Prune Belly Syndrome

Prune Belly Syndrome. Also called Triad Syndrome and Eagle Barrette Syndrome with deficient abdominal muscles, undescended testes and urinary tract anomalies, because of severe urethral obstruction in fetal life. The incidence is approximately 1 in 40 to 50 thousand births. Amongst affected 95 percent are males. Many affected infants are stillborn. In the perinatal period, oligohydramnios and pulmonary hyoplasia are frequent complications. The urinary tract anomaly includes vesico-ureteric reflux, large bladder, dilated ureters and upper tracts, Hypoplastic prostate with dilated prostatic urethra. Rarely urethral stenosis or atresia is seen. Many times the kidneys show various degrees of dysplasias. The undescended testes are usually intra-abdominal. Often mal-rotation of the bowel is associated anomaly. Cardiac anomaly is seen in 10 percent of these infants. More than 50 percent of cases have musculo-skeletal system abnormalities.
Treatment of frequent occurring urinary tract infection {UTI} and its complications need prompt attention. The intra-abdominal undescended testes need to be pulled down surgically at appropriate timing. Abdominal wall repair is to be done.

E-published: August 2008, Vol 5 Issue 8 Art # 34