What is Prune Belly Syndrome?

Also referred to as triad syndrome or Eagle-Barrett syndrome, prune belly syndrome is a congenital condition that relates to weakness in the stomach, issues in the testicles and trouble with formation of the urinary system.

Prune belly syndrome is very rare, but can be severe and even fatal in some cases. It can often be detected in an unborn child in the womb, and there are treatment methods available. Here are the basics of prune belly syndrome.

 

Features, Symptoms and Possible Complications

 

Prune belly syndrome is present at birth in all cases, and is characterized by three primary features:

 

• Muscles in the stomach are extremely weak, or are not present at all. Because of this, the baby’s stomach skin will be wrinkled like a prune.
• In baby boys, the testicles fail to drop from inside the body into the scrotum, the sac behind the penis meant to hold them.
• There are issues in the formation of the urinary system.

 

In some cases, prune belly syndrome can also affect other parts of the body such as the heart, lungs, intestines and skeletal system. A few symptoms that can be caused by weak abdominal muscles include:

• “Little Buddha” appearance
• Constipation
• Delay in sitting or walking
• Trouble coughing

 

In cases where urinary tract problems are present, these can lead to difficulty in urinating.

Possible complications from prune belly syndrome vary depending on the related problems that come with it. Some of the most common complications include:

• Constipation
• Bone deformities such as clubbed foot, dislocated hip, missing limb, finger or toe, or funnel chest
• Urinary tract disease, which might require dialysis or a kidney transplant
• Infertility or cancer due to undescended testicles

 

Causes and Risk Factors

 

Prune belly syndrome is rare, affecting only one in between 30,000 and 40,000 babies. In about 95 percent of these cases, the babies affected are boys.

The exact cause of prune belly syndrome is unknown, but doctors know that it originates in the womb. A buildup of urine in the bladder, which might be caused by a block in the urethra (the tube that carries urine out of the body), can cause urine to flow backward and expand the bladder.

The large bladder can then get in the way of stomach muscles, which keeps them from forming correctly and also causes issues in the testicles and urinary system. Prune belly syndrome may also be genetic—some pairs of brothers and sisters will have it.

 

Testing and Treatment

 

In some cases, prune belly syndrome can be found while the mother is still pregnant. This is done using a fetal ultrasound, which can show that the baby has a swollen bladder or enlarged kidney. It can also help test for heart issues, bone or muscle abnormalities, stomach and intestinal issues, and underdeveloped lungs.

 

If the condition is not diagnosed before birth, the doctor can usually tell by looking at the baby after it’s born. If signs are mild, the doctor may use one of the following tests to confirm the diagnosis:

• Blood test
• Intravenous pyelogram (IVP)
• Ultrasound
• Voiding cystourethrogram (VCUG)
• X-ray

 

Treatment for prune belly syndrome varies based on the baby’s health, symptoms, future outlook and ability to handle certain treatments. In mild cases, treatment might be limited to drugs that keep germs from infecting the urinary symptoms.

In more severe cases, early surgery may be undertaken to fix weak abdominal muscles, urinary tract issues or undescended testicles. This could be a small procedure or more intense surgical repair.

Prune belly syndrome can cause death before birth, and some babies with it will die shortly after birth. Some babies who survive prune belly syndrome will go on to live normal lives, but some others will have medical problems throughout life. About 30 percent of these people will need a kidney transplant later in life. People with prune belly syndrome will need lifelong follow-up care to check the urinary system and reduce the chances of kidney damage.

If your child has prune belly syndrome and shows signs of a urinary tract infection, call your doctor right away. If you are pregnant, your doctor will advise you on a course of action if prune belly syndrome is discovered during pregnancy.

 

Our staff offers a variety of services, including in-office PSA testing, a screening for prostate cancer. We work with you and your primary care physician to develop an individualized care plan for you based on the latest technology and research.

 

Sources:

 

“Prune Belly Syndrome.” Cleveland Clinic. https://my.clevelandclinic.org/health/articles/prune-belly-syndrome

“Prune belly syndrome.” MedlinePlus. https://medlineplus.gov/ency/article/001269.htm