Pediatric Prune Belly Syndrome
Prune Belly syndrome causes partial or complete absence of the stomach muscles, undescended testes and/or urinary tract malformations.
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)
The cause of this condition is unknown.
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)