Eagle-Barrett syndrome

Alternative names

Prune belly 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