Eagle-Barrett Syndrome (Prune Belly Syndrome)

What are the signs and symptoms of Eagle-Barrett syndrome?

Children with Eagle-Barrett syndrome may have a range of symptoms. The most common symptoms include:

• The abdomen has a wrinkled appearance, similar to the appearance of a prune.
• The abdominal muscles are underdeveloped, leading to possible issues with standing or sitting up.
• In boys, the testicles are undescended. In girls, the external genitalia may appear abnormal.
• The urethra may be underdeveloped or blocked. In rare cases, the supporting tissue of the urethra may be underdeveloped, leading to an enlarged or dilated urethra.
• The bladder is enlarged and may not empty well.
• The ureters are often enlarged and twisted and may have associated poor drainage or vesicoureteral reflux.
• The kidneys may have evidence of renal dysplasia (abnormal development).

What tests are used to diagnose Eagle-Barrett syndrome?

Most cases of Eagle-Barrett syndrome are diagnosed during routine prenatal ultrasounds. If doctors suspect the condition, a fetal MRI may help us see lung and heart abnormalities associated with the syndrome.

After a baby is born, we may perform additional tests, including:

• Ultrasound: This procedure uses sound waves to help us see the kidneys and bladder and whether the ureter and bladder are enlarged.
• Voiding cystourethrogram: This study tests for reflux of urine into the ureters and kidneys. It also allows us to see urethral blockages. We insert a catheter (small tube) through your child’s urethra into the bladder and slowly fill the bladder with a solution. While the bladder is being filled, an imaging machine called a fluoroscopy takes pictures. A radiologist looks to see if any of the solution is going back up into the kidneys, which confirms a vesicoureteral reflux diagnosis. We also offer a new ultrasound technique called contrast-enhanced voiding urosonography (ceVUS) that uses a special ultrasound contrast to look for reflux without any additional radiation exposure.
• MAG3 nuclear medicine imaging: This study is performed to determine how each kidney is functioning. It also shows blockages and how severe they are. We place an intravenous line (IV) in your child’s arm and inject small amounts of radioactive contrast material to help see the kidneys and determine how well they are working.
• MRI/MRU: MRI is a radiation-free imaging process that uses a magnetic field and radio waves to produce detailed images of the body. MRU (magnetic resonance urography) focuses on the kidneys, ureters and bladder to give detailed information about these structures.
• Additional tests: Blood tests help us look at overall kidney function and identify any early signs of kidney stress. We also use blood pressure measurements and information about your child’s growth to evaluate early kidney stress.

How is Eagle-Barrett syndrome treated?

Treatment of Eagle-Barrett syndrome depends on when your child is diagnosed and how severe the condition is. Before a baby is born, we may perform a procedure to add fluid into the amniotic sac (the fluid-filled sac that protects a fetus) to create a larger “cushion” around the baby. This process supports lung development in babies whose lungs appear underdeveloped.

After birth, our multidisciplinary fetal genitourinary anomalies team provides lung support if needed, evaluates and supports kidney, ureter and bladder function to reduce risk of urinary tract infections and ensures adequate drainage of the kidneys. Procedures we perform on babies with prune belly syndrome include:

• Antibiotic medication once daily to reduce the risk of urinary tract infection
• Surgery to improve urine drainage:
  • Vesicostomy: This may be used in newborns who have large bladders that do not drain well. The dome (top) of the bladder is brought to the outside of the body to allow urine to drain freely and give the kidneys time to heal and function properly. We close this opening when your child is 3 to 5 years old.
  • Cutaneous distal ureterostomy: This may be necessary in newborns with severe ureteral dilation or poor kidney function. We surgically bring the ureter (the tube that connects the bladder and kidneys) to the surface of the skin. Urine then drains into your child’s diaper. After the kidneys and ureter have had time to heal (when your child is about 18 months old) we reconnect the ureter to the bladder.
  • Ureteral reimplantation: We remove the abnormal section of the ureter and reconnect the remaining ends. In some cases, the ureter is tailored to allow for creation of an anti-reflux valve mechanism.
• Surgery to improve testicular function:
  • Orchiopexy: Boys with Eagle-Barrett syndrome often have undescended testicles. We usually recommend surgery to bring the testicles down into the scrotum before a baby’s first birthday.
• Abdominal wall surgery: In children whose abdominal muscles are too weak to allow them to sit, stand or breathe normally, we recommend abdominal wall reconstruction (abdominoplasty) between ages 2 and 3.

Long-term treatment and outcomes

The long-term outlook for babies with Eagle-Barrett syndrome varies. Babies with poor lung development may require close follow-up with pulmonary (breathing) specialists. Some require breathing support with a ventilator. Babies with Eagle-Barrett syndrome may also be at greater risk of respiratory infections.

We also closely monitor bladder development to make sure urine drains normally. In some cases, children need to drain the bladder via a surgically created channel that remains there throughout their life. Some children with prune belly syndrome may eventually need a kidney transplant.

Why choose us for treatment of Eagle-Barrett syndrome?

The Colorado Fetal Care Center has the expertise to diagnose prune belly syndrome early and help parents plan for the best care after a baby is born.

We work with the congenital anomalies of the kidney and urinary tract (CAKUT) program to create a seamless transition from prenatal diagnosis to newborn care. You’ll work with the same providers before and after your baby is born.

We care for more than just your child’s kidney and urologic needs. Our multidisciplinary team looks at kids’ whole health and provides care from infancy through adolescence. For older teens, we work closely with the Urology Transitional Care Program to ensure effective and consistent care into adulthood.

Helpful resources

The National Organization for Rare Diseases (NORD) is a patient advocacy organization dedicated to individuals with diseases like Eagle-Barrett syndrome and the organizations that serve them.