11β-hydroxylase deficiency surgery: Difference between revisions

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{{Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency}}
{{11β-hydroxylase deficiency }}
{{CMG}}; {{AE}} {{Ammu}}
{{CMG}} {{AE}} {{MJ}}
==Overview==
== Overview ==
The predominant therapy for ambigous genitalia in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is surgical correction.
In patients with 11β-hydroxylase deficiency, girls with [[ambiguous genitalia]] mostly undergo [[reconstructive surgery]] such as clitoroplasty and [[vaginoplasty]].
==Surgery==
== Surgery ==
* Suregry is used to correct ambigous genitalia.
In patients with 11β-hydroxylase deficiency, girls with [[ambiguous genitalia]] mostly undergo [[reconstructive surgery]] such as clitoroplasty and [[vaginoplasty]]. In clitoral reduction surgery [[neurovascular bundle]], the [[glans]], and the preputial skin related to the [[glans]] should be kept completely.<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><ref name="pmid7007606">{{cite journal |vauthors=Hendren WH, Donahoe PK |title=Correction of congenital abnormalities of the vagina and perineum |journal=J. Pediatr. Surg. |volume=15 |issue=6 |pages=751–63 |year=1980 |pmid=7007606 |doi= |url=}}</ref>
==Indications==
 
* Ambigous genitalia
== References ==
==References==
{{Reflist|2}}
{{Reflist|2}}
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Latest revision as of 16:58, 3 August 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

Overview

In patients with 11β-hydroxylase deficiency, girls with ambiguous genitalia mostly undergo reconstructive surgery such as clitoroplasty and vaginoplasty.

Surgery

In patients with 11β-hydroxylase deficiency, girls with ambiguous genitalia mostly undergo reconstructive surgery such as clitoroplasty and vaginoplasty. In clitoral reduction surgery neurovascular bundle, the glans, and the preputial skin related to the glans should be kept completely.[1][2]

References

  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.
  2. Hendren WH, Donahoe PK (1980). "Correction of congenital abnormalities of the vagina and perineum". J. Pediatr. Surg. 15 (6): 751–63. PMID 7007606.