Prune Belly Syndrome in Children
What is prune belly syndrome in children?
Prune belly syndrome is a rare type of birth defect. The defect can range from mild
to severe. It is also known as triad syndrome or Eagle-Barrett syndrome. It includes
three main problems:
Missing muscles or severely weak muscles in the belly (abdomen)
One or both testicles not in the scrotal sac (undescended testicles)
Abnormal, large bladder and problems with the kidneys and the tubes that lead from
the kidneys to the bladder (ureters)
A child with prune belly syndrome often can't fully empty their bladder. This can
cause serious bladder, ureter, and kidney problems.
A child with prune belly syndrome may also have other birth defects. Most often, these
defects affect the skeletal system, intestines, lungs, and heart. Girls may have defects
in their external genitals.
Some babies who have prune belly syndrome may die in the uterus at 20 weeks of pregnancy
or later (stillborn). Some babies with this condition die a few months after birth.
What causes prune belly syndrome in a child?
Experts don't know what causes prune belly syndrome. Some cases have occurred in siblings.
This means a gene may be a cause.
It may occur if there is a blockage of the urethra during a baby’s growth in the uterus.
The urethra is the tube that drains urine from the bladder to the outside of the body.
A blockage prevents urine from flowing through the urinary tract. Urine can reverse
flow and cause the bladder to become large.
Which children are at risk for prune belly syndrome?
Prune belly syndrome is not common. It occurs in about 1 in 30,000 to 40,000 births.
Most cases occur in boys.
What are the symptoms of prune belly syndrome in a child?
Symptoms can be a bit different for each child. They may be mild to severe. They can
Belly (abdomen) that looks wrinkly with multiple folds of skin
Bladder causing a bulge in the belly above the pubic bones
Urinary tract organs easy to feel through the belly
Outline of the intestines seen through the belly
No testes in the scrotum (baby boys)
Trouble sitting upright or walking, because of weak muscles in the belly
Frequent urinary tract infections (UTIs) if the urinary tract is blocked.
The symptoms of prune belly syndrome can seem like other health conditions. Have your child
see their healthcare provider for a diagnosis.
How is prune belly syndrome diagnosed in a child?
Often prune belly syndrome is diagnosed by ultrasound while a woman is still pregnant.
Or a healthcare provider can often make a diagnosis after a newborn baby’s first exam.
Children who are diagnosed later often have UTIs. The healthcare provider will ask
about your child’s symptoms and health history. The provider will give your child
a physical exam. Your child may also have tests such as:
Intravenous pyelogram (IVP). This is an imaging test that uses an X-ray to see the urinary tract. A contrast dye
is given so that the tract can be seen on film. An IVP also shows the rate and path
of urine flow through the urinary tract.
CT scan. This is an imaging test that uses X-rays to see the belly (abdomen) and pelvis. A
computer puts the X-ray images together to create pictures of the anatomy.
Voiding cystourethrogram (VCUG). This is a type of X-ray of the urinary tract. The healthcare provider puts a thin,
flexible tube (catheter) into the tube that drains urine from the bladder to the outside
of the body (the urethra). The provider fills the bladder with a liquid dye. The provider
takes X-ray images as the bladder fills and empties. The images will show if there
is any reverse flow of urine into the ureters and kidneys.
Kidney (renal) ultrasound. This imaging test uses sound waves and a computer to create images of blood vessels,
tissues, and organs. The test can show internal organs as they function and help assess
blood flow through vessels.
Blood tests. These are done to see how well your child's kidneys work.
How is prune belly syndrome treated in a child?
Treatment will depend on your child’s symptoms, age, and general health. It will also
depend on how severe the condition is. Your child may see a urologist. This is a doctor
who treats the urinary tract and the male genital tract.
If your child has mild prune belly syndrome, they may take antibiotics to prevent
urinary tract infections.
Some children may need surgery. A vesicostomy is surgery to make a small opening in
the bladder through the belly to empty the bladder of urine. Other children may need
to have surgery to rebuild the belly wall and urinary tract. In boys, a surgery to
advance the testes into the scrotum may be done. This surgery is called orchiopexy.
Talk with your child’s healthcare providers about the risks, benefits, and possible
side effects of all treatments.
What are possible complications of prune belly syndrome in a child?
Even with treatment, some children may develop kidney failure.
When should I call my child’s healthcare provider?
Call the healthcare provider if your child has:
Key points about prune belly syndrome in children
Prune belly syndrome is a rare type of birth defect.
It includes three main problems. A child will have missing muscles or severely weak
muscles in the belly. One or both testicles are not in the scrotal sac (undescended
testicles). And the bladder is large, and there are problems with the kidneys and
A child with this syndrome often can't fully empty their bladder. This can cause serious
bladder, ureter, and kidney problems.
If your child has a mild case, they may take antibiotics to prevent urinary tract
infections. But some children may need surgery.
Even with treatment, some children may develop kidney failure.
Tips to help you get the most from a visit to your child’s healthcare provider:
Know the reason for the visit and what you want to happen.
Before your visit, write down questions you want answered.
At the visit, write down the name of a new diagnosis and any new medicines, treatments,
or tests. Also write down any new instructions your provider gives you for your child.
Know why a new medicine or treatment is prescribed and how it will help your child.
Also know what the side effects are.
Ask if your child’s condition can be treated in other ways.
Know why a test or procedure is recommended and what the results could mean.
Know what to expect if your child does not take the medicine or have the test or procedure.
If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
Know how you can contact your child’s provider after office hours. This is important
if your child becomes ill and you have questions or need advice.