Definition

What is Prune-Belly syndrome?

Prune-Belly syndrome is a group of birth defects that involves these 3 main problems:

  • Poor development of the abdominal muscles, causing the skin of the belly area to wrinkle like a prune
  • Undescended testicles
  • Urinary tract problems

How common is Prune-Belly syndrome?

Prune belly syndrome is an uncommon birth defect occurring in about one in 30,000 to 40,000 births. In 95 percent of the cases, it occurs in males. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Prune-Belly syndrome?

The common symptoms of Prune-Belly syndrome are:

  • Partial or complete absence of the stomach (abdominal) muscles
  • Failure of both testes to descend into the scrotum (bilateral cryptorchidism)
  • Urinary tract malformations

The urinary malformations may include abnormal widening (dilation) of the tubes that bring urine to the bladder (ureters), accumulation of urine in the ureters (hydroureter) and the kidneys (hydronephrosis), and/or backflow of urine from the bladder into the ureters (vesicoureteral reflux).

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Prune-Belly syndrome?

The causes of prune belly syndrome are unknown. While in the womb, the developing baby’s abdomen swells with fluid. Often, the cause is a problem in the urinary tract. The fluid disappears after birth, leading to a wrinkled abdomen that looks like a prune. This appearance is more noticeable due to the lack of abdominal muscles.

Risk factors

What increases my risk for Prune-Belly syndrome?

Please discuss with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Prune-Belly syndrome diagnosed?

A woman who is pregnant with a baby who has prune belly syndrome may not have enough amniotic fluid (the fluid that surrounds the fetus). This can cause the infant to have lung problems from being compressed in the womb.

An ultrasound done during pregnancy may show that the baby has a swollen bladder or enlarged kidney.

In some cases, a pregnancy ultrasound may also help determine if the baby has:

  • Heart problems
  • Abnormal bones or muscles
  • Stomach and intestinal problems
  • Underdeveloped lungs

The following tests may be performed on the baby after birth to diagnose the condition:

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

How is Prune-Belly syndrome treated?

Specific treatment for prune belly syndrome will be determined by your child’s doctor based on:

  • Your child’s age, overall health, and medical history
  • The extent of the disease
  • Your child’s tolerance for specific medications, procedures, or therapies
  • Expectations for the course of the disease
  • Your opinion or preference

Treatment for the syndrome depends on the severity of symptoms. If your child has mild prune belly syndrome, he or she may be maintained on precautionary antibiotic therapy to prevent upper and lower urinary tract infections.

Your child may be referred to a urologist (doctor who specializes in disorders and care of the urinary tract and the male genital tract). Some children may require a vesicostomy (a small opening made in the bladder through the abdomen) to facilitate emptying the bladder of urine. Other children may need to undergo extensive surgical remodeling of the abdominal wall and urinary tract. In boys, a surgical procedure to advance the testes into the scrotum, called an orchiopexy, may be performed.

Despite available surgical interventions, some children may develop extensive kidney failure.

Lifestyle changes & home remedies

The following lifestyles and home remedies might help you prevent Prune-Belly syndrome:

There is no known way to prevent this condition. If the baby is diagnosed with a urinary tract obstruction before birth, in rare cases, surgery during the pregnancy may help prevent the problem from progressing to prune belly syndrome.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: January 3, 2018 | Last Modified: January 3, 2018

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