Prune Belly Syndrome

QUESTION: My grandson was just born with an abnormally wrinkled stomach, or
so I have been told. My daughter has been informed that he has "prune belly"
syndrome. What is this? Is it dangerous? What must be done to help him?
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ANSWER: Prune belly syndrome (sometimes referred to as Obrinsky's Syndrome or
abdominal muscle deficiency) affects boys almost exclusively, and is caused by
a lack of the muscles in the lower and central portions of the abdomen. It is
also associated with abnormal development of the urinary and genital systems.
The cause is unknown, but may be related to a sex-linked recessive trait.
The skin of the baby's abdominal wall gets severely stretched because of
the absence of abdominal musculature. Because much of the muscle mass is
missing, the skin of the abdomen is severely stretched by the pressure of the
bowel and bladder. When the pressure is relieved, the skin then collapses
back into a mass of wrinkles, looking like the surface of a large prune, thus
the "prune belly" syndrome. Fortunately, as long as your daughter works
closely with a doctor who understands this problem, your grandson can lead a
normal life.
The baby will need proper abdominal support and he'll need diligent
urological care.
The physician will keep an eye on the growth and development of the
bladder, which may be distended in the early stages of this problem. It is
also imperative that the doctor monitor the kidneys continuously, as there are
some cases in which the kidney degenerates. Major complications are usually
avoided with proper care. Good luck to your grandson.