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Developmental disorders of the female reproductive tract - Treatment

Treatment:

Doctors often recommend surgery for girls with developmental problems of the internal reproductive organs. For example, a blocked vagina can usually be corrected with surgery.

If the baby girl is missing a vagina, the doctor may prescribe a dilator when the child reaches young adulthood. A dilator is a device that helps stretch or widen the area where the vagina is supposed to be. This non-surgical process takes from 4 to 6 months. Surgery may also be done to create a new vagina. Surgery should be done when the young woman is able to use a dilator to keep the new vagina open.

Doctors have reported good results with both surgical and non-surgical techniques.

Treatment of cloacal abnormalities usually involves multiple complex surgeries to fix any problems with the rectum, vagina, and urinary tract.

If the birth defect causes life-threatening complications, the first surgery is done shortly after birth. Surgeries for other developmental reproductive disorders may also be done while the baby is an infant. Some surgeries may be delayed until the child is much older.

Early recognition is important, particularly in cases of ambiguous genitalia. Careful consideration should be given before assigning a gender -- that means, before you decide that child is a boy or girl. Treatment should include counseling for the parents and, eventually, the child. For specific treatment information, see the articles on intersex and ambiguous genitalia.

Support Groups:

Support is important for families of children who are diagnosed with abnormalities of the sexual and reproductive organs. Experts also recommend counseling and support groups for the children themselves, as they get older.

Different support groups may differ in their thoughts regarding this very sensitive topic. Look for one that supports your thoughts and feelings on the topic.

Support organizations include:

  • C.A.R.E.S Foundation -- www.every1cares.org
  • Congenital Adrenal Hyperplasia Education and Support Network -- www.congenitaladrenalhyperplasia.org
  • DES Action USA -- www.desaction.org
  • Intersex Society of North America -- www.isna.org

Expectations (prognosis):

With expert advice, early treatment, and counseling, the outcome is usually excellent.

Complications:

Cloacal abnormalities can cause life-threatening complications at birth.

Potential complications may develop if the diagnosis is made late or is wrong. Children with ambiguous genitalia who are assigned one gender may later be found to have internal organs related to the sex opposite from which they were raised. This can cause severe psychological distress.

Undiagnosed problems in a girl's reproductive tract can lead to infertility and sexual difficulties.

Other complications that occur later in life include:

  • Endometriosis
  • Going into labor too early (preterm delivery)
  • Painful abdominal lumps requiring surgery
  • Repeated miscarriages

Calling your health care provider:

Call for an appointment with your health care provider if your daughter has:

  • Abnormal looking genitals
  • Male characteristics
  • Monthly pelvic pain and cramping, but does not menstruate
  • Not started menstruation by age 16
  • Not started developing breasts at puberty
  • Unusual lumps in the abdomen or groin

Call your health care provider if you are a woman who:

  • Has a mother who used DES while she was pregnant with you
  • Has had repeated miscarriages or several preterm deliveries
  • Reviewed last on: 5/12/2008
  • Linda Vorvick, MD, Seattle Site Coordinator, Lecturer, Pathophysiology, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine; Susan Storck, MD, FACOG, Clinical Teaching Faculty, Department of Obstetrics and Gynecology, University of Washington School of Medicine, Chief, Eastside Department of Obstetrics and Gynecology, Group Health Cooperative of Puget Sound, Redmond, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Diamond DA. Abnormal Sexual Differentiation. Evaluation and Management of the Newborn With Ambiguous Genitalia. In: Wein AJ, ed. Campbell-Walsh Urology. 9th ed. Philadelphia, PA: Saunders Elsevier; 2007:chap 128.

Emans SJ, Laufer MR, Goldstein DP. Pediatric and Adolescent Gynecology. 5th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2005.

Katz VL, Lentz GM. Congenital Abnormalities of the Female Reproductive Tract. In: Gabbe SG, Niebyl JR, Simpson JL, eds. Comprehensive Gynecology. 5th ed. Philadelphia, PA: Mosby Elsevier; 2007: chap 12.

Katz VL. Spontaneous and Recurrernt Abormtion: Etiology, Diagnosis, Treatment. In: Gabbe SG, Niebyl JR, Simpson JL, eds. Comprehensive Gynecology. 5th ed. Philadelphia, PA: Mosby Elsevier; 2007: chap 16.

Niebyl JR, Simpson JL. Drugs and Environmental Agents in Pregnancy and Lactation: Embryology, Teratology, Epidemiology. In: Gabbe SG, Niebyl JR, Simpson JL, eds. Comprehensive Gynecology. 5th ed. Philadelphia, PA: Mosby Elsevier; 2007: chap 8.

Park JM. Normal Development of the Urogenital System. In: Wein AJ, ed. Campbell-Walsh Urology. 9th ed. Philadelphia, PA: Saunders Elsevier; 2007:chap 106.