Prune Belly Syndrome

Prune belly syndrome, also called Eagle-Barrett syndrome, is a rare birth defect that occurs in the womb as a baby is developing. It is defined by three abnormalities: no abdominal muscles, an abnormally expanded bladder coupled with upper urinary tract problems and in males, testicles that have not descended into the scrotal sac. Often times, other birth defects accompany belly prune syndrome, such as abnormalities with the heart, skeletal system, intestines and, in females, external genitalia problems.

Causes

• It is not known what causes prune belly syndrome. It most likely occurs when the urethra is obstructed during fetal development. This causes urine to reverse its flow away from the urinary tract, resulting in an expanded bladder.

  The birth defect is most common in males. Prune belly syndrome may have a genetic link, since there are cases where siblings have the syndrome as well.

Early Diagnosis

• Since prune belly syndrome occurs in the womb, the disease is traditionally diagnosed before or during the time of birth. Ultrasounds conducted during pregnancy reveal enlarged kidneys, a swollen abdomen due to fluid and a distended bladder.

  A specialist in perinatology or high-risk pregnancies is usually brought in for belly prune syndrome cases because it is life-threatening. Many infants diagnosed with Eagle-Barrett syndrome are stillborn or die within weeks of being born as a result of kidney, lung and other complications. Those who survive have some recurring problems, but they are treatable.

Symptoms

• The typical symptoms of prune belly syndrome are a wrinkly abdomen with several folds of skin, underdeveloped or missing abdominal muscles, the appearance of a pot belly and recurring urinary tract infections. In addition, you can usually see the outline of intestines through the abdomen and easily feel urinary tract organs when touching the abdominal area. Often times, there is an abdominal mass right above the pubic bones.

  The degree of symptoms ranges from mild to severe, with indicators varying among those with the problem.

Other Problems

• Infants born with belly prune syndrome also may have associated urinary and kidney problems. A pediatric urologist handles urinary problems connected with belly prune syndrome, such as ureters reflux. This occurs when urine travels back up the ureters to the kidneys. A pediatric nephrologist handles the health of the kidneys as the baby develops by conducting blood test profiles.

  In addition, many children with belly prune syndrome suffer from respiratory problems because their diaphragm is weak. This makes children with the birth defect prone to pneumonia and asthma. A pulmonologist often addresses and treats breathing problems associated with Eagle-Barrett syndrome.

Treatment

• Mild cases of prune belly syndrome are generally treated with antibiotic therapy to ward off urinary, kidney or bladder infections. Surgical interventions are often used to treat severe Eagle-Barrett syndrome. A vesicostomy removes urine from the bladder, while extensive modification of the urinary tract and abdominal wall repairs weakened or incomplete areas. The specific treatment is based on the child's age, the extent of the disorder, medical history and the child's overall health.