Prune Belly Syndrome (Eagle-Barrett Syndrome)
Prune belly syndrome (PBS; also known as triad syndrome, Eagle-Barrett syndrome or Obrinsky syndrome) is a genetic disorder that affects approximately 1 in 30,000 to 40,000 live births. Boys account for 95% of the cases. Babies with this rare condition are born with poorly developed or missing abdominal muscles, undescended testicles (in boys), and an abnormal, expanded bladder.
Symptom severity ranges from mild to severe. Most infants with prune belly syndrome also have urinary tract abnormalities, including:
- An undeveloped urethra
- Enlarged kidneys
- Poorly developed kidney tissue
- Enlarged and twisted ureters
- A large bladder with irregularities in the bladder wall.
These issues can lead to a bladder which does not empty very well, so children must be monitored for the development of chronic kidney disease. Defects of the skeletal system, lungs, intestines, and heart can also be associated with PBS.
While the cause of prune belly syndrome is unknown, it can occur in association with Trisomy 18 and Trisomy 21 (Down syndrome).
What You Need to Know About Prune Belly Syndrome
Prune belly syndrome is often diagnosed before birth during a routine fetal ultrasound.
When you are referred to the SSM Health Cardinal Glennon St. Louis Fetal Care Institute with a suspected diagnosis of prune belly, our dedicated and multi-disciplinary team focuses on monitoring you and your baby’s health and preparing for delivery. This includes performing ultrasounds and carefully watching for any signs of fetal distress. During your pregnancy, you will meet with members of our multidisciplinary team from:
- Maternal Fetal Medicine
- Neonatology
- Pediatric urology
- Pediatric nephrology
- Pediatric surgery
- Genetics
This team will work with you to decide the best treatment for your child after delivery. You will also have the opportunity to visit our neonatal intensive care unit (NICU) where your baby will receive the necessary care after delivery.
Every baby's situation is different, and the SSM Health Cardinal Glennon St. Louis Fetal Care Institute works with each family to prepare for their baby’s delivery and care after birth.
Babies born with a mild case of prune belly syndrome may need simple follow-up care and monitoring from a pediatric urologist, while those with a severe case may require surgical treatment to rebuild the abdominal wall and urinary tract.
After delivery, the following tests may be needed to determine the severity of your baby's condition.
- Renal bladder ultrasound provides images of the bladder and kidney
- Voiding cystourethrogram helps determine whether a blockage in the urethra is preventing urine from passing through
- MAG III renal scan provides insight into kidney function and the degree of any blockage.
- Magnetic resonance urography creates detailed pictures of the kidneys, ureters, and bladder
- Video urodynamic study assesses how well the bladder stores urine and contracts to empty it
- Blood tests measure kidney function
Treatment options for babies with prune belly syndrome depend on the severity of symptoms. A team of specialists, including nephrologists and urologists, can treat other birth defects associated with the condition.
Other babies may require dialysis if the kidneys are not functioning properly. Abdominal wall reconstruction, surgery for undescended testicles, and other procedures to correct anatomical abnormalities may also be necessary.
There is no cure for prune belly syndrome, though treatment options and our understanding of the condition are consistently growing. The prognosis for newborns with this condition varies and depends on the severity of symptoms and kidney function. In severe cases, dialysis or a kidney transplant may eventually be required. Overall, many people with prune belly syndrome have good physical and mental health.