Prune belly syndrome is a rare type of birth defect. The defect can range from mild to severe. It is also known as triad syndrome or Eagle-Barrett syndrome. It includes 3 (a triad) main problems:
A child with prune belly syndrome often can't completely empty his or her bladder. This can cause serious bladder, ureter, and kidney problems.
A child with prune belly syndrome may also have other birth defects. Most often, these defects involve the skeletal system, intestines, lungs, and heart. Girls may have defects in their external genitals.
Some babies who have prune belly syndrome may die in the uterus at 20 weeks of pregnancy or later (are stillborn). Some babies with this condition die a few months after birth.
The cause of prune belly syndrome is not known. Some cases have occurred in siblings, which means a gene may be a cause.
It may occur if there is a blockage of the urethra during a baby’s growth in the uterus. The urethra is the tube that drains urine from the bladder to the outside of the body. A blockage prevents urine from flowing through the urinary tract. Urine can reverse flow and cause the bladder to become large.
Symptoms can occur a bit differently in each child, and may be mild to severe. They can include:
The symptoms of prune belly syndrome can be like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.
Often prune belly syndrome is diagnosed by ultrasound while a woman is still pregnant. Or a healthcare provider can often make a diagnosis after a newborn baby’s first exam. Children who are diagnosed later often have urinary tract infections. The healthcare provider will ask about your child’s symptoms and health history. The provider will give your child a physical exam. Your child may also have tests such as:
Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. Your child may see a urologist. This is a doctor who treats the urinary tract and the male genital tract.
If your child has mild prune belly syndrome, he or she may take antibiotics to prevent urinary tract infections.
Some children may need surgery. A vesicostomy is surgery to make a small opening in the bladder through the abdomen to empty the bladder of urine. Other children may need to have surgery to rebuild the abdominal wall and urinary tract. In boys, a surgery to advance the testes into the scrotum may be done. This surgery is called orchiopexy.
Talk with your child’s healthcare providers about the risks, benefits, and possible side effects of all treatments.
Call the healthcare provider if your child has:
Tips to help you get the most from a visit to your child’s healthcare provider:
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