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ;Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

Overview

Reconstructive surgery and surgical adrenalectomy are the surgical procedures used for patients with 21-hydroxylase deficiency. Reconstructive gynecologic surgery may be used in adolescents or adults, including clitoroplasty and vaginoplasty. Surgical adrenalectomy can be done in some cases, if there is no response to medical therapy.

Surgery

Reconstructive surgery

Reconstructive gynecologic surgery may be used in adolescents or adults, including clitoroplasty and vaginoplasty. ^[1]

Surgical adrenalectomy

Surgical adrenalectomy can be done in some cases, if there is no response to medical therapy.

The major benefit is lowering adrenal androgen and progesterone secretion. However, it increases dependency on glucocorticoid and mineralocorticoid replacement therapy. ^[2]

References

↑ Premawardhana LD, Hughes IA, Read GF, Scanlon MF (1997). “Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience”. Clin Endocrinol (Oxf). 46 (3): 327–32. PMID 9156043.
↑ Crocker MK, Barak S, Millo CM, Beall SA, Niyyati M, Chang R; et al. (2012). “Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia”. J Clin Endocrinol Metab. 97 (11): E2084–9. doi:10.1210/jc.2012-2298. PMC 3485588. PMID 22904181.

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[pmid9156043-1] Premawardhana LD, Hughes IA, Read GF, Scanlon MF (1997). “Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience”. Clin Endocrinol (Oxf). 46 (3): 327–32. PMID 9156043.

[pmid22904181-2] Crocker MK, Barak S, Millo CM, Beall SA, Niyyati M, Chang R; et al. (2012). “Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia”. J Clin Endocrinol Metab. 97 (11): E2084–9. doi:10.1210/jc.2012-2298. PMC 3485588. PMID 22904181.

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