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Cited by 89Sexual Differentiation and DisordersAdrenal Hormones and DisordersHormonal Regulation and Hypertension

The role of bilateral adrenalectomy in the treatment of congenital adrenal hyperplasia

Van Wyk, Judson J., Ritzen, E. Martin·2003·The Journal of Clinical Endocrinology & Metabolism
DOI 10.1210/jc.2002-022026
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Abstract

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.

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Crossref · 10.1210/jc.2002-022026

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19 of 19 references verified0 citation errors · 0 not checkable

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  • Bilateral adrenalectomy has been successfully used to treat female infertility with uncontrolled hyperandrogenism in 21OH deficiency and uncontrolled hypertension in 11βOH deficiency.

    “Bilateral adrenalectomy has been successfully used to treat female infertility with uncontrolled hyperandrogenism in 21OH deficiency 87 and uncontrolled hypertension in 11βOH deficiency.88”

    From Congenital adrenal hyperplasia (2017) · cites this paper as [87]

  • Case reports and smaller case series suggest adrenalectomy might be a therapeutic option to tackle excess androgen in CAH, reducing need for supraphysiological glucocorticoid replacement.

    “Case reports and smaller case series suggest that adrenalectomy might be a therapeutic option to tackle the excess androgen feature of the disease, thereby reducing the need for supraphysiological glucocorticoid replacement therapy142–144.”

    From Management challenges and therapeutic advances in congenital adrenal hyperplasia (2022) · cites this paper as [143]

  • Eight patients (44%) had high steroid precursors after surgery while receiving a lower hydrocortisone dose, probably from adrenal rests, and needed higher hydrocortisone doses.

    “Eight patients (44%) had elevated steroid precursors postoperatively while on a reduced HC dose, presumably from adrenal rests, which required increased HC doses.”

    From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [330]

  • Every patient in that series described subjective postoperative benefits, such as weight loss, less need for frequent monitoring, and fewer signs and symptoms of androgen excess.

    “All patients reported subjective benefits after surgery, including weight loss, a reduced need for frequent monitoring, and reduced signs and symptoms of androgen excess.”

    From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [330]

  • Adrenalectomy may not fully eliminate hyperandrogenemia because adrenal rest tumors can arise in the testes, ovaries, or retroperitoneum.

    “Adrenalectomy may not totally remove hyperandrogenemia owing to the potential development of adrenal rest tumors in the testes (330), ovaries (332), or retroperitoneum (333).”

    From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [330]

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Authors
Van Wyk, J. J., & Ritzen, E. M.
Affiliations
  • Division of Pediatric Endocrinology (J.J.V.W.), The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599;
  • the Division of Pediatric Endocrinology (E.M.R.), Department of Woman and Child Health, Karolinska Institute, S171-76 Stockholm, Sweden
Venue
The Journal of Clinical Endocrinology & Metabolism
Publisher
The Endocrine Society
Published
2003-07-01
Volume
88
Issue
7
Pages
2993-2998
ISSN
  • 0021-972X (ISSN-L)
  • 1945-7197
DOI
10.1210/jc.2002-022026
OpenAlex
W2067662698
Cited by
89
References
21
Citations by year
2026: 1 · 2025: 2 · 2024: 3 · 2023: 1 · 2022: 3 · 2021: 3 · 2020: 2 · 2019: 4 · 2018: 3 · 2017: 3 · 2016: 7 · 2015: 5 · 2014: 2 · 2013: 6 · 2012: 7
FWCI
1.15
Type
journal article
Language
en
Open access
closed
Locations
  • The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • Karolinska Institutet
  • Vetenskapsrådet
  • Gillings School of Public Health
  • University of North Carolina at Chapel Hill
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal Regulation and Hypertension
Keywords
congenital adrenal hyperplasia, bilateral adrenalectomy, CYP21 mutations, adrenal crisis, hyperandrogenism, obesity, laparoscopic adrenalectomy, long-term follow-up
MeSH terms
Adolescent, Adrenal Hyperplasia, Congenital, Adrenalectomy, Adult, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Male, Postoperative Complications, Quality of Life, Treatment Outcome, Patient Satisfaction

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