MacKay, Diana

Nordenström, Anna

Falhammar H

2018-03-15

Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic-pituitary-adrenal (HPA) axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing's syndrome being a frequent complication of adequate suppression. Bilateral adrenalectomy, with subsequent replacement of glucocorticoids and mineralocorticoids at physiological doses, has been proposed as an alternative therapeutic strategy. To review the outcomes following bilateral adrenalectomy for CAH. A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to January 10, 2018. Case reports or case series were included if they reported individual patient data in patients with CAH who had undergone bilateral adrenalectomy. Information regarding the following was extracted: first author, country, sex, age at adrenalectomy, year of adrenalectomy, diagnosis, molecular abnormality, pre- and post-operative biochemistry, pre- and post-operative medications, pre- and post-operative body mass index (BMI), indication for adrenalectomy, surgical technique, gross and microscopic adrenal characteristics, follow-up duration, and short-term as well as long-term post-operative outcomes. Forty-eight cases of bilateral adrenalectomy in CAH were identified, with patients aged from four months to 56 years at time of surgery. The most common indication for surgery was inability to control hyperandrogenism/virilization and/or Cushing's syndrome (n=30, 62%). Most patients (n=34, 71%) reported symptomatic improvement post-operatively, with some cases of short-term (n=5, 10%) and long-term (n=13, 27%) adverse outcomes. Bilateral adrenalectomy in CAH appears to be a reasonable therapeutic option in carefully selected patients who have had unsatisfactory outcomes with conventional medical management.

J Clin Endocrinol Metab . 2018 May 1;103(5):1767-1778. doi: 10.1210/jc.2018-00217.

https://pubmed.ncbi.nlm.nih.gov/29554355/?otool=iaurydwlib

https://hdl.handle.net/10137/5130

Bilateral Adrenalectomy in Congenital Adrenal Hyperplasia: A Systematic Review and Meta-Analysis.

Journal Article

Publication