Prune Belly Syndrome Network

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Prune belly syndrome gets its name from the wrinkly abdomen of babies born with the syndrome. As the baby grows into a toddler, the abdomen fills out. It becomes softer and pooches out as the child starts to sit and stand. Some or all of the abdominal muscles are missing in the child with PBS.

Because the child will likely have some degree of kidney and/or urinary tract problems, you should find a pediatric urologist who knows about PBS. Testing is usually done to determine if your child has urinary reflux, in which urine travels backward: from the bladder, up the ureters, to the kidneys. If urinary reflux is present, it must be addressed to preserve the health of the kidneys. Some children see a pediatric nephrologist to keep a close eye on kidney health. Blood tests (specifically for creatinine level and BUN) are done routinely to monitor kidney function.

Children with the syndrome may need several surgeries while they are young. Those with a mild case of PBS may have few symptoms and be quite healthy, requiring only one surgery to bring the testicles down.

Often the bladder does not drain completely because it has been so stretched out and is very large and thick-walled. Therefore, children cannot feel it when their bladder is full. If the bladder does not empty completely, urinary tract infections may result. Many children need to be catheterized daily to be sure the bladder is totally emptied. Also, they need to be on a regular schedule to urinate and must "double void" (urinating for a second time about 20 minutes after the first time) to be sure to empty the bladder. 

Children with PBS may have respiratory issues because the diaphragm (which makes you breathe) can be weak. A weak diaphragm produces a weak cough and may predispose the child to getting pneumonia. It may be wise to request an evaluation by a pulmonologist (lung specialist) and to be on the alert for symptoms of increasing breathing problems. Watch specifically for fever, having a hard time while attempting to cough, shortness of breath, or any difficulty breathing. Sometimes children with PBS seem to have asthma. Breathing treatments and medicines used for asthma can help. Also, using an abdominal binder may assist with the cough reflex and lessen the chance of pneumonia.

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