
Prune-Belly syndrome, also known as abdominal muscle deficiency, is a rare disorder characterized by partial or complete absence of the stomach (abdominal) muscles, failure of both testes to descend into the scrotum (bilateral cryptorchidism), and/or urinary tract malformations. The urinary malformations may include abnormal widening (dilation) of the tubes that bring urine to the bladder (ureters), accumulation of urine in the ureters (hydroureter) and the kidneys (hydronephrosis), and/or backflow of urine from the bladder into the ureters (vesicoureteral reflux).
Complications associated with Prune-Belly syndrome may include underdevelopment of the lungs (pulmonary hypoplasia) and/or chronic renal failure. The exact cause of Prune-Belly syndrome is not known.
How common is abdominal muscle deficiency syndrome?
Prune Belly syndrome is a very rare disorder that is present at birth. The disorder affects mostly males but a few female cases have been described in the medical literature.
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Until now the exact cause of Prune Belly syndrome is still unknown. However, there are several theories.
You may have higher risks for this condition if you are experiencing these following conditions:
Most children with abdominal muscle deficiency syndrome have a family history of migraine, and most go on to develop this condition as adults.
Prune Belly syndrome is characterized by:
The diagnosis is usually obvious from birth, but care and time are needed to determine the location and number of abnormalities. A full understanding of the complications will involve imaging tests such as:
These tests are ordered in order to determine the extent of involvement of the genitourinary tract, intravenous pyelogram (IVP). An IVP makes use of a dye to map the degree of involvement of the kidneys and their ducts.
Depending on the severity of the symptoms, treatments will be determined. Some children will require rather modest surgical procedures such as the creation of a small opening in the bladder through the abdomen (vesicostomy) that will facilitate voiding of urine, or a procedure to help the testicles descend into the scrotum (orchiopexy). More extensive surgical procedures such as bladder reconstruction (cystoplasty), surgical widening of the urethra, and augmentation of the muscles that contract the bladder (detrusor augmentation) using a paired graft of a hip muscle (rectus femoris) have been successfully undertaken on children with prune belly syndrome. In rare cases, kidney transplantation may be necessary.
There is no known way to prevent this condition. If the baby is diagnosed with a urinary tract obstruction before birth, in rare cases, surgery during the pregnancy may help prevent the problem from progressing to prune belly syndrome.
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It changes rapidly
This data changes rapidly, so what’s shown may be out of date. Table totals may not always represent an accurate sum. Information about reported cases is also available on the World Health Organisation site.
It doesn’t include all cases
Confirmed cases aren’t all cases. They only include people who tested positive. Testing rules and availability vary by country.