Bilateral Adrenalectomy in Congenital Adrenal Hyperplasia

A Systematic Review and Meta-Analysis

Diana Mackay, Anna Nordenström, Henrik Falhammar

    Research output: Contribution to journalArticleResearchpeer-review

    Abstract

    Context: Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic-pituitary-adrenal axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing syndrome 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. 

    Objective: To review the outcomes after bilateral adrenalectomy for CAH. 

    Data Sources: A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to 10 January 2018. 

    Study Selection: Case reports or case series were included if they reported individual patient data from patients with CAH who had undergone bilateral adrenalectomy. 

    Data Extraction: Information regarding the following was extracted: first author, country, sex, age at adrenalectomy, year of adrenalectomy, diagnosis, molecular abnormality, pre- and postoperative biochemistry, pre- and postoperative medications, pre- and postoperative body mass index, indication for adrenalectomy, surgical technique, gross and microscopic adrenal characteristics, follow-up duration, and short- and long-term postoperative outcomes. 

    Data Synthesis: We identified 48 cases of bilateral adrenalectomy for CAH, with patients aged from 4 months to 56 years at surgery. The most common indication for surgery was the inability to control hyperandrogenism/virilization and/or Cushing syndrome (n = 30; 62%). Most patients (n = 34; 71%) reported symptomatic improvement postoperatively, with some cases of short-term (n = 5; 10%) and long-term (n = 13; 27%) adverse outcomes. 

    Conclusions: Bilateral adrenalectomy for CAH appears to be a reasonable therapeutic option for carefully selected patients who have had unsatisfactory outcomes with conventional medical management.

    Original languageEnglish
    Pages (from-to)1767-1778
    Number of pages12
    JournalJournal of Clinical Endocrinology and Metabolism
    Volume103
    Issue number5
    DOIs
    Publication statusPublished - 1 May 2018

    Fingerprint

    Congenital Adrenal Hyperplasia
    Adrenalectomy
    Surgery
    Glucocorticoids
    Meta-Analysis
    Mineralocorticoids
    Biochemistry
    Cushing Syndrome
    Information Storage and Retrieval
    Virilism
    Hyperandrogenism
    PubMed
    MEDLINE
    Body Mass Index
    Therapeutics

    Cite this

    Mackay, Diana ; Nordenström, Anna ; Falhammar, Henrik. / Bilateral Adrenalectomy in Congenital Adrenal Hyperplasia : A Systematic Review and Meta-Analysis. In: Journal of Clinical Endocrinology and Metabolism. 2018 ; Vol. 103, No. 5. pp. 1767-1778.
    @article{cd4633ee77ac446fbb04cea7182a0869,
    title = "Bilateral Adrenalectomy in Congenital Adrenal Hyperplasia: A Systematic Review and Meta-Analysis",
    abstract = "Context: Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic-pituitary-adrenal axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing syndrome 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. Objective: To review the outcomes after bilateral adrenalectomy for CAH. Data Sources: A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to 10 January 2018. Study Selection: Case reports or case series were included if they reported individual patient data from patients with CAH who had undergone bilateral adrenalectomy. Data Extraction: Information regarding the following was extracted: first author, country, sex, age at adrenalectomy, year of adrenalectomy, diagnosis, molecular abnormality, pre- and postoperative biochemistry, pre- and postoperative medications, pre- and postoperative body mass index, indication for adrenalectomy, surgical technique, gross and microscopic adrenal characteristics, follow-up duration, and short- and long-term postoperative outcomes. Data Synthesis: We identified 48 cases of bilateral adrenalectomy for CAH, with patients aged from 4 months to 56 years at surgery. The most common indication for surgery was the inability to control hyperandrogenism/virilization and/or Cushing syndrome (n = 30; 62{\%}). Most patients (n = 34; 71{\%}) reported symptomatic improvement postoperatively, with some cases of short-term (n = 5; 10{\%}) and long-term (n = 13; 27{\%}) adverse outcomes. Conclusions: Bilateral adrenalectomy for CAH appears to be a reasonable therapeutic option for carefully selected patients who have had unsatisfactory outcomes with conventional medical management.",
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    Bilateral Adrenalectomy in Congenital Adrenal Hyperplasia : A Systematic Review and Meta-Analysis. / Mackay, Diana; Nordenström, Anna; Falhammar, Henrik.

    In: Journal of Clinical Endocrinology and Metabolism, Vol. 103, No. 5, 01.05.2018, p. 1767-1778.

    Research output: Contribution to journalArticleResearchpeer-review

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    T1 - Bilateral Adrenalectomy in Congenital Adrenal Hyperplasia

    T2 - A Systematic Review and Meta-Analysis

    AU - Mackay, Diana

    AU - Nordenström, Anna

    AU - Falhammar, Henrik

    PY - 2018/5/1

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    N2 - Context: Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic-pituitary-adrenal axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing syndrome 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. Objective: To review the outcomes after bilateral adrenalectomy for CAH. Data Sources: A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to 10 January 2018. Study Selection: Case reports or case series were included if they reported individual patient data from patients with CAH who had undergone bilateral adrenalectomy. Data Extraction: Information regarding the following was extracted: first author, country, sex, age at adrenalectomy, year of adrenalectomy, diagnosis, molecular abnormality, pre- and postoperative biochemistry, pre- and postoperative medications, pre- and postoperative body mass index, indication for adrenalectomy, surgical technique, gross and microscopic adrenal characteristics, follow-up duration, and short- and long-term postoperative outcomes. Data Synthesis: We identified 48 cases of bilateral adrenalectomy for CAH, with patients aged from 4 months to 56 years at surgery. The most common indication for surgery was the inability to control hyperandrogenism/virilization and/or Cushing syndrome (n = 30; 62%). Most patients (n = 34; 71%) reported symptomatic improvement postoperatively, with some cases of short-term (n = 5; 10%) and long-term (n = 13; 27%) adverse outcomes. Conclusions: Bilateral adrenalectomy for CAH appears to be a reasonable therapeutic option for carefully selected patients who have had unsatisfactory outcomes with conventional medical management.

    AB - Context: Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic-pituitary-adrenal axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing syndrome 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. Objective: To review the outcomes after bilateral adrenalectomy for CAH. Data Sources: A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to 10 January 2018. Study Selection: Case reports or case series were included if they reported individual patient data from patients with CAH who had undergone bilateral adrenalectomy. Data Extraction: Information regarding the following was extracted: first author, country, sex, age at adrenalectomy, year of adrenalectomy, diagnosis, molecular abnormality, pre- and postoperative biochemistry, pre- and postoperative medications, pre- and postoperative body mass index, indication for adrenalectomy, surgical technique, gross and microscopic adrenal characteristics, follow-up duration, and short- and long-term postoperative outcomes. Data Synthesis: We identified 48 cases of bilateral adrenalectomy for CAH, with patients aged from 4 months to 56 years at surgery. The most common indication for surgery was the inability to control hyperandrogenism/virilization and/or Cushing syndrome (n = 30; 62%). Most patients (n = 34; 71%) reported symptomatic improvement postoperatively, with some cases of short-term (n = 5; 10%) and long-term (n = 13; 27%) adverse outcomes. Conclusions: Bilateral adrenalectomy for CAH appears to be a reasonable therapeutic option for carefully selected patients who have had unsatisfactory outcomes with conventional medical management.

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    JO - Journal of Clinical Endocrinology and Metabolism

    JF - Journal of Clinical Endocrinology and Metabolism

    SN - 0021-972X

    IS - 5

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