Editing Pediatrics: Prune-Belly Syndrome (section)

=== Genitourinary Anomalies ===

# '''<span style="color:#ff0000">Kidneys'''
#* '''<span style="color:#ff0000">Non-obstructive hydronephrosis is the rule'''
#** Collecting system is characteristically dilated, often to a severe degree, however, the calyceal morphology may be well preserved
#** '''The degree of dilation does not correlate with the degree of renal dysplasia'''
# '''<span style="color:#ff0000">Ureters'''
#* '''<span style="color:#ff0000">Dilated and tortuous'''
#* '''Ureteral dysfunction is related to''':
#** Ureteral dilatation with failure of luminal coaptation during peristalsis
#** Reduced smooth muscle tissue and increased amount of fibrous connective tissue
#** Failure of propagation of an electrical conduction due to increased fibrous connective tissue.
#** Abnormal myofilament content in smooth muscle cells
#* '''The most normal portion of the ureter with the greatest amount of smooth muscle is in the proximal segment'''. This is the segment that should be preserved for reconstruction.
#* '''<span style="color:#ff0000">Vesicoureteral reflux is present in 75% of children'''
# '''<span style="color:#ff0000">Bladder'''
#* Usually massively '''enlarged with a pseudodiverticulum at the urachus'''
#* '''Urachus is patent''' at birth in 25-30% of children  
#* '''Wide bladder neck''' opening into a dilated prostatic urethra
#* Usually, urodynamics reveals
#** Excellent detrusor compliance; the end-filling pressure assumes a normal value; and the bladder functions well as a reservoir.
#** Delayed first sensation to void, and bladder capacity may be more than double the normal volume.
#** Presence of vesicoureteral reflux and reduced detrusor contractility.
#* '''≈50% patients void spontaneously with normal voiding pressures''', normal flow rates, and low postvoid residuals
# '''<span style="color:#ff0000">Prostate and Accessory Sex Organs'''
#* '''Prostatic hypoplasia'''
#** Thought to be a factor in the '''ejaculatory failure''' of PBS patients
#** '''Dilation of the posterior urethra is caused by prostatic hypoplasia and results in retrograde ejaculation'''
#* The vas and seminal vesicles are often atretic.
# '''<span style="color:#ff0000">Anterior urethra'''
#* '''Most common anterior urethral anomalies:'''
#** '''Urethral atresia'''
#** '''Hypoplasia and megalourethra'''
#*** The fusiform type of megalourethra involves deficiencies of the corpus cavernosum and corpus spongiosum, whereas the scaphoid variety involves a deficiency of corpus spongiosum only.
# '''<span style="color:#ff0000">Testes'''
#* '''<span style="color:#ff0000">Bilateral intra-abdominal testes</span>''' lying over the iliac vessels and adjacent to the dilated ureters are the most typical findings
#* '''No difference in germ cell counts''', '''Ad spermatogonia, and Leydig cells between PBS testes and non-PBS intra-abdominal testes'''
#** This implies that the environmental state of the abdomen is a major factor in their later spermatogenic potential