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 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 May 2013 by Ronald Rabinowitz, MD; Jimena Cubillos, MD