AUA Summit - What Are Urogenital Sinus Abnormalities?

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What Are Urogenital Sinus Abnormalities?

As a baby develops, three openings normally form for the intestinal, genital and urinary tracts. In early stages of pregnancy, these three openings are together. They then separate from each other to form the anus, vagina and urethra. In rare cases, this area may develop incorrectly with the urethra and vagina still joined together and draining from one hole. When the urethra and vagina are still joined together, this is called a “common channel” or “persistent or chronic urogenital sinus”.

Symptoms

The severity of the condition depends upon the length of the urogenital sinus or common channel. If the channel is long, urine from the bladder may fill the vagina. A vagina full of fluid (urine) is called “hydrocolpos.” Hydrocolpos can be found by a swelling in the lower abdomen or by ultrasound.

Causes

A common cause of chronic urogenital sinus is congenital adrenal hyperplasia (CAH). This occurs when the child’s adrenal gland makes too much male hormones which cause “virilization” or the formation of male-looking external genitalia in a female child.

Diagnosis

Physical Exam

This birth defect is most often found in infants during a physical exam. The doctor will order blood tests to rule out other health problems. If the child has CAH, the genitalia may look more male-like from the adrenal glands making too much male hormones. Also, the adrenal glands do not make enough of a vital steroid called cortisol. This requires immediate diagnosis and treatment with replacement steroids.

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.

Treatment

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

Vaginoplasty

This treatment can be done in many ways. You and your child’s doctor will decide the best plan, based on the length of the sinus (common channel). The surgery will be more complex if the the confluence (joining) of the urethra and vagina is higher. If the channel is short, the surgeon can often move the urethra and vagina into their appropriate locations. If the channel is long, the surgeon may have to replace the lower part of the vagina with skin or intestine.

Clitoroplasty

If the child has an enlarged clitoris from CAH, the urologic surgeon may offer reconstruction of the clitoris. Special care will be taken with the nerves in the clitoris so sexual feelings can be experienced. Some parents may choose to delay this aspect of the surgery until the child is older.

Labioplasty

If the labia are fused together and enlarged, the urologic surgeon may offer reconstruction of the labia.

After Treatment

Many parents have questions about their child’s quality of life after surgery. If the problem is fixed, she should be able to pass 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.

Updated March 2024. 


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