Ambiguous genitalia

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Treatments and drugs

By Mayo Clinic staff

When treating ambiguous genitalia, the goals are to ensure long-term psychological and social well-being, sexual function, fertility, a gender-appropriate appearance and stable gender identity. The timing of treatment depends on a child's specific situation. Your medical team can explain the options available for your child and suggest a course of action.

Medications
Depending on the severity of the condition, hormone therapy may be enough to correct the initial hormonal imbalance. In a genetic female with a slightly enlarged clitoris caused by a minor to moderate case of congenital adrenal hyperplasia, proper levels of hormones may shrink the tissue close to a normal size.

Surgery
The goal of reconstructive surgery may be cosmetic, to make the genitals look natural. Surgery can also be done with the aim of preserving normal sexual functioning. Many doctors prefer to postpone surgery done for cosmetic reasons alone until the person with ambiguous genitalia is mature enough to participate in the decision about sex assignment.

For girls with ambiguous genitalia, the sex organs often work normally despite the ambiguous outward appearance. If a girl's vagina is hidden under her skin, surgery in childhood can help with sexual function later. For boys, surgery to reconstruct an incomplete penis can provide a natural appearance and erectile functionality.

Results of surgery are often satisfying, but repeat surgeries may be needed later. Risks include a disappointing cosmetic result or sexual dysfunction, such as an impaired ability to achieve orgasm.

References
  1. Baskin LS. Abnormalities of sexual determination & differentiation. In: Tanagho EA, et al. Smith's General Urology. 17th ed. New York, N.Y.: McGraw-Hill; 2008. http://www.accessmedicine.com/content/aspx?aID=3131473. Accessed Jan. 4, 2010.
  2. Houk CP, et al. Evaluation of the infant with ambiguous genitalia. http://www.uptodate.com/home/index.html. Accessed Jan. 4, 2010.
  3. Houk CP, et al. Management of the infant with ambiguous genitalia. http://www.uptodate.com/home/index.html. Accessed Jan. 4, 2010.
  4. Cunningham FG, et al. Fetal growth and development. In: Cunningham FG, et al. Williams Obstetrics. 23rd ed. New York, N.Y.: Mc-Graw Hill; 2005. http://www.accessmedicine.com/content.aspx?aID=6037835. Accessed Jan. 4, 2010.
  5. Kajbafzadeh A-M, et al. Detubularized pedicled vaginal onlay flap urethroplasty: A new method for hypospadias repair of ambiguous genitalia. The Journal of Urology. 2008;180:2159.
  6. Hackam DJ, et al. Pediatric surgery. In: Brunicardi FC, et al. Schwartz's Principles of Surgery. 9th ed. New York, N.Y.: Mc-Graw Hill; 2010. http://www.accessmedicine.com/content.aspx?aID=5029784. Accessed Jan. 4, 2010.
  7. Diamond DA. Sexual differentiation: Normal and abnormal. In: Wein AJ, ed. Campbell-Walsh Urology. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/177172771-6/935283878. Accessed Jan. 4, 2010.
  8. Crouch NS, et al. Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia. The Journal of Urology. 2008;179:634.
DS00668 Jan. 29, 2010

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