Pseudoprune-Belly syndrome: imaging findings and clinical outcome.

Abstract

The purpose of this study was to describe the imaging features and clinical outcome of patients with an incomplete form of the prune-belly syndrome, called pseudoprune-belly syndrome. Imaging and the clinical course of eight boys with pseudoprune-belly syndrome (prune-belly syndrome uropathy, normal abdominal wall examination, and incomplete or absent cryptorchidism) were retrospectively reviewed. Voiding cystourethrography (n = 8) showed a dilated posterior urethra and megacystis in each patient (100%) and vesicoureteral reflux in six (75%). Among the eight patients, IV urography (n = 7) and renal sonography (n = 4) showed dysmorphic kidneys in each patient (100%), with bilateral hydroureteronephrosis in six (75%), and unilateral hydroureteronephrosis with a poorly functioning (or nonfunctioning) contralateral kidney in two (25%). Urologic procedures included vesicostomy (n = 3, 38%), ileal conduit (n = 2, 25%), pyeloplasty (n = 2, 25%), ureteral reimplantation (n = 3, 38%), and nephroureterectomy (n = 3, 38%). Renal failure developed in five patients (63%). Uropathy in patients with pseudoprune-belly syndrome is typically moderate to severe. Despite urologic intervention, renal insufficiency develops in most patients.