Prune Belly Syndrome
A baby born with prune belly syndrome has weak belly muscles and problems with their bladder and kidneys. The condition mostly affects males.
What Is Prune Belly Syndrome?
Prune belly syndrome (PBS) is a rare condition that affects about 1 in every 50,000 children. PBS affects the belly (abdominal) muscles, bladder and testicles. The name comes from the wrinkled appearance of the belly, which some children have, which can look like a prune. Nearly all babies (95%) with PBS are males. There are three main features of the condition, which are called the prune belly triad:
- Weak and/or absent belly muscles
- Undescended testicles in males
- Urinary tract dilation which can damage kidney, ureter and bladder development
Babies are born with PBS, which means it is a congenital condition. Upon birth, the baby typically has an extremely full bladder. Because urine has not been emptying completely from the baby’s body, 10-20% of babies are in kidney failure at birth. Because fetal urine makes up the amniotic fluid, lung development may also be affected because of the decrease in amniotic fluid. For other babies, the kidney function is good at birth but must be watched closely for the rest of their lives to keep them safe.
Prune belly syndrome is also known as Eagle-Barrett Syndrome and triad syndrome. About 1,500 people under age 18 currently live with PBS in the United States. About half (40-60%) of babies born with PBS have other birth defects, too.
What Are the Symptoms of Prune Belly Syndrome?
Prune belly syndrome typically is found during pregnancy. Prune belly syndrome can be seen around 15 weeks on ultrasound. It can be challenging to diagnose due to similar conditions such as posterior urethral valves (when tissue blocks the urethra).
After the baby is born, there are visible symptoms. Some of the most commons signs include:
- Distended belly sometimes with wrinkled belly skin (before the bladder is emptied)
- Bladder full of urine (pee)
- Belly looks concave and saggy after the bladder is emptied of urine
- Testicles that did not descend into the scrotum in males
- Urinary system problems such as urine backup, enlarged bladder and/or enlarged kidneys
On some milder cases, PBS may not be diagnosed right away, but that is not very common.
What Causes Prune Belly Syndrome?
Prune belly syndrome is very rare. While doctors do not know exactly what causes it in most cases, researchers at Nationwide Children's Hospital are some of the only doctors in the world that are studying prune belly syndrome to identify a cause and to improve the lives of individuals with this disorder. See below for more information.
How Is Prune Belly Syndrome Diagnosed?
Only a doctor can diagnose prune belly syndrome. Some cases can be seen on an ultrasound during pregnancy. Some cases cannot be diagnosed until the baby is born. Pediatric urologists are experts in this condition. If you would like help concerning this, please contact our office.
How Is Prune Belly Syndrome Treated?
Each case of prune belly syndrome is unique. Treatment usually requires surgery and unfortunately, many babies need more than one surgery. This depends on the child’s condition, the severity of the PBS and the decisions you make with your surgeon (pediatric urologist and other specialists). Surgeries often include:
- Abdominal/“tummy tuck” (abdominoplasty) to reconstruct the belly and abdominal wall (done when the baby is 4 to 12 months old)
- Genital orchiopexy surgery to bring the undescended testicles into the scrotum (done by age 1 year). This may be done using laparoscopy or combined with other surgeries to decrease the number of trips to the operating room.
- Urinary tract (bladder and ureter) reconstruction including ureteral reimplantation reflux surgery
About 67% of people with PBS will go into kidney failure during their lifetime. Many are born with poorly formed or dilated kidneys. The lack of strong abdominal muscles means most people with PBS don’t completely empty their bladder. They make a lot of urine, which is diluted with lots of water. Children with PBS tend to have a lot of urinary tract infections (UTIs) because they are not able to fully empty their bladder. Combined, these three problems can lead to kidney damage and failure. People with PBS require life-long monitoring to save their kidneys from damage.
We can see all ages of individuals with PBS here at Nationwide Children’s Hospital.
When Should I Get Help for My Child?
Children and adults with PBS should be seen by their medical team at least once per year, and possibly more often. Ongoing treatment includes yearly ultrasound.
It’s important to be very aware of how often your child pees (urinates). Children with PBS may not be aware that their bladder is full, so they must be reminded often to use the bathroom. Signs of a UTI can include:
- Need/urge to use the bathroom more than usual
- Fever or chills
- Burning while peeing (urinating)
- Blood in urine
- Cloudy or dark urine
- Pain or pressure on back or low belly
Make sure to call your child's doctor or go to the emergency room to have the urine tested. Don't forget to contact your pediatric urologist if a UTI is confirmed, since it might be best to admit your child to the hospital to treat the UTI and drain the bladder of infected urine.
LOOKING FOR MORE INFORMATION?
Our team is happy to answer questions or address any concerns you may have.
Contact us at The Kidney and Urinary Tract Center at (614) 722-6250
