What Are Urogenital Sinus Abnormalities?

Female Reproductive System
Female Reproductive System

Female Urinary Tract
Female Urinary Tract
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Normally, the urethra and vagina are separate in females. In rare cases a birth defect causes the urethra and vagina to become one. This is known as urogenital sinus.

There are 2 types of urogenital sinus abnormalities:

  • In a low confluence (joined) case:
    • The common opening is short
    • The urethra is close to its normal place
    • The vagina is almost normal length
  • In a high confluence case:
    • The common opening is long
    • The urethra is inside the body
    • The vagina is short
    • The anus can be too far forward


What are the Symptoms of Urogenital Sinus

Urogenital sinus symptoms depend on how severe the condition is.

During development, the urogenital sinus will form two separate openings. The openings become the urethra or opening of the urinary tract and the vagina. The urogenital sinus links the urethra or opening of the urinary tract and the vagina.

If you have chronic urogenital sinus can cause urine from the bladder to fill the vagina. The filling of the vagina can cause “hydrocolpos.” Hydrocolpos is when the vagina swells because of fluid. Hydrocolpos can be found by an ultrasound. It can also be felt if there is a mass in the abdomen.

The organs outside of the body may appear abnormal. Instead of having two openings, your child may have one single opening. Most of these abnormalities are found in infancy.



What Causes Urogenital Sinus?

The cause of a chronic urogenital sinus is not known.

Virilization of the female genitalia may cause urogenital sinus. Virilization is when a female develops male features. Males features they could get are:

  • Muscle bulk
  • Body hair
  • Deep voice

Virilization is often a result of a too much androgen (male hormones) in the body. This condition can occur if you have congenital adrenal hyperplasia.



How Are Urogenital Sinus Abnormalities Diagnosed?

Physical Exam

This birth defect is most often found in infants during a physical exam. It is often linked to issues called “disorders of sex differentiation .” The doctor will order blood tests to rule out other health problems. Things like congenital adrenal hyperplasia can be tied to this birth defect. Congenital adrenal hyperplasia should be treated right away. Congenital adrenal hyperplasia is a group of genetic conditions. This condition limits your adrenal glands' ability to make certain vital hormones. Often, the adrenal glands do not make enough cortisol. This condition can cause problems with normal growth and development in children – including with the genitals.

Genitogram

If this problem is suspected in a baby, an exam called a retrograde genitogram will be performed. Contrast dye will be injected into the common opening. The doctor will use an X-ray to figure out:

  • How long the opening is
  • How the urethra and the vagina are joined

This will allow the doctor to work out the best treatment.

Endoscopy

If a retrograde genitogram is not clear, endoscopy may be done. In this test a fiber-optic camera is placed into the opening so the anatomy can be seen. In some cases ultrasound and MRI may be used as well.



How Are Urogenital Sinus Abnormalities Treated?

Surgery to separate the vagina and urethra is the only way to fix urogenital sinus. There are 3 parts of the surgery.

Clitoroplasty

This treatment involves reshaping the tissue into a female clitoris. Special care will be taken with the nerves in this tissue so sexual feelings can be experienced. Extra skin is also used to make labia minora.

Labioplasty

This treatment allows the labia majora to be made with nearby skin (that looks like part of the scrotum).

Vaginoplasty

This treatment can be done in many ways. You and your child’s doctor will decide the best plan, based on the type of case.

Flap Vaginoplasty

This treatment is done for a low confluence (joined) case. The surgeon opens the area so that the vagina and urethra have separate outer openings.

Pull-through Vaginoplasty

This treatment is done for a high confluence (joined) case. The vagina is brought to its normal place on the surface of the skin. The urethra will still drain through what had been the common opening. The surgeon may need to use a piece of skin or a section of bowel.



What Can Be Expected After Treatment?

Many parents have questions about their daughter’s quality of life after surgery. If the problem is fixed, she should be able to urinate normally. She can also expect to have a future enjoyable sex life. It will be possible for her to have children. If the surgery was very complex, giving birth by C-section may be best.