Prune belly syndrome

Definition

Prune belly syndrome is a group of birth abnormalities marked by three major findings:

• Lack of development of abdominal muscles, causing the skin of the abdomen to wrinkle like a prune
• Undescended testicle
• Urinary tract problems

Causes, incidence, and risk factors

The underlying causes of prune belly syndrome are unknown. Boys make up 96 - 97% of cases. Expectant mothers carrying affected infants may develop varying degrees of oligohydramnios (not enough amniotic fluid) that make the infant likely to have lung problems. (See also Potter syndrome.)

The newborn infant has a wrinkled abdomen that looks like a prune. This is because the abdomen swelled with fluid in the womb, then lost that fluid after birth, leading to puckering of excess skin. The appearance is more noticeable because the lack of enough abdominal muscles. Many infants are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of birth problems.

Symptoms

• Wrinkled skin on abdomen
• Undescended testicle
• Club foot or abnormal limbs (rare)

Signs and tests

• Oligohydramnios (decreased or absent amniotic fluid)
• Enlarged renal collecting structures
• Enlarged bladder

Other findings may include:

• Underdeveloped lungs
• Heart problems
• Stomach and intestinal problems
• Musculoskeletal abnormalities

The following tests may be used to diagnose the condition:

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

Treatment

• Surgery to repair genital and bladder problems (common)
• Antibiotics to avoid or treat urinary tract infection

Expectations (prognosis)

Prune belly syndrome is a serious and often life-threatening problem. Many newborns survive with varying degree of recurring problems. Others are stillborn or die in the first 2 years of life.

Complications

Complications depend on the related problems. The most common are chronic renal failure and club foot.

Calling your health care provider

Prune belly syndrome is usually diagnosed before birth or at the time of birth.

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, before birth, your baby is seen to have a distended bladder or enlarged kidneys on prenatal ultrasound, seek the advice of a specialist in high-risk pregnancy or perinatology.

Prevention

There is no known guaranteed prevention. If a prenatal diagnosis of urinary tract obstruction is made, it may be possible to prevent it from progressing to prune belly syndrome with prenatal surgery.

Illustrations

Abdominal muscles

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