Prune belly syndrome is also known as triad syndrome or Eagle-Barrett syndrome. It is characterized by a triad of abnormalities that include the following:
Because of the substantial involvement of the urinary tract, children with prune belly syndrome are usually unable to completely empty their bladders and have serious bladder, ureter, and kidney impairment.
A child with prune belly syndrome may also have other birth defects. Most commonly, these defects involve the skeletal system, intestines, and heart. Girls may have defects in their external genitalia, as well.
Some infants who have prune belly syndrome may be stillborn or die within a few months of birth.
Prune belly syndrome is an uncommon birth defect occurring in about one in 30,000 to 40,000 births. In 95 percent of the cases, it occurs in males. It may occur if there is a urethral obstruction during fetal development preventing any urine from flowing through the urinary tract. The urethra is the tube that drains urine from the bladder to the outside of the body for elimination. If there is obstruction, urine can reverse flow and cause an expanded bladder.The cause of prune belly syndrome is unknown; however, some cases have been reported in siblings, suggesting a genetic component.
The syndrome may occur in varying degrees from mild to severe. The following are the most common symptoms of prune belly syndrome. However, each child may experience symptoms differently. Symptoms may include:
The symptoms of prune belly syndrome may resemble other conditions and medical problems. Always consult your child's doctor for a diagnosis.
The severity of the defects often determines how a diagnosis is made. Often prune belly syndrome is diagnosed by fetal ultrasound while a woman is still pregnant. Because of the distinct abnormalities of prune belly syndrome, a doctor is usually able to make a diagnosis after initial examination during the newborn period. A child who may not have the outward signs of prune belly syndrome may experience a urinary tract infection, which will prompt further testing by your child's doctor. Additional diagnostic procedures may include the following:
Specific treatment for prune belly syndrome will be determined by your child's doctor based on:
Your child's age, overall health, and medical history
The extent of the disease
Your child's tolerance for specific medications, procedures, or therapies
Expectations for the course of the disease
Your opinion or preference
Treatment for the syndrome depends on the severity of symptoms. If your child has mild prune belly syndrome, he or she may be maintained on precautionary antibiotic therapy to prevent upper and lower urinary tract infections.
Your child may be referred to a urologist (doctor who specializes in disorders and care of the urinary tract and the male genital tract). Some children may require a vesicostomy (a small opening made in the bladder through the abdomen) to facilitate emptying the bladder of urine. Other children may need to undergo extensive surgical remodeling of the abdominal wall and urinary tract. In boys, a surgical procedure to advance the testes into the scrotum, called an orchiopexy, may be performed.
Despite available surgical interventions, some children may develop extensive kidney failure.
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PUV is an abnormality of the urethra, which is the tube that drains urine from the bladder to the outside of the body for elimination.
Ureteropelvic junction obstruction is a blockage in the area that connects the renal pelvis (part of the kidney) to one of the tubes (ureters) that move urine to the bladder.