Ectopic adrenal rests in congenital adrenal hyperplasia as a cause of androgen excess after adrenalectomy detected by pelvic venous sampling.

Abstract

BACKGROUND:

Patients with classic congenital adrenal hyperplasia (CAH) due to CYP21 deficiency are treated with supraphysiological doses of glucocorticoids to suppress elevated androgen production. This implies also side effects of high-dose glucocorticoids, possibly leading to iatrogenic Cushing's syndrome. Bilateral adrenalectomy has been suggested as the ultimate therapy in severe cases, when insufficient androgen suppression was obtained despite high glucocorticoid doses. Usually, ACTH levels rise after bilateral adrenalectomy, and this could imply an increased risk for the development of ectopic adrenal rests. In female CAH patients ovarian adrenal rests are not commonly detected by conventional radiological techniques.

METHODS:

We report the case of an adult female CAH patient who underwent bilateral adrenalectomy in early puberty because of poorly controlled CAH.

RESULTS AND CONCLUSIONS:

Several years after surgery, she developed secondary amenorrhea and hair loss as a result of androgen overproduction in ovarian adrenal rests that appeared to be detectable only by pelvic venous sampling. After unilateral oophorectomy androgen levels normalized.

• Authors: H L Claahsen-van der Grinten, M M L Stikkelbroeck, J Bulten, M den Heyer
• Journal: Hormone research in paediatrics (Horm Res Paediatr) Vol. 80 Issue 4 Pg. 293-8 ( 2013) ISSN: 1663-2826 [Electronic] Switzerland
• PMID: 24107716 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2013 S. Karger AG, Basel.

Chemical References

• Androgens

Topics

• Adrenal Glands
• Adrenal Hyperplasia, Congenital (blood, surgery)
• Adult
• Androgens (blood)
• Choristoma (surgery)
• Female
• Humans