Pediatric Prune Belly Syndrome (Eagle-Barrett syndrome)

Pediatric Prune Belly Syndrome (Eagle-Barrett syndrome)

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Summary

Prune Belly syndrome causes partial or complete absence of the stomach muscles, undescended testes and/or urinary tract malformations.

Expanded overview

Prune Belly syndrome – also known as Eagle-Barrett syndrome – is a very rare, congenital (present at birth) disorder that causes a child’s stomach to have a wrinkled, prune-like appearance. This condition can cause a variety of complications, including:

  • Chronic renal (kidney) failure
  • Failure of both testes to descend into the scrotum (bilateral cryptorchidism)
  • Malformations of the urinary tract, including abnormal dilation (widening) of the ureters (tubes that drain urine from the kidneys to the bladder), pooling and/or backflow of urine in the ureters (hydroureter) and kidneys (hydronephrosis)
  • Partial or complete absence of the stomach (abdominal) muscles
  • Underdevelopment of the lungs (pulmonary hypoplasia)

Causes

The cause of this condition is unknown.

Symptoms

Signs and symptoms of Prune Belly syndrome include:

  • Blood and pus in the urine
  • Clubfoot (twisted foot or feet)
  • Deformed chest, such as flaring of the ribs, a horizontal depression under the chest (Harrison groove) or narrowing of the chest (pigeon breast)
  • Navel (belly button) that appears as a vertical slit
  • Wrinkled or prune-like appearance of the stomach, due to the missing abdominal muscles
  • Undescended testes (in males)

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