Prune belly syndrome

Alternative names
Eagle-Barrett syndrome

Definition

Prune belly syndrome is a group of congenital anomalies characterized by three major findings:

     
  • Deficient development of abdominal muscles (causing the skin of the abdomen to wrinkle like a prune)  
  • Undescended testicle  
  • Urinary tract anomalies

Causes, incidence, and risk factors

The underlying causes of prune belly syndrome are unknown. 96-97% of cases are boys. Expectant mothers carrying affected infants may develop varying degrees of oligohydramnios (insufficient amniotic fluid) that make it likely the infant will 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 wrinkles of excess skin. The appearance is accentuated by the lack of adequate abdominal musculature. Many infants are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of congenital anomalies.

Symptoms

     
  • skin on abdomen wrinkled like a prune  
  • undescended testicle  
  • club foot or abnormal limbs are occasionally observed

Signs and tests

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

Other findings may include:

     
  • Underdeveloped lungs  
  • Heart anomalies  
  • Gastrointestinal anomalies  
  • Musculoskeletal abnormalities

The following tests may be used to diagnose the condition:

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

Treatment

     
  • Surgery to repair genitourinary anomalies is 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 chronic problems, but others are stillborn or die in the first two years of life.

Complications

Complications depend on the associated abnormalities. 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 your child is diagnosed with distended bladder or enlarged kidneys on prenatal ultrasound, seek prompt consultation with a specialist in high-risk obstetrics or a perinatologist.

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.

Johns Hopkins patient information

Last revised: December 7, 2012
by Mamikon Bozoyan, M.D.

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