21-hydroxylase deficiency surgery: Difference between revisions

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Latest revision as of 15:31, 24 July 2020

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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@@ Line 1: / Line 1: @@
 __NOTOC__
-{{Congenital adrenal hyperplasia due to 21-hydroxylase deficiency}}
+{{21-hydroxylase deficiency}}
+{{CMG}} ;{{AE}}{{MJ}}
+== Overview ==
+Reconstructive [[surgery]] and surgical [[adrenalectomy]] are the surgical procedures used for patients with 21-hydroxylase deficiency. Reconstructive [[Gynaecology|gynecologic]] [[surgery]] may be used in [[Adolescent|adolescents]] or [[Adult|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 [[Gynaecology|gynecologic]] [[surgery]] may be used in [[Adolescent|adolescents]] or [[Adult|adults]], including clitoroplasty and [[vaginoplasty]]. <ref name="pmid9156043">{{cite journal| author=Premawardhana LD, Hughes IA, Read GF, Scanlon MF| title=Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience. | journal=Clin Endocrinol (Oxf) | year= 1997 | volume= 46 | issue= 3 | pages= 327-32 | pmid=9156043 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9156043  }}</ref>
-{{CMG}} {{AE}} {{AAM}}
+=== '''Surgical adrenalectomy''' ===
+* Surgical adrenalectomy can be done in some cases, if there is no response to medical therapy.
-==Overview==
+* The major benefit is lowering [[Adrenal gland|adrenal]] [[androgen]] and [[progesterone]] secretion. However, it increases dependency on [[glucocorticoid]] and [[mineralocorticoid]] replacement therapy. <ref name="pmid22904181">{{cite journal| author=Crocker MK, Barak S, Millo CM, Beall SA, Niyyati M, Chang R et al.| title=Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia. | journal=J Clin Endocrinol Metab | year= 2012 | volume= 97 | issue= 11 | pages= E2084-9 | pmid=22904181 | doi=10.1210/jc.2012-2298 | pmc=3485588 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22904181  }}</ref>
-[[Surgery]] is not the first-line treatment option for patients with congenital adrenal hyperplasia due to 21-hydroxylase deficieny. Surgical reconstruction of abnormal genitalia is usually reserved for  severely virilized girls.
-==Surgery==
-===Early-onset: Severe 21-hydroxylase deficient congenital adrenal hyperplasia===
-====Reconstructive surgery====
-Surgery is not recommended for genetically male (XY) infants because the excess androgens do not produce anatomic abnormality. However, [[intersex surgery|surgery]] for severely virilized XX infants is often performed and has become a subject of [[history of intersex surgery|debate]] in the last decade.
-Surgical reconstruction of abnormal genitalia has been offered to parents of severely virilized girls with congenital adrenal hyperplasia since the first half of the 20th century. The purposes of surgery have generally been a combination of:
-# To make the external genitalia look more female than male
-# To make it possible for these girls to participate in normal sexual intercourse when they grow up
-# To improve their chances of fertility
-# To reduce the frequency of urinary infections
 == References ==
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+{{WH}}
-{{WikiDoc Sources}}
+{{WS}}
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 [[Category:Genetic disorders]]
 [[Category:Intersexuality]]
+[[Category:Medicine]]
+[[Category: Up-To-Date]]