Does your baby have a urethra and a vagina? (Urogenital Sinus) Let's talk about this!

How much does it worry a mother or father when they look at a newborn baby and see some small change in their body? Sometimes, even something that is very rare, can affect our baby and make us feel very sad. Today, we are going to talk about a condition that is rarely heard about, but is very important to know about. We call this (Urogenital Sinus).

What is this (Urogenital Sinus)? Let's understand it simply?

Simply put, Urogenital Sinus is a congenital defect in the urinary system and reproductive system of a female. Now, look, normally a female baby has three openings for waste to exit the body: one is the rectum, where stool comes out, the other is the urethral opening, where urine comes out, and the third is the vagina . However, a baby with Urogenital Sinus has two openings. One is the rectum, where stool comes out, and the other is a common opening for both the urethra and vagina. This means that urine comes out and connects to the vagina from the same place.

This (Urogenital Sinus) condition occurs during the early stages of fetal development. Imagine, for a very short time in the fetal stage, all three systems – the intestinal tract (rectum), the genital tract (vagina), and the urinary tract (urinary bladder) – are all connected to a single cavity and opening. We call this ( Cloaca ). As the fetus grows, these three systems separate and form three separate openings (vagina, urethra , and anus). But sometimes, for some reason, the urinary tract and reproductive system – specifically the urethra and vagina – do not separate properly, but continue to maintain a single opening. That common opening is called (Urogenital Sinus). Some people also call this (Persistent Urogenital Sinus - PUGS).

What usually causes the Urogenital Sinus?

During normal development, this area (called the Urogenital Sinus) will later become the urethra and the skin around the opening of the vagina (vulvar vestibule). The urethra is the tube through which urine leaves your body. The skin around the opening of the vagina (vulvar vestibule) is the skin on the inside of the labia, between the urethra and the opening of the vagina.

Where is the Urogenital Sinus located in the body?

This (Urogenital Sinus) is connected to the urethra and vagina.

Are there types of Urogenital Sinus?

Yes, there are two main types of Urogenital Sinus. Identifying this is very important for planning treatment.

1. Low-joined / Low confluence: In this type, the urethra and vagina join very close to the external opening of the body, that is, at the bottom. Therefore, this common passage is very short. In this case, the urethra and vagina are very similar in size and position to normal. This is a slightly less serious condition.

2. High-joined / High confluence: In this case, the urethra and vagina join at a distance from the external opening of the body, that is, at the top. Then this common passage is very long. This type of high confluence is the most serious. Because, other complications can occur with this. For example, the anus can be located much further forward than normal in the body.

How rare is this (Urogenital Sinus) condition?

Urogenital Sinus is a very rare condition . Roughly speaking, it affects about 0.6 out of 10,000 newborns.

What are the symptoms of Urogenital Sinus?

Symptoms of Urogenital Sinusitis can vary depending on the severity of the condition. Some of the most common symptoms include:

• Having only one outlet for feces and one other outlet common to both the vagina and urethra.
• Swelling of the vagina due to accumulation of urine. This is also called (Hydrocolpos).
• The vagina may be abnormally closed.
• Sometimes there may be no vagina at all.
• The uterus, ovaries, or fallopian tubes are not developed properly.

What are the causes of Urogenital Sinus?

To be honest, even medical experts find it difficult to pinpoint a definitive cause for Urogenital Sinus.

In some cases, this can be linked to a condition called Congenital Adrenal Hyperplasia (CAH). CAH is a group of genetic diseases. If you have CAH, one or both of your adrenal glands do not produce an enzyme needed to make hormones. This can cause the sex organs to develop abnormally. CAH can affect anyone, but girls are more likely to have abnormal sex organs.

A condition called virilization can also cause urogenital sinusitis. Virilization is when a boy has too many male sex hormones (androgens) in his body. Androgens are what help the body mature, and they also affect things like deepening of the voice, body hair, and muscle growth.

How is Urogenital Sinus disease diagnosed?

(Urogenital Sinus) is extremely important in determining how to treat the condition. This can affect not only your child's genitals, but also their overall growth and development.

Sometimes, doctors can detect this condition (Urogenital Sinus) during an ultrasound while the baby is still in the womb. However, most often, doctors find it during the first physical exam after the baby is born. They may then do blood tests to learn more about the baby's overall health and genetic problems.

The doctor may order further tests to confirm the diagnosis or to learn more about the extent of the Urogenital Sinus. These may include:

• Ultrasound: This uses high-frequency sound waves to take a video or pictures of the organs inside the body. This can show problems with your baby's kidneys, bladder, vagina, and rectum. It can also detect if your baby has a buildup of urine in the vagina (hydrocolpos).
• Retrograde genitogram: This is also a type of imaging test. In this test, a doctor injects a special dye into the urethra/vagina and takes X-rays. This dye allows the size, shape, and location of tissues and organs to be clearly identified.
• Magnetic Resonance Imaging (MRI) Test: An MRI uses a magnet, radio waves, and a computer to take clear pictures of the body. This can provide very detailed images of the Urogenital Sinus. MRI can also be used to check for any problems with the child's pelvis and spine.
• Endoscopy: An endoscopy uses a thin, long tube (endoscope) with a light and camera on one end. This endoscope is inserted into the common urethra/vagina to look at the anatomy inside.

How to treat Urogenital Sinus?

The only way to treat a Urogenital Sinus condition is to have surgery to separate your baby's vagina and urethra. Sometimes, especially in children who are virilized (due to Congenital Adrenal Hyperplasia (CAH)) and have been diagnosed with Disordered Sexual Development (DSD), there can be mixed opinions about performing surgery. Therefore, before having surgery, you should talk in great detail with the medical team treating your child. This team usually includes:

• A doctor who specializes in diseases of the urinary system and reproductive system ((Urologist)).
• An endocrinologist is a doctor who specializes in diseases related to the endocrine system. Your endocrine system is what makes and releases hormones.

The surgery is usually performed within the baby's first year . This surgery is performed by a urologist.

For a low-joined (Urogenital Sinus) condition, a (Urologist) will most often perform a surgery called (Flap vaginoplasty). In this, they create two separate openings to the outside of the body – one for the vagina, and the other for the urethra.

For a high-joined (Urogenital Sinus) condition, a (Urologist) usually performs a surgery called (Pull-through vaginoplasty). This is a slightly more complicated surgery. In this, the (Urologist) separates the vagina from the common opening, creates a separate one, and closes that common opening. Then, urine continues to flow out of the place where the common opening used to be.

In some cases of high-joined (Urogenital Sinus), a (Urologist) may perform a procedure called (Urogenital mobilization) along with (Flap vaginoplasty). During (Urogenital mobilization), they will:

• The rectum is separated.
• The urethra and vagina are separated and mobilized together and brought to the perineum.
• The rectum is connected to the anal sphincter.
• Then (flap vaginoplasty) is performed.

Some children may also need to have a surgery called Clitoroplasty and/or Labiaplasty. Clitoroplasty involves reshaping the tissue to create a normal clitoris. The surgeon tries to preserve as many nerves as possible so that the child can have a healthy sex life when they grow up. Labiaplasty involves reshaping the labia, which are the lips around the vagina.

How long does it take for a baby to recover after Urogenital Sinus treatment?

Your child will need to stay in the hospital for a few days after the (Urogenital Sinus) surgery. The pain should start to subside within a week, and the child should be completely healed within a month. However, depending on the severity of your child's (Urogenital Sinus), your child's doctor will give you a clearer idea of ​​this.

Can Urogenital Sinus be prevented?

If someone in your family has a condition called Congenital Adrenal Hyperplasia (CAH), and you are pregnant or trying to get pregnant, it is important to talk to your doctor. To prevent a Urogenital Sinus, hormones can be given prenatally.

What should I expect if my child has Urogenital Sinus?

Your child's outcome depends on the size of his or her urogenital sinus. The goal of surgery is to give your child normal function and appearance of the genitals. Most children who have surgery are able to urinate normally after recovery.

As the baby grows, the vagina may become narrower (vaginal stenosis) and shorter. This can cause problems during vaginal sexual intercourse.

A child with a Urogenital Sinus condition does not prevent them from becoming pregnant. However, if the surgery was very extensive, it may be best to have a cesarean section (C-section) during delivery. Your doctor will discuss this with your child at an appropriate time.

How do I take care of my child?

Your child's doctors will tell you how best to treat him. The severity of the (Urogenital Sinus) and the results of the surgery will determine how you will continue to care for him.

When should I see a doctor?

After surgery, your child will need to have regular follow-up appointments to make sure their body is healing properly and functioning as expected. Be sure to attend all of these appointments.

If you notice any new symptoms, such as urinary incontinence, talk to your child's pediatrician or urologist.

What questions should I ask my child's doctor?

Here are some questions you can ask your doctor:

• What type of Urogenital Sinus does my child have?
• What surgery do you recommend?
• At what age is surgery recommended?
• Will my child be in pain, or is he already in pain?
• Will the child need more surgeries as he grows older?
• Do you expect your child to have problems with their urethra or vagina as they grow up?
• Will this affect my child's menstrual cycle?
• Will this affect my ability to conceive a child?
• Will my child be able to give birth safely?

Important: Never be afraid to ask your doctor any questions you have, no matter how small. You have the right to know everything clearly.

What is a Urogenital Disorder in general?

A Urogenital Disorder is any condition that affects your urinary system and reproductive system. Other examples include:

• Benign tumors.
• Hernia.
• Sexually Transmitted Infections (STIs).
• Cancers such as bladder cancer, ovarian cancer, or uterine cancer.
• Interstitial cystitis/bladder pain syndrome (IC/BPS).

Finally, things to remember (Take-Home Message)

It's normal to feel shocked and scared when you find out that your child has a Urogenital Sinus. But remember, this condition can be treated with surgery to separate your child's urethra and vagina. This means that your child should be able to urinate comfortably. As she gets older, she should also start menstruating. However, sometimes vaginal sex can be difficult as she gets older.

The important thing is, you don't have to deal with these things alone. Talk to your child's doctor. They can answer all your questions, ease your fears, and help you get the best treatment and care for your child.

` Urogenital sinus, PUGS, congenital defects, urinary system, reproductive system, surgery, child health