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Prune Belly Syndrome

By Philip E. Gleason, MD

Prune Belly Syndrome is a rare Pediatric Urologic diagnosis affecting the urinary tract, testes and abdominal wall musculature in boys. This section topic will provide introductory information regarding prune belly syndrome.

Prune Belly Syndrome involves a weak abdominal wall musculature, undescended testicles and a dilated urinary tract. There is some theory that abnormalities of muscle tone may produce the weakness of the abdominal wall musculature as well as contribute to the undescended testicles. There may also be some degree of decreased muscle tone of the urinary tract musculature producing poor urinary peristalsis and the urinary tract dilation. However, the exact cause of Prune Belly Syndrome is unknown.

Prune Belly Syndrome may be diagnosed in-utero by ultrasound due to dilation and hydronephrosis of the urinary tract. It is otherwise usually diagnosed at birth noting the triad of a lax abdominal wall, undescended testicles and a dilated urinary tract.

The management of the boy with PBS involves an orchiopexy to bring the undescended testicles into the scrotum. Urinary stasis and possible vesico-ureteral reflux are managed to prevent urinary tract infections and the kidneys. Abdominal wall laxity may require management not only for abdominal support but also from a cosmetic standpoint.

Prune Belly Syndrome requires careful management of the urinary tracts to prevent infections and protect the kidneys. For reasons that are not entirely understood, there is unfortunately a high chance of infertility even with orchiopexy for the undescended testicles. With management and reconstruction however, these boys typically do well both functionally and cosmetically.