Prune Belly (Eagle-Barrett) Syndrome: What You Need to Know

Learn More About the Distinctive, Physical Problems of Prune Belly Syndrome

Mother massaging baby's stomach. Credit: Stella / Getty Images

Prune Belly syndrome, also known as Eagle-Barrett or triad syndrome, is a congenital defect with a distinctive set of physical problems that a child is born with. These problems are:

  • The abdominal muscles in the front of the body are weak or missing
  • Parts of the urinary tract develop abnormally. These include abnormal widening (dilation) of the urinary tubes, accumulation of urine in the ducts that pass urine from the kidney to the bladder, and/or back flow of urine from the bladder into the tubes and ducts (called vesicoureteral reflux). 
  • In males (95% of cases), the testes stay up inside the abdomen and don't come down into the scrotum.

There may be other physical problems in the infant, such as spinal curvature, hip dislocations, clubfoot, respiratory or heart problems, and gastrointestinal problems.

Causes of Prune Belly Syndrome

The cause of the syndrome is unknown, but researchers believe that disruption of the growth of the fetus causes the problems to develop. It is thought that something blocks a part of the fetus' urinary tract, and this causes other parts of the tract to develop abnormally. Prune Belly syndrome occurs in 1 in 40,000 live births in the U.S.

Diagnosis of the Condition

Ultrasound during pregnancy can sometimes see the abnormal development of the bladder and urinary tract. While the fetus grows, fluid develops in its abdomen, which stretches larger and larger. The fluid is reabsorbed before birth, so at the time of birth the infant has a sagging or wrinkled abdomen (thus the "prune belly" name).

After birth, ultrasound and x-rays can determine what type of urinary tract abnormalities are present. Prune Belly syndrome is often characterized by: 

  • Some or most abdominal muscles are partially absent. This gives the wrinkled or prune-like appearance.
  • Though the attachments of the muscles to the bones may be present, the muscles are smaller and thinner over the bladder.
  • The abdominal wall is thin, the abdomen itself shows to be large and loose, and the intestines can be seen through the thin abdominal wall.
  • Skin folds seem to radiate from the belly button or go across the stomach.
  • A crease from the navel to pubic area may sometimes be present and sometimes the bellybutton may be connected to the bladder through a canal (urachus) or a cyst.
  • Flaring of the ribs or narrowing of the chest can cause the chest to be deformed in children with the syndrome.
  • An enlarged bladder is present in almost all cases.

Unfortunately, Prune Belly syndrome can have very severe effects on the infant. The statistics show that 20% of babies die before birth, and 30% die from kidney problems with the first two years of life. In the remaining 50% of infants, there are varying degrees of urinary problems.

Prune Belly Syndrome is often diagnosed under a variety of different names, such as abdominal muscle deficiency syndrome, congenital absence of the abdominal muscles, deficiency of abdominal musculature, Eagle-Barrett syndrome, obrinsky syndrome or triad syndrome.

Treatment for Eagle-Barrett

There is no cure for  Eagle-Barrett (prune belly syndrome), but repairs to the urinary tract can be made. Some surgeons attempt to correct problems on the fetus before it is born, but this has had mixed results. Children prone to urinary tract infections are given preventive antibiotics and surgery can bring a boy's testes down into his scrotum.

Sometimes despite the best treatment, a child's kidneys will stop working (renal failure). The treatment for this is either dialysis or kidney transplantation.

Better surgical techniques, especially using laparoscopy, will make the necessary repair operations for a child with Prune Belly syndrome less difficult. As more is learned about the syndrome, treatments can be improved and children helped to survive longer.

Sources:
Prune Belly Syndrome Network. About Prune Belly.
Franco, I. (2001). Prune Belly syndrome. eMedicine, accessed at http://www.emedicine.com/med/topic3055.htm
Leeners, B., Sauer, I., Schefels, J., Cotarelo, C.L., Funk, A. (2000). Prune-belly syndrome: Therapeutic options including in utero placement of a vesicoamniotic shunt. J Clin Ultrasound, Vol. 28, No. 9, pp 500-507.

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