Pseudoprune-Belly syndrome: imaging findings and clinical outcome.
OBJECTIVE
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.


METHODS
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.


RESULTS
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%).


CONCLUSIONS
Uropathy in patients with pseudoprune-belly syndrome is typically moderate to severe. Despite urologic intervention, renal insufficiency develops in most patients.
Bellah
R D
RD
Department of Radiology, Children's Hospital of Philadelphia, PA, USA.
States
L J
LJ
Duckett
J W
JW
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011535:Prune Belly Syndrome / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D011859:Radiography