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 Vesicoureteral Reflux (VUR) Vesicoureteral reflux is retrograde passage of urine from the bladder back into the ureter and sometimes also into the renal collecting system, depending on severity. Reflux predisposes to urinary... read more , and urethral abnormalities Penile and Urethral Anomalies Congenital anomalies of the urethra in boys usually involve anatomic abnormalities of the penis and vice versa. In girls, urethral anomalies may exist without other external genital abnormalities... read more . Severe cases may involve renal failure, bronchopulmonary dysplasia Bronchopulmonary Dysplasia (BPD) Bronchopulmonary dysplasia is chronic lung disease of the neonate that typically is caused by prolonged ventilation and is further defined by degree of prematurity and extent of supplemental... read more , 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.