Is your baby's belly wrinkled? Is it Prune Belly Syndrome? Let's be aware of this.

When you look at your newborn baby, have you noticed that the skin on his stomach is wrinkled like a prune? Or does his belly look unusually large? It is normal for any mother or father to be scared when they see these things. But this could be a symptom of a rare condition called Prune Belly Syndrome. Don't worry, we will talk about everything clearly and simply.

What is Prune Belly Syndrome?

Simply put, Prune Belly Syndrome is a very rare condition that is present at birth (congenital). The name comes from the fact that the skin on the stomach of these children looks wrinkled and looks like a prune. This is caused by three main factors.

1. Weak or absent abdominal muscles: The baby's abdominal muscles are not fully developed, which causes the skin on the abdomen to appear saggy and wrinkled.

2. Undescended testicles: In boys, the testicles have not descended from the body into the scrotum where they should be.

3. Urinary system problems: There are problems with the way the baby's urinary system, that is, the kidneys and bladder, is formed.

In addition to these three main issues, this condition can also affect other parts of the baby's body. For example, the heart, lungs, intestines, and skeletal system may be affected. This is also called Triad Syndrome or Eagle-Barrett Syndrome.

How common is this condition?

This is a very rare condition. It affects about one in 30,000 to 40,000 newborns. What is particularly striking is that 95% of cases of this condition are reported in boys .

What are the main symptoms of this disease?

There are over 50 symptoms of Prune Belly Syndrome. However, these symptoms can vary from baby to baby. Your doctor will examine your baby and perform the necessary tests to confirm the condition.

Let's look at some of the main features that are commonly seen.

Why is this happening? What is the reason?

Researchers haven't really found a reason for this yet, but there are several theories that suggest it could be the cause.

• Bladder problems: Some people believe that this condition is caused by an abnormality in the bladder that develops while the baby is growing in the womb. Urine cannot flow properly and accumulates, causing the bladder, ureters, and kidneys to swell. As the bladder grows, it puts pressure on the abdominal muscles, causing them to atrophy.
• Muscle development problem: Others believe that the primary cause is that the baby's abdominal muscles are not developing properly.
• Genetic Causes: It is believed that this condition may have a genetic influence, as it is seen in siblings. Prune Belly Syndrome is also seen in children with genetic conditions such as Trisomy 18 (Edwards syndrome) and Down syndrome (Trisomy 21).

What complications can occur due to this condition?

There are many side effects and complications that come with Prune Belly Syndrome. It varies from baby to baby. These are the main complications that can be seen.

• Constipation: Difficulty passing stool due to weak stomach muscles.
• Bone deformities: things like hip dislocation, missing hands, fingers, or toes.
• Urinary tract diseases: Frequent occurrence of urinary tract infections (UTIs).
• Infertility or cancer: Failure to descend the testicles may lead to infertility or a risk of cancer in the future.
• Reduced lung development: Lungs not developing properly (pulmonary hypoplasia).
• Chronic kidney failure: This is the most severe complication.

How to diagnose this condition?

In most cases, this condition can be detected early during a scan (fetal ultrasound) you have during your pregnancy. The scan allows the doctor to see the baby's:

• Bone or muscle abnormalities
• Heart problems
• Low lung development
• Digestive system problems
• The bladder is swollen.
• The kidneys are enlarged.

After the baby is born, the doctor may suspect that the baby has this condition just by looking at it. To confirm that suspicion, several tests can be performed, such as:

• Blood tests
• Ultrasound scan
• X-ray
• CT scan
• MRI scan
• Voiding cystourethrogram (VCUG) - This involves injecting a special fluid into the bladder and taking X-rays while the baby urinates to check the function of the urinary system.

How is it treated?

The treatment for this varies from child to child. A team of specialists will work together to develop the most appropriate treatment plan for the child. This will take into account many factors, including the child's age, health status, severity of the condition, and the parents' wishes.

• Mild cases: Some children whose symptoms are not as severe may benefit from antibiotics alone to prevent or treat urinary tract infections.
• Surgery: However, many children will need multiple surgeries to repair the abdominal wall, genitals, bladder, and other parts of the urinary system.

These are some of the main types of surgeries performed:

• Vesicostomy: A small opening is made through the abdomen into the bladder, allowing urine to drain through it.
• Orchiopexy: The testicles are surgically brought down and placed in the scrotum.
• Cystoplasty: A more complex surgery that completely reconstructs the bladder.

In some severe cases, the child may also need a kidney transplant .

What will the future of these children be like?

The life expectancy of these children depends on the severity of the disease. Sadly, between 10% and 25% of children born with this condition die in the womb or die from complications within the first few weeks of life.

However, approximately 40% of children with Prune Belly Syndrome have normal kidney function and can live a fairly normal life.

Another 30% will need a kidney transplant at some point in their lives.

What are the best times to see the doctor?

Children with Prune Belly Syndrome are at high risk for urinary tract infections (UTIs), so if you notice any signs of a urinary tract infection, call your doctor immediately.

Additionally, seek medical advice if your child has any other difficulty urinating or shows any other unusual symptoms. It is also very important to take your child for a medical check-up at least once a year .

Caring for a baby born with this condition can be challenging. But remember, you are not alone. Working with a doctor and a team of specialists who are knowledgeable about this condition can give you and your baby the strength and guidance they need.