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Developmental disorders of the vagina and vulva
DefinitionDevelopmental disorders of the vagina and vulva include a variety of structural abnormalities that occur during fetal development.
Causes, incidence, and risk factors
With the union of a sperm and an ovum, an embryo acquires the chromosomes that determine the sex of the fetus (XX=female; XY=male). For the first 6 weeks, the sex of the fetus is not distinguishable.
At about 6 weeks gestation, ovaries begin to develop in those with two X chromosomes, and testicles develop in those with an X and Y chromosome. Sexual ambiguity may arise when additional X chromosomes are present, or the Y chromosome is not functioning correctly.
The X chromosome is the primary determinant of feminine traits. However, geneticists have noted over 200 traits that are thought to be X-chromosome linked (such as hemophilia and color blindness ). In contrast, the Y chromosome has only been linked with testicular formation and the development of male secondary sexual characteristics during puberty.
As fetal development progresses through the pregnancy, problems may arise in the development of internal and/or external sexual organs. Since embryonic tissue is initially the same, with no male and/or female differentiation, such problems may cause genetic males to have "female" genital structures and genetic females to have "male" genital structures.
An example of this is true hermaphroditism. In the past, most of these individuals were raised as males since their external genitalia appeared more masculine. In fact, had early diagnosis been available, most should have been raised as females since they all developed significant breasts and many menstruated. After surgical removal of testicular tissue, some have even become pregnant and delivered normal children.
Symptoms vary according to the abnormality present. Abnormalities include:
Signs and tests
Inspection of the external genitalia, may reveal:
Examination of the vagina may reveal:
Early recognition of developmental disorders is important, particularly for those that involve sexual ambiguity.
Psychosocial support/counseling is required for the parents (and child when applicable) to address concerns and provide anticipatory guidance specific to the child's development.
Optimal outcome is based upon early identification of the abnormalities (during the neonatal period), early chromosomal studies, expert advice, and early intervention with treatment aimed at addressing physical, emotional, and social issues.
Potential complications arise if a diagnosis is made late or in error. Children with apparent gender-specific external characteristics may be found, at puberty, to have internal sexual organ functions specific to the sex opposite from which they were raised. Occasionally, 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 abnormal, observable gynecological structures are present, expected female characteristics (breasts, pubic hair) do not develop in girls at puberty, expected menstrual cycle functions do not develop in girls at puberty, or unexpected male characteristics develop in girls.
Adequate prenatal nutrition and avoiding exposure to illness, medications, and alcohol are all important for adequate fetal growth and development. Developmental disorders may still occur despite the mother's efforts to assure a healthy pregnancy. There is no current means of prevention.
Update Date: 2/1/2002Peter Chen, M.D., Department of Obstetrics & Gynecology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT