Is your baby's belly wrinkled like a prune? Let's talk about Prune Belly Syndrome!

When we look at a newborn baby's belly, we usually see it as very smooth and full, right? But imagine, sometimes, unexpectedly, we see that the skin on the baby's belly is very wrinkled, like a prune. Seeing this, as a mother or father, you may feel very scared and shocked. "Oh, what happened to my baby?" You may think. This is actually a very rare condition. Today we are talking about the rare condition that can cause this, namely Prune Belly Syndrome . Don't worry, we will talk about everything simply and clearly.

Simply put, what is Prune Belly Syndrome?

Prune Belly Syndrome is a rare congenital condition. It gets its name because the skin on a baby's belly looks like a prune. It has three main symptoms. That's why it's sometimes called the 'Triad Syndrome'. It's also called 'Eagle-Barrett Syndrome' in honor of the doctors who discovered it.

These three main features are:

1. Weak or absent abdominal muscles: Our abdomen has a layer of muscles that help keep its shape and protect the organs inside, such as the intestines. In babies with this syndrome, these muscles are not developed properly. Sometimes they are not formed at all. That's why the skin on the abdomen looks wrinkled and saggy.

2. Undescended testicles: In a male baby, the testicles are normally located outside the body in the scrotum. However, in babies with this condition, the testicles are located inside the abdomen. They have not descended.

3. Urinary system problems: This is the most serious part of the condition. The baby's kidneys, bladder, and the tubes that connect them (ureters) are not fully developed. This can cause serious problems with urination.

In addition to these three main symptoms, some babies may also have various problems with the heart, lungs, intestines, and skeletal system.

How common is this condition?

This is a very rare condition. It affects about one in 30,000 to 40,000 newborns. What's special is that 95% of these cases are reported in boys . That's because the undescended testicle problem we talked about earlier only affects boys.

What are the signs that can help you identify if your baby has this condition?

Prune Belly Syndrome is said to have more than 50 symptoms. However, these symptoms can vary from baby to baby. Some babies may be very severely affected, while others may not be so severe. Your doctor will examine your baby and perform the necessary tests to confirm this for sure.

Let's look at the table below to see what are the most common symptoms.

Why does this happen to babies?

To be honest, doctors still haven't found the exact cause of this, but there are several theories.

Others believe that this problem occurs because the abdominal muscles do not develop properly in the first place. Others say that it may be due to a genetic cause. This condition has also been reported among siblings. Also, some children with chromosomal abnormalities such as `Trisomy 18 (Edwards Syndrome)` and `Trisomy 21 (Down Syndrome)` also experience this Prune Belly Syndrome.

What complications can occur due to this condition?

The severity of Prune Belly Syndrome depends on other associated problems, especially the extent of damage to the urinary system.

The most common complications are:

• Constipation: Weak stomach muscles make it difficult to push through the stool.
• Bone deformities: things like hip dislocation, deformities of the hands, fingers, or toes.
• Frequent urinary tract infections: Infections can easily occur because urine is not being drained properly. These infections can damage the kidneys.
• Infertility: Failure of the testicles to descend can affect the ability to have children in the future. There is also a risk of testicular cancer.
• Pulmonary Hypoplasia: The lungs may not develop properly due to lack of space in the womb.
• Chronic Kidney Failure: This is the most serious complication. Continuous problems with the urinary system can lead to gradual kidney failure.

How is this condition diagnosed?

In most cases, this condition can be detected early on during your pregnancy scan (Fetal Ultrasound).Doctors will suspect this if the scan shows the baby's bladder is very large or the kidneys are swollen. The scan may also show that the baby has no abdominal muscles.

After the baby is born, the doctor may suspect this based on the baby's external appearance, i.e. the wrinkled appearance of the abdomen. Then, various tests are performed to confirm the disease and determine the extent of organ damage.

What tests are performed?

• Blood Tests: Learn about kidney function.
• Ultrasound Scan: Check the condition of organs inside the abdomen, such as the kidneys and bladder.
• X-ray: To check for any problems with the skeletal system.
• CT Scan or MRI Scan: Get a clearer, more detailed picture of the organs.
• VCUG (Voiding Cystourethrogram): This is a special X-ray test. This involves injecting a special fluid into the bladder and watching the fluid flow through it as the baby urinates. This helps to determine if urine is flowing backward (reflux).

What are the treatments for this?

The treatment for Prune Belly Syndrome varies from baby to baby. Because not every baby is affected in the same way. Usually, a team of specialists, such as pediatricians, pediatric urologists, and nephrologists, work together to develop the best treatment plan for your baby.

When deciding on treatment, these factors are taken into account:

• The baby's overall health.
• The baby's age.
• The severity of the disease and which organs are damaged.
• The baby's ability to tolerate surgery and treatment.
• Your opinions and wishes as parents.

However, many babies will have to undergo multiple surgeries. These surgeries are intended to strengthen the abdominal wall, lower the testicles, and correct problems with the urinary system.

There are several main types of surgeries performed:

• Vesicostomy: This involves surgically making a small opening in the lower abdomen into the bladder. This allows urine to drain directly out. This can reduce damage to the kidneys.
• Orchiopexy: This involves surgically moving the testicles, which are located inside the abdomen, down into the scrotum and fixing them.
• Cystoplasty: This is a slightly more complex surgery. It involves completely reconstructing the bladder.

In some severe cases, if the kidneys fail completely,A kidney transplant may also be necessary.

What will the future of these children be like?

I know this is a little hard to hear. But it's important to know the truth. The lifespan of these children depends on the severity of the disease. Unfortunately, between 10% and 25% of babies die in the womb or die from complications within the first few weeks of birth.

But, there is a bright side. About 40% of children with Prune Belly Syndrome, despite having minor problems with their urinary system, maintain normal kidney function and lead largely normal lives.

Another 30% may need a kidney transplant at some point in their lives. Most importantly, anyone with this condition needs to be under medical supervision for the rest of their lives. Regular monitoring of the urinary system is essential to minimize any damage to the kidneys.

How to care for your child and when to see a doctor?

If your child is diagnosed with Prune Belly Syndrome, the most important thing is to find an experienced doctor who is knowledgeable about the condition. He or she will explain everything to you and guide you on how to care for your child.

Urinary Tract Infections (UTIs) are very dangerous for these children. Therefore, if you notice even the slightest sign of a urinary tract infection, you should call your doctor immediately.

If you notice any of these symptoms, don't delay. Seek medical advice immediately. Also, it's essential to go to the clinics at the scheduled times and have your child checked, as the doctor recommends.