Prune belly syndrome is a rare birth defect that affects newborns, particularly boys, causing underdeveloped abdominal muscles, urinary tract problems, and undescended testicles. Diagnosis involves blood tests, ultrasounds, and X-rays, and treatment typically involves surgery. Long-term complications can include kidney disease, ongoing urinary problems, and infertility.
Prune belly syndrome is a serious birth defect. Generally, the syndrome consists of three different problems in a newborn. The defects that make up this syndrome involve severely underdeveloped abdominal muscles, multiple urinary tract problems, and undescended testicles. While it is possible for a child of any gender to have this rare syndrome, it is generally seen in boys. Babies with this syndrome usually have a flat-stomach shaped abdomen that may be wrinkled like a prune in appearance.
The abdominal muscles in a child with plum belly syndrome may be underdeveloped, very weakened, or absent altogether. Due to insufficient or deficient muscles, the baby’s stomach can swell significantly. Additionally, most children with the condition have severely distended bladders and other significant abnormalities in their urinary tract. The cause of this syndrome is not distinctly known, although scientists believe that the birth defect of plum belly syndrome forms during the first few weeks of gestation.
Usually, the most obvious symptom of prune belly syndrome is the flat belly appearance of the abdomen. If the bladder is very enlarged, this will also present a number of urinary problems. It may be difficult to urinate, and urine may backflow into the urinary ureters and kidneys. There may also be frequent urinary tract infections. This syndrome can cause a delay in sitting and walking even in children.
Sometimes, mothers who carry babies with this birth defect will show some troubling symptoms. In many cases, an ultrasound performed on an expectant mother will reveal that the unborn child has an abnormally large abdomen or bladder. The kidneys can also appear particularly large. Also, the mother may have a low level of amniotic fluid throughout the pregnancy.
Some tests can be done on a baby after it is born to diagnose the syndrome. Initial tests may include blood tests, ultrasounds, and X-rays. Specialized tests to examine the urinary tract may also be done. An intravenous pyelogram (IVP) can be an example of a specialized test. This type of test can be done for a close-up view of urinary organs such as the bladder, kidneys, and ureters.
Treatment of prune belly syndrome typically involves surgery in most cases. Children commonly have surgery early in life to correct abdominal muscle deformities. Urinary problems can be extensive in children with this syndrome. For this reason, surgery can also be very helpful in correcting urinary tract abnormalities. Infants suffering from undescended testicles may have additional surgery to correct the abnormality.
In general, the prognosis of prune belly syndrome can vary. This is a very serious condition and can be life threatening. Even after having surgery, many children continue to have serious problems. Long-term complications can include kidney disease, ongoing urinary problems, and constipation. Additionally, males with this syndrome may develop infertility issues due to pre-existing testicular problems.
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