The joy you feel as a parent when you look at a newborn baby is indescribable. But sometimes, when the doctor examines the baby and tells you that there is something wrong with his body, you may feel a little scared and worried. Today, we are talking about a very rare, but important birth defect. This is called Urogenital Sinus.
What is this Urogenital Sinus?
Simply put, this is a congenital defect that only affects girls . Imagine, a girl usually has three openings in her lower body.
1. The opening of the urinary tract (urethra) through which urine exits.
2. Vaginal opening (vagina).
3. The opening in the rectum (anus) through which feces exit.
However, a child with Urogenital Sinus has two openings. One is for the exit of feces. The other opening is a common opening formed by the union of the urethra and vagina. This means that both the urethra and the vagina are connected to this same opening.
Why is this happening?
To understand this, we need to go back to the early stages of a baby's development in the womb. When a baby is developing as a fetus, the three systems we mentioned (the intestinal system, the urinary system, and the reproductive system) initially join together and open into a common chamber. This is called the ``cloaca''. As the baby grows in the womb, these three systems gradually separate and form three separate openings. But sometimes, for some reason, the urinary system and the vagina do not separate properly. That's when they join together and form a condition called the ``Urogenital Sinus.''
Are there main types of Urogenital Sinus?
Yes, this condition can be divided into two main types, depending on how long the common pathway is and where in the body it connects.
| Type | Simple explanation |
|---|---|
| Low-joined / low confluence | In this type, the urethra and vagina join very close to the external opening of the body. Therefore, the common passage is very short . The urethra and vagina may be of normal size and position. This is a slightly less serious condition. |
| High-joined / high confluence | Here, the two paths join inside the body, away from the external opening. Therefore, the common path is much longer . This is considered a more serious condition . Because it is associated with a higher risk of other complications. |
This is a very rare condition. It usually occurs in less than one in 10,000 births. So don't be alarmed by this news.
What are the symptoms of this?
Symptoms can vary depending on the severity of the condition. These are often seen when a doctor examines the baby after birth.
- The main feature is that, as we mentioned earlier , there are only two openings : one for feces and the other as a common opening for the urethra and vagina.
- Urine collects inside the vagina, causing the vagina to swell. This is medically called ``hydrocolpos``.
- Sometimes the vagina may not be fully developed.
- You may also see internal organs such as the uterus, ovaries, or fallopian tubes completely missing.
Why is this happening? What are the reasons?
It's often difficult to pinpoint a specific cause, but there are a few main causes that doctors have identified.
- Congenital Adrenal Hyperplasia (CAH): This is a genetic condition. In this condition, the baby's adrenal glands do not produce an enzyme needed to produce hormones. This can cause hormonal imbalances and abnormal development of the genitals, especially in girls.
- Virilization: Simply put, it is the excessive amount of male hormones (androgens) in a female fetus. This can also affect the development of the genitals.
How do doctors diagnose this condition?
Diagnosing this condition correctly is very important for planning treatment. Sometimes it can be detected during prenatal ultrasound scans. But most of the time, the doctor notices this change during the first physical examination after the baby is born . Then, several more tests are ordered to confirm the condition and determine the severity.
| Test | Simply put, what do you do with it? |
|---|---|
| Ultrasound Scan | This helps to see the condition of the baby's internal organs, such as the kidneys, bladder, vagina, and rectum. It can also check for hydrocolpos (swelling of the vagina). |
| Retrograde Genitogram | This is a special X-ray test. The doctor inserts a special dye through the common opening and takes X-ray pictures. This allows the shape, size, and position of the organs inside to be clearly seen. |
| MRI Scan | This allows for very clear, detailed images of the inside of the body. It can also show the exact location of the Urogenital Sinus and any other problems that may be present, such as the surrounding spine. |
| Endoscopy | This method uses a thin tube (endoscope) equipped with a camera and light to be inserted through the common orifice and to view the inside directly on a screen. |
How is it treated?
The only way to treat this condition is to surgically separate the vagina and urethra .
This is a very complex decision. Before the surgery, you will have a long discussion with your child's medical team. This team usually includes a doctor who specializes in the urinary and reproductive systems (a `Urologist`) and a doctor who specializes in hormones (an `Endocrinologist`).
This surgery is usually done within the first year of a child's life . The type of surgery performed depends on whether the condition is `low-joined` or `high-joined`, which we discussed earlier.
- For low-jointed conditions: A surgery called `flap vaginoplasty` is often performed. In this, the surgeon uses part of the common opening to create two separate openings for the vagina and urethra.
- For high-joined conditions: A more complex surgery, such as a `pull-through vaginoplasty`, is required. Here, the vagina is separated from the common passage and a separate opening is created for it.
Some children may also need to undergo surgery, such as a `clitoroplasty` or `labiaplasty`, to restore the shape of the clitoris or labia to normal.
Will this affect my child's future?
This is a big question for every parent. The main goal of the surgery is to allow the child to perform normal bodily functions and restore the appearance of the genitals to normal.
- Urination: After surgery, most children are able to urinate normally without any problems .
- Menstrual cycle: This will not affect the normal menstrual cycle after the child grows up.
- Sex life: Sometimes, as the baby grows, the vagina can become a little narrower or shorter (vaginal stenosis). This may cause some difficulties during sexual intercourse in the future. You can talk to your doctor about this and seek necessary treatment.
- Pregnancy: Having this condition does not mean that a child will not be able to get pregnant in the future. However, if the surgery is very complicated, doctors may recommend a cesarean section (C-section) when the baby is born.
These things may vary depending on your child's condition, so it's best to discuss everything openly with your doctor.
Questions to ask the doctor
Ask your doctor about all your questions and fears. For example, you can ask questions like these:
- What type of Urogenital Sinus does my child have?
- What kind of surgery do you recommend? At what age is it best to have it done?
- Is my child in pain now?
- Will more surgeries be needed as the child grows?
- Will this affect the child's menstrual cycle?
- Will this affect adopting a child in the future?
Take-Home Message
- Urogenital sinus is a rare birth defect in girls, where the urethra and vagina connect to a single common opening.
- This condition is often diagnosed during an examination by a doctor shortly after the baby is born.
- The only treatment for this is to surgically separate the two pathways. This is an important decision that the parents and medical team make together.
- After proper surgery, most children can lead normal lives. They are able to urinate normally.
- Talk openly with your child's doctor about any doubts or fears you may have. They will provide you with the best possible guidance.
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