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Background: The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure., Objective: The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure., Design, Setting, and Participants: From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5-37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8-33m]., Intervention: RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction., Outcome Measurements: The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage., Results and Limitations: All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3-30] follow-up., Complications: Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1-3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery., Conclusion: The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy., (Copyright © 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Patients: Eleven patients with 46,XY PGD were divided into two groups. Six symptomatic girls (group 1) were referred for amenorrhea (n = 3), gonadal tumor (n = 2) or campomelic dysplasia (n = 1). Five asymptomatic screened patients (group 2) were diagnosed as 46,XY PGD after familial investigation of the two probands with gonadal tumor. Bilateral gonadectomy was performed in all patients., Results: In group 1, pathologic examination revealed an association of dysgerminoma with gonadoblastoma (n = 2), bilateral gonadoblastoma (n = 2) and streak gonads (n = 2). Prophylactic gonadectomy in asymptomatic patients (group 2) also showed asymptomatic dysgerminoma with gonadoblastoma (n = 1), bilateral gonadoblastoma (n = 2) and streak gonads (n = 2)., Conclusions: A gonadal tumor arising in a girl with pubertal delay may be related to dysgenesis of the gonad. Primary amenorrhea or diagnosis of dysgerminoma should warrant karyotype, and familial study if 46,XY PGD is found. Considering the high incidence of gonadoblastoma and the early occurrence of dysgerminoma, early bilateral gonadectomy is recommended., (Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Non-neurogenic elimination disorders cover a wide spectrum of lower urinary tract and bowel dysfunctions, observed in the absence of a neurological background or lower urinary tract malformation. We reviewed conditions responsible for incontinence during bladder filling with normal voiding patterns (such as overactive bladder, giggle incontinence, post-void dribbling), and dysfunctional voiding syndromes. Dysfunctional elimination syndrome usually includes detrusor-sphincter dyscoordination, small-capacity overactive bladder or decompensated large poorly contractile bladder, and large-volume post-micturition residuals, occasionally associated with bowel dysfunction. At the most severe end of the spectrum lies the non-neurogenic neurogenic bladder syndrome, characterized by the association of a severe impairment of the upper urinary tract with a dysfunctional elimination syndrome. It must be emphasized that if the term 'non-neurogenic' relates to the absence of a neurological lesion, it is however conceivable that these conditions actually do have an underlying neurological cause that remains to be identified., (Copyright (c) 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: Although thought to be an acquired condition, non-neurogenic neurogenic bladder may sometimes be a congenital dysfunction, revealed before toilet training. We report our experience with the condition diagnosed prenatally or during early infancy., Patients and Method: We retrospectively reviewed cases of severe bladder dysfunction with upper-tract impairment, without neurological or obstructive pathology, in children diagnosed before toilet training: five with prenatal diagnosis of severe hydro-ureteronephrosis (group 1) and six with signs of bladder dysfunction during infancy (group 2)., Results: Follow up of group 1 showed decompensation toward severe bladder dysfunction, diagnosed after either toilet training or ureteral reimplantation (n=3). After a median follow up of 14 years (0.5-20), four were on clean intermittent catheterization with bladder augmentation and one required sphincteric re-education with good result. Two of the five had chronic renal failure. In group 2, six children (two females) presented at median age of 20 months (2-30) with indirect signs of bladder dysfunction, including vesicoureteral reflux (n=4) and/or hydro-ureteronephrosis (n=4). After a median follow up of 11 years (5-20), three were on clean intermittent catheterization (two Mitrofanoff channels), and three underwent bladder augmentation. Three children had chronic renal failure of whom one underwent renal transplant., Conclusion: These cases of severe bladder dysfunction were initially misdiagnosed. In both groups, follow up revealed severe dilatation of the upper tract and secondary renal impairment. Antenatal diagnosis of bilateral pyeloureteral dilatation may be the first sign of early bladder dysfunction.