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

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.

See also: Hermaphroditism

Symptoms may include:

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

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

• 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)

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 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.

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

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.

Updated 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.