Prune belly syndrome

Definition

Prune belly syndrome is a group of birth defects that involve three main problems:

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

Alternative Names

Eagle-Barrett syndrome; Triad syndrome; Urethral obstruction malformation sequence

Causes, incidence, and risk factors

The causes of prune belly syndrome are unknown. The condition affects mostly boys.

While in the womb, the developing baby's abdomen swells with fluid. That fluid disappears after birth, leading to a wrinkled abdomen that looks like a prune. The appearance is more noticeable due to the lack of abdominal muscles.

Symptoms

Weak abdominal muscles can cause:

• "Little Buddha" appearance
• Constipation
• Delay in sitting and walking
• Difficulties coughing

Urinary tract problems can cause difficulty urinating.

Signs and tests

A woman who is pregnant with a baby who has prune belly syndrome may not have enough amniotic fluid (oligohydramnios). This can cause the infant to have lung problems.

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
• Bone/muscle (musculoskeletal) abnormalities
• 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

Treatment

Early surgery is recommended to fix weak abdominal muscles, urinary tract problems, and undescended testicles.

The baby may be given antibiotics to treat or help prevent urinary tract infections.

Expectations (prognosis)

Prune belly syndrome is a serious and often life-threatening problem.

Many infants with prune belly syndrome are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of birth problems.

Some newborns survive but continue to have problems.

Complications

Complications depend on the related problems. The most common are:

• Constipation
• Bone deformities (clubfoot, dislocated hip, missing limb or digit, funnel chest)
• Disease of the urinary tract (patients may need dialysis and a kidney transplant)

Undescended testicles can lead to infertility or cancer.

Calling your health care provider

Prune belly syndrome is usually diagnosed before birth or when the baby is born.

If you have a child with diagnosed prune belly syndrome, call your health care provider at the first sign of a urinary tract infection or other urinary symptoms.

If a pregnancy ultrasound shows that your baby has a distended bladder or enlarged kidneys, talk to a specialist in high-risk pregnancy or perinatology.

Prevention

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.

References

Caldamone AA, Woodard JR. Prune belly syndrome. In: Wein AJ, ed. Campbell-Walsh Urology. 9th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 118.