Editing Pediatrics: Prune-Belly Syndrome (section)

== Management ==

=== Pre-natal ===
* '''The most appropriate indication for prenatal intervention in suspected prune-belly syndrome is urethral atresia and progressive oligohydramnios'''
** The only situation with prune-belly syndrome that is potentially reversible is the pulmonary hypoplasia that is seen from urethral atresia and due to progressive oligohydramnios.
** Oligohydramnios that occurs early in the second trimester is generally indicative of severe renal dysplasia.
** Oligohydramnios, hydronephrosis, pulmonary hypoplasia, distended bladder, and urinary ascites do not independently warrant prenatal intervention
*** Hydronephrosis in prune-belly syndrome is well tolerated

=== Neonates ===
* '''A neonatology, nephrology, cardiology, and urology team is necessary'''
** '''Other associated abnormalities, such as cardiac or pulmonary, often take precedence over the urinary tract'''
*** '''Pulmonary complications are most likely to threaten the early survival'''
**** '''Immediate CXR is necessary to exclude commonly associated pulmonary abnormalities'''
*** '''Early urologic intervention is indicated only for neonates with evidence of bladder outlet obstruction (e.g. urethral atresia), preferably with a percutaneous suprapubic tube while the newborn is in the neonatal intensive care unitI'''
* '''After stabilization, urologic evaluation proceeds with:'''
** '''History and physical examination'''
** '''Labs'''
*** '''Serum creatinine, BUN and electrolytes'''
**** '''Assess for the potential systemic acidosis and electrolyte imbalances that may be seen in renal insufficiency'''
** '''Imaging'''
*** '''Ultrasound'''
*** '''VCUG'''
**** '''Should be avoided to reduce unless potential for introduction of bacteria from catherization unless the results are necessary for immediate clinical decision making.'''
***** '''Necessary in neonates with renal insufficiency to rule out as the etiology bladder outlet obstruction versus urinary stagnation'''
**** '''Prophylactic antibiotic therapy is recommended, especially before urinary tract instrumentation, including the initial VCUG'''
***** Once bacteria are introduced, infection in a static system may be difficult to eradicate.
* '''Reconstruction is best delayed until the child is approximately 3 months old, to allow for pulmonary maturation.'''
** Early retailoring of the urinary system to reduce stasis and eliminate reflux or obstruction has included ureteral shortening, tapering and vesicoureteral reimplantation, and reduction cystoplasty.
* '''Surgical reconstruction'''
*# '''Circumcision'''
*#* '''Advisable in the absence of a structural penile abnormality to reduce risk of UTI'''
*# '''Orchidopexy'''
*#* Best accomplished by transperitoneal mobilization before 1 year of age to maintain the germ cell population and protect spermatogenesis.
*#** Transabdominal complete mobilization of the spermatic cord almost always allows the testis to be positioned in the dependent portion of the scrotum without dividing the vascular portion of the spermatic cord.
*# '''Abdominal wall reconstruction'''
*#* '''Benefits:'''
*#** '''Improved bladder emptying'''
*#** '''Improved defecation'''
*#** '''More effective cough'''
*#** '''Psychosocial benefits'''
*# '''Reduction Cystoplasty'''
*#* '''In many PBS patients, poor bladder contractibility leads to incomplete and infrequent emptying from the complicating urinary stasis and VUR issues. This leads to the concept of reducing the size of the bladder and remodeling it into a more spherical shape to direct better the contractible forces'''
*# '''Upper urinary tract reconstruction'''
*#* '''Controversial'''
*#* '''Indications (3):'''
*#*# '''Declining renal function in the presence of hydronephrosis'''
*#*# '''Progression of the hydroureteronephrosis'''
*#*# '''Recurrent upper tract infections'''
*# '''Internal urethrotomy'''
*#* Indicated in the rare patient with true anatomic urethral obstruction or in patients with urodynamic evidence of urethral obstruction by pressure-flow studies.