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What is Prune Belly Syndrome?

Medically Reviewed by Mahammad Juber, MD on August 29, 2022

Prune belly syndrome may have a funny name, but it can have serious health consequences. 

What Is Prune Belly Syndrome?

Prune belly syndrome, also called Eagle-Barrett syndrome, is a very rare birth defect. It is also known as triad syndrome because it has three key characterizations:

  • Missing or very weak abdominal (stomach) muscles
  • One or two undescended testicles
  • An abnormally large bladder and resulting problems with the kidneys and urinary system

The most significant problem in cases of prune belly syndrome is that many children are unable to fully empty their bladder. This may cause significant issues with the bladder, kidneys, and ureters, the tubes that connect the kidneys and bladder.

Urinary tract malformations are common in children with prune belly syndrome. These cause:

  • Abnormal widening of the ureters
  • Accumulation of urine in the ureters and kidneys
  • Backflow of urine into the ureters

Other birth defects often accompany prune belly syndrome. These may include defects in the lungs, heart, intestines, and skeletal system. Defects in the external genitals may occur in girls, but prune belly syndrome mainly affects boys.

Prune Belly Syndrome Causes

There is currently no known cause of prune belly syndrome. However, there are some theories about how it develops. 

One theory is that while the baby is developing, some sort of blockage occurs in the bladder or urethra. The urethra is the tube that takes the urine from your bladder to the outside of the body. When blockage occurs, urine can’t leave the body, and it accumulates, resulting in enlargement of the bladder, ureters, and kidneys. If the bladder becomes too large, that can cause the abdominal muscles to waste away or prevent the testes from descending.

Another theory is that the abdominal muscles don’t develop completely, leading to difficulty urinating and causing swelling and infection.

A third theory is that both the underdeveloped muscles and the urinary problems are the result of a shared, currently unknown cause, such as a nervous system defect.

There have been a few instances of siblings being born with prune belly syndrome, leading researchers to wonder if there is a genetic component. 

Prune Belly Syndrome Symptoms

Common symptoms of prune belly include:

  • A wrinkly abdomen
  • A bulge above the pubic bones caused by the bladder
  • Visible outline of intestines
  • Urinary tract organs that are easy to feel
  • Trouble with core strength leading to difficulty sitting or walking
  • Recurring urinary tract infections
  • No testes in the scrotum

The symptoms and severity of symptoms may vary from child to child, but an enlarged bladder is almost always present. Clubfoot, a condition in which a baby’s foot is twisted at birth, is common in children with prune belly syndrome. 

Experts have also found some associations between prune belly syndrome and spina bifida. Spina bifida is a birth defect that prevents the spinal cord from developing properly.

Prune Belly Syndrome Diagnosis

Your doctor may be able to identify prune belly syndrome during a prenatal ultrasound or during your newborn’s first checkup. The doctor will likely run some tests to verify the diagnosis and see the full extent of the condition. These tests may include:

  • Ultrasound, including a kidney ultrasound
  • X-rays, including an intravenous pyelogram (IVP) and voiding cystourethrogram (VCUG), which both use dye to show problems with the urinary tract
  • Blood tests to determine how well your child’s kidneys work

Prune Belly Syndrome Treatment

Because there are many different symptoms of prune belly syndrome and varying degrees of severity, there is a range of treatment options that a doctor may employ to help treat this illness. 

Children with prune belly syndrome may require surgery. Common surgeries for prune belly syndrome include:

  • Vesicostomy. A vesicostomy is a procedure in which the surgeon cuts through the abdomen to create a small opening in the bladder. This helps the child empty their bladder.
  • Orchiopexy. An orchiopexy is a surgery to help the testicles descend into the scrotum.
  • Cystoplasty. A cystoplasty is another term for bladder reconstruction.
  • Detrusor augmentation. Detrusor augmentation is a surgery used to augment the bladder muscles, often by using a graft of hip muscles.
  • Widening the urethra. Surgery to widen the urethra helps prevent blockage.
  • Kidney transplants. While rare, it is sometimes necessary for children with prune belly syndrome to receive a kidney transplant.

Prune Belly Syndrome Prognosis

The prognosis of prune belly syndrome is different for each child. Prognosis depends on the extent of the condition, your child’s age, and your child’s overall health.

Some babies with prune belly syndrome may be stillborn. Other babies born with prune belly syndrome may not live more than a few months past birth.

Even with treatment, some children may develop kidney failure. Kidney failure is described as 85 to 90% of your kidney function being gone, resulting in your kidney not working well enough to keep you alive.

Your kidneys are very important organs within your body. They’re responsible for:

  • Getting rid of your body’s waste, any toxins, and extra water
  • Helping control your blood pressure
  • Helping your body produce red blood cells

Kidney failure often happens slowly and ultimately results in waste and excess fluid building up in your body. This can make you extremely sick. Other symptoms of kidney failure include:

  • Tiredness
  • Trouble sleeping
  • Weakness
  • Itching
  • Poor appetite
  • Weight loss
  • Muscle cramps
  • Swollen feet or ankles
  • Low red blood cell count (anemia)

The only ways to cure kidney failure are with dialysis or a kidney transplant. Dialysis is a process that helps your kidney remove excess waste and fluid from your body. Following a kidney transplant, your child will receive a donor kidney to replace their failing kidney.

What to Do If You Suspect Prune Belly Syndrome in Your Child

Prune belly syndrome is hard to miss, as it typically displays obvious symptoms. It’s unlikely your doctor will miss it. If you’re in a situation where you don’t have access to medical care during your pregnancy or birth, though, or if you notice symptoms that were mild enough to be initially missed, call the doctor right away. Some cases of prune belly syndrome can cause death in newborns, but early intervention may lead to a better outcome.

Show Sources

SOURCES:

Mayo Clinic: “Clubfoot,” “Spina bifida.”

National Kidney Foundation: “Kidney Failure.”

National Organization for Rare Diseases: “Prune Belly Syndrome.”

Stanford Medicine: “Prune Belly Syndrome in Children.”

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