21-hydroxylase deficiency surgery: Difference between revisions

	Congenital adrenal hyperplasia due to 21-hydroxylase deficiency Microchapters
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Revision as of 15:41, 4 September 2015

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Surgery

Early-onset: Severe 21-hydroxylase deficient congenital adrenal hyperplasia

Reconstructive surgery

Surgery need never be considered for genetically male (XY) infants because the excess androgens do not produce anatomic abnormality. However, surgery for severely virilized XX infants is often performed and has become a subject of 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

Template:WikiDoc Sources

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 # to improve their chances of fertility
 # to reduce the frequency of urinary infections
-In the 1950s and 1960s, surgery often involved clitorectomy (removal of most of clitoris), an operation that also reduced genital sensation. In the 1970s, new operative methods were developed to preserve innervation and clitoral function. However, a number of retrospective surveys in the last decade suggest that (1) sexual enjoyment is reduced in many women even after nerve-sparing procedures, and (2) women with congenital adrenal hyperplasia who have not had surgery also have a substantial rate of sexual dysfunction. (''See [[Intersex surgery]] for an overview of procedures and potential complications, and [[History of intersex surgery]] for a fuller discussion of the controversies. Many patient advocates and surgeons argue for deferring surgery until adolescence or later, while some surgeons continue to argue that infant surgery has advantages.
 == References ==