Prune belly syndrome

Alternative names:

Eagle-Barrett syndrome

Definition:

A disease characterized by three major findings (triad): deficient abdominal muscles (causing the skin of the abdomen to wrinkle like a prune), undescended testicles, and a dilated abnormal urinary tract.

Causes, incidence, and risk factors:

The cause of prune belly syndrome is unknown. The dilatation of the urinary tract may develop as a result of urethral obstruction during fetal life. The incidence is approximately 1 in 30,000 to 40,000 births. Boys are almost solely affected.

Expectant mothers carrying affected infants may develop varying degrees of oligohydramnios (insufficient amniotic fluid) that predisposes the infant to lung problems (see Potter syndrome). The newborn infant has a wrinkled abdomen (it looks like a prune) due to lack of adequate abdominal musculature. Genital abnormalities may be prominent as undescended testes. Approximately one third of the infants are either stillborn or die within the first few weeks of life from severe lung, kidney, or combined problems.

Other abnormalities may be present including musculoskeletal defects, heart abnormalities, and a rotated bowel.