Prune-Belly Syndrome

(Triad Syndrome)

By Ronald Rabinowitz, MD, Professor of Urology and Pediatrics;Chief of Pediatric Urology, University of Rochester School of Medicine and Dentistry;Strong Memorial and Rochester General Hospitals ; Jimena Cubillos, MD, Assistant Professor of Urology and Pediatrics, University of Rochester School of Medicine and Dentistry

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Congenital Renal and Genitourinary Anomalies
Overview of Congenital Genitourinary Anomalies
Bladder Anomalies
Cryptorchidism
Penile and Urethral Anomalies
Prune-Belly Syndrome
Renal Anomalies
Testicular and Scrotal Anomalies
Ureteral Anomalies
Vaginal Anomalies
Vesicoureteral Reflux (VUR)

Prune-belly syndrome consists of abdominal muscle deficiency, urinary tract abnormalities, and intra-abdominal undescended testes.

The name prune-belly syndrome derives from the characteristic wrinkled appearance of the abdominal wall in neonates. The cause of this congenital syndrome, which occurs primarily but not exclusively in males, is unclear. Urinary abnormalities may include hydronephrosis, megaureters, vesicoureteral reflux, and urethral abnormalities. Severe cases may involve renal failure, bronchopulmonary dysplasia, and fetal demise.

Diagnosis of prune-belly syndrome is often made during routine prenatal ultrasonography. In addition to postnatal ultrasonography, further evaluation may include voiding cystourethrography and/or an isotope renography.

Urinary tract abnormalities may require open surgical reconstruction. If no urinary intervention is necessary, orchiopexy is done in conjunction with an abdominoplasty.

Last full review/revision November 2016 by Ronald Rabinowitz, MD; Jimena Cubillos, MD