21-Hydroxylase Deficiency surgery

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21-Hydroxylase Deficiency Microchapters


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Differentiating 21-Hydroxylase Deficiency from other Diseases

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


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 CAH since the first half of the 20th century. The purposes of surgery have generally been a combination of:

  1. to make the external genitalia look more female than male
  2. to make it possible for these girls to participate in normal sexual intercourse when they grow up
  3. to improve their chances of fertility
  4. 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 CAH 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.