Prune Belly, otherwise known as Eagle-Barrett syndrome and triad syndrome, is a congenital defect (birth defect). It is very rare, affecting about one in 30,000 to 40,000 births. Ninety-five percent of all cases are in males, but it can occur in females as well.
Prune Belly syndrome involves three abnormalities, including:
- severe weakness of or absence of abdominal muscles
- undescended testicles (when it occurs in males)
- an abnormal bladder and problems with the urinary tract (bladder, ureters and kidneys)
Eagle-Barrett syndrome often occurs with other birth defects, including problems with the skeletal system, intestines, heart and genitalia.
Most children with Eagle-Barrett syndrome are unable to empty their bladders on their own and have severe defects in the bladder, ureters and kidneys.
Experts do not know what causes Eagle-Barrette syndrome, but there is some evidence that there is a genetic link. We do know that it is not caused by anything the parents did or didn’t do prior to or during pregnancy.
What are the signs and symptoms of Prune Belly syndrome?
Because the severity of the syndrome varies, the signs and symptoms may vary from child to child. The most common signs and symptoms of Eagle-Barrett syndrome are:
- a wrinkly abdomen with multiple skin folds
- urinary tract organs, such as the bladder and kidneys, that are easy to feel through the abdomen because of lack of muscle mass
- a lump or mass in the abdomen above the pubic bones (due to an expanded bladder)
- intestines that are visible through the abdomen (you may also be able to see the way intestines move to help digest food)
- absence of testes and scrotum in males and abnormal genitalia in females
- inability to walk or sit upright due to weak or absent abdominal muscles
- frequent urinary tract infections
- underdeveloped or weak lungs and/or frequent chest infections
How do you diagnose Prune Belly?
When and how a child is diagnosed with Prune Belly syndrome may depend on the severity of the defect. Sometimes doctors can diagnose Prune Belly syndrome on a fetal ultrasound during pregnancy. When the defect is severe, it can be diagnosed at birth. But when the defect is less severe and there aren’t any obvious outward signs, it might not be diagnosed until frequent urinary tract infections lead to diagnostic testing.
Some tests used to diagnose Prune Belly syndrome include:
- Ultrasound of the kidneys
- Voiding cystourethrogram (VCUG)
- Intravenous pyelogram (IVP)
- Blood tests
How do you treat Eagle-Barrett syndrome?
If your child has Eagle-Barrett syndrome, the treatment will depend upon the severity of your child’s defect and the symptoms he or she is experiencing. Mild cases may be treated with antibiotics to prevent urinary tract infections, and your child’s doctor may recommend that you see a pediatric urologist for follow up care.
If your child has a more severe defect, surgery may be necessary. Surgical options include:
- Vesicostomy – This is a procedure that creates a small opening in the bladder through the abdomen to help your child empty his or her bladder.
- Ureterostomy – Sometimes we perform a ureterostomy to direct urine away from the bladder. This is done by creating a stoma that diverts the urine.
- Repair and remodeling of the abdominal wall and urinary tract
- Orchiopexy – This is a surgery done to help advance the testes into the scrotum. We sometimes perform this surgery in boys who have undescended testicles.
At Beaumont, our pediatric urologists collaborate with pediatric nephrologists and other specialists to provide true multidisciplinary care for all of our children. If you bring your child to Beaumont, you can expect competent, compassionate care from a team of doctors all working together to provide the best possible treatment for your child.