Health Encyclopedia

Developmental disorders of the vagina and vulva

Definition

Developmental disorders of the vagina and vulva include many different structural problems that occur while the baby is developing in the mother's womb.

Causes, incidence, and risk factors

As the baby develops during the pregnancy, problems may occur in the development of sexual organs. Sometimes males are born with "female" genitals and females have "male" genitals.

Symptoms

Symptoms may include:

Inability to empty the bladder (urinary retention)
Lack of menstrual periods
Painful intercourse
Pelvic pain that comes back

Signs and tests

Finding problems with development early is important, especially when the gender is unclear (sexual ambiguity).

An examination of the outside (external) genitals may show:

Enlarged clitoris
One side of labia larger than the other, or unusually large on both sides
Opening of the vagina very close to the urethra or anus
Urethra located on the clitoris

An examination of the vagina may show:

Abnormal "wall" of tissue (septum) in the vagina that may either partly or completely divide the vagina across or straight up and down
Blockage of the opening of the vagina (imperforate hymen), and a bulge at the opening of the vagina
Labia that is stuck together (fused labia)
Missing or partially formed vagina

Treatment

Counseling for the parents (and child, if necessary) to address concerns and provide guidance for the child's development
Hormones (depending on the condition)
Surgery when the child is a newborn or infant (or sometimes not until after puberty) to make the genitals match with the child's gender (with the expert advice of a geneticist or other specialist)

Expectations (prognosis)

It helps to find the problem while the child is still a newborn. Getting all of these as soon as possible can provide the child with the best outcome:

Chromosomal studies
Expert advice
Treatment of the physical, emotional, and social concerns

In the past, most hermaphrodites were raised as males because their outside (external) genitals looked more masculine. However, they can grow breasts, and many get their periods (menstruate). After removing the testicles with surgery, some hermaphrodites can become pregnant and deliver normal children.

Complications

Complications can occur if the diagnosis is made late or is not correct.

It is possible for a child who has the outside (external) genitals of one gender to have internal sexual organs of the opposite sex. Sometimes, these internal sexual organs are at risk for cancer and must be surgically removed around the time of puberty.

Calling your health care provider

Call for an appointment with your health care provider if you notice:

Abnormal genitals
Menstruation does not begin at puberty
Pubic hair or breasts do not develop at puberty
Unexpected male traits

Prevention

There is no current way to prevent this condition.

Getting the right nutrition during pregnancy and avoiding exposure to illness, certain medications, and alcohol are all important for the baby to grow and develop. However, development problems may still occur, even if the mother makes every effort to ensure a healthy pregnancy.

Review Date:2/19/2008
Reviewed By:Peter Chen, MD, Department of Obstetrics & Gynecology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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