Development Disorders of the Vagina & Vulva

DEFINITION

There are a variety of structural abnormalities that occur during fetal development.

CAUSES AND RISKS ARE AS FOLLOWS:

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, genetics 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 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.

And example of this is true hermaphrodites. 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

Symptoms vary according to the abnormally present. Abnormalities include:

  • Imperforate hymen – An adolescent girl may complain of recurrent pelvic pain and lack of menstrual periods. Urinary retention may also occur.
  • Vaginal septum (complete-An adolescent girl may have those symptoms similar to imperforate hymen. Incomplete vaginal septum may allow adequate menstrual flow but later may cause pain with intercourse.
  • Menstruation, absent – This may also be associated with this disease.
    Signs and tests.

Inspection of the external genitalia may reveal:

  • One side of labia larger than the other, or unusually large on both sides.
  • Vaginal opening extremely close to the urethra or anus.
  • Urethra located on the clitoris.
  • Enlarged clitoris.

Examination of the vagina may reveal:

  • Absence of, or partially-formed, vagina.
  • Fusion of the labia.
  • Abnormal septum (“wall” of connective tissue) in the vaginal canal that may either partially or completely divide the vagina horizontally or vertically.
  • Imperforate hymen (solid membrane obstructing the vaginal opening). In preadolescent girls, secretions may accumulate causing vaginal distension and a shiny, bulging protuberance at the vaginal opening. In adolescent girls, menstrual blood accumulates causing a purplish-red, bulging protuberance at the vaginal opening.

TREATMENT

Early recognition of developmental disorders is important, particularly for those that involve sexual ambiguity.

  • Surgical treatment, indicated in some cases depending upon the abnormality, is usually recommended during the neonatal/infant period. In some instances reconstruction may be deferred until after puberty. Surgical reconstruction is done to most closely associate physical characteristics with the gender role assigned to the individual child. This is best done with the expert advice of a genetics or other specialist after chromosomal studies are completed.
  • Hormonal supplementation may also be necessary depending on the condition present.

PROGNOSIS

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.

These are some of the developmental abnormalities of the female genital system.

The pictures below show VAGINAL SEPTUM

This is a congenital abnormality in the formation of the vagina, the the halves of the vagina fail to fuse completely, this septum causes problems during childbirth and may in some cases make sexual intercourse difficult, Treatment is by surgical excision of the septum and restoration of a normal vagina.

LABIAL FUSION

Labial fusion is a condition that is common in girls below 12 years it is mainly caused by the adhesion of the two labia minora sticking together , closing the vestibulum as a result if unsatisfactory vulval hygiene It is common condition that is easily treated and prevented. It is also called AGGLUTINATION OF THE LABIA MINORA.

ABSENT VAGINA-
-MAJOR ROKITANTZKY-KUSTER-HAUSER SYNDROME

CONGENITAL ABSENCE OF THE VAGINA

This is a congenital malformation of the upper third of the vagina(mullerian component)The patient usually present with primary amenorhoea or inability to have satisfactory sexual intercourse.

The picture below is the end of the operation that was done to correct the congenital absence of the vagina.

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.

ALL PARENTS ARE ADVISED TO INSPECT THEIR CHILDREN WHEN BATHING THEM AND ASK THEIR CAREGIVER OR GYNAECOLOGIST IF ANY ORGAN SHOWS SOME DEVIATION FROM NORMAL