A Single-Center, Observational Study of 607 Children and Young People Presenting With Differences of Sex Development (DSD).

Context: Differences of sex development (DSD) represent a wide range of conditions presenting at different ages to various health professionals. Establishing a diagnosis, supporting the family, and developing a management plan are important.

Objective: We aimed to better understand the presentation and prevalence of pediatric DSD.

One-stage combined delayed bladder closure with Kelly radical soft-tissue mobilization in bladder exstrophy: preliminary results.

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.

Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how?

Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46,XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46,XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments …); gonosomic mosaicisms (45,X/46,XY patients); ovo-testicular DSD; and "non-hormonal/non chromosomal" DSD. Questions are summarized for each DSD group with the support of literature and the feed-back of several world experts.

Timing and nature of reconstructive surgery for disorders of sex development - introduction.

The ideal timing and nature of surgical reconstruction in individuals with Disorders of Sex Development (DSD) is highly controversial. Despite the increasing number of publications on this topic, evidence-based recommendations still cannot be made. However it is generally accepted that optimal care for DSD requires an experienced multidisciplinary team.

Long-term outcome of vaginal reconstruction: comparing techniques and timing.

Objective: In a retrospective review, to analyse the long-term outcomes of vaginal reconstruction, comparing techniques and timing.

Patients And Methods: We identified 63 patients who underwent a total of 71 vaginoplasties in 1985-2000. The techniques used were posterior skin flap (42), intestinal replacement (21) and pull through (8).

Adenocarcinoma at the ureterosigmoidostomy site in a 16-year-old demonstrates the importance of screening in children.

We report the death of a 17-year-old male exstrophy patient from adenocarcinoma arising at the ureterosigmoidostomy. This was detected at reconstructive surgery at the age of 16 years, 13 years after ureterosigmoidostomy. This case highlights the importance of including children in endoscopic surveillance.

Injectable polydimethylsiloxane for treating incontinence in children with the exstrophy-epispadias complex: long-term results.

Objective: To present our experience with the use of injectable polydimethylsiloxane (Macroplastique, Uroplasty, Minneapolis, MI, USA) for treating incontinence in children with the exstrophy-epispadias complex (EEC), as incontinence continues to be a challenging problem in such children, and although the primary management of EEC has developed over the last few decades, with early closure and reconstruction of the penis, achieving satisfactory continence status remains elusive.

Patients And Methods: We retrospectively reviewed the hospital records of 52 patients (41 boys and 11 girls, mean age at first injection 6.6 years, range 3.

Long-term followup of patients after redo bladder neck reconstruction for bladder exstrophy complex.

Purpose: The aim of this study was to determine whether redo bladder neck reconstruction is effective in achieving continence after a failed bladder neck reconstruction procedure.

Materials And Methods: We retrospectively reviewed the hospital records of patients with bladder exstrophy who had undergone redo bladder neck reconstruction. There were 30 patients in the study, including 20 boys and 10 girls.

Y-type urethral duplication in the male.

Objective: To present a single series of Y-type duplication with an analysis of the presenting features, the management and outcome, as complete Y-type urethral duplication in the male is a rare congenital anomaly that presents many challenges.

Patients And Methods: We retrospectively reviewed patient charts, identifying those with urethral duplication, and then those with complete Y-type duplication. The age at presentation, investigations, management and outcomes were reviewed.

Urinary retention, erectile dysfunction and penile anaesthesia after circumcision: a mixed dissociative (conversion) disorder.

We report on the long-term followup of a mixed dissociative (conversion) disorder after circumcision in childhood.

Renal outcome following heminephrectomy for duplex kidney.

Purpose: We evaluated functional outcome following heminephrectomy in duplex kidneys.

Materials And Methods: A total of 101 patients were identified who underwent upper or lower pole heminephrectomy between January 1995 and December 2000. We retrospectively reviewed 60 case notes with complete data.

Renal function and outcome following salvage surgery for bilateral Wilms' tumor.

Purpose: Maintaining adequate renal function without compromising cure in children with bilateral Wilms' tumor is surgically demanding and challenging. The aim of this study was to assess renal function and outcome in children treated in one institution for bilateral Wilms' disease by renal salvage procedures.

Methods: This study is a retrospective review of all patients with histologically proven bilateral nephroblastoma who underwent a renal salvage procedure between November 1973 and June 2002.

Use of the silastic sheath in bladder neck reconstruction.

Objective: The study compared two populations of patients undergoing bladder neck reconstruction using the silastic sheath in two major pediatric centers. The success with this technique was markedly different in the two centers. The purpose of the study was to determine factors that might explain the divergent results.

Cloacal exstrophy: morbidity associated with abnormalities of the gastrointestinal tract and spine.

Background/purpose: The aim of this study was to review the management of the gastrointestinal tract in cloacal exstrophy and to assess the effect of spinal dysraphism on nutritional outcome.

Methods: Twenty-two patients with cloacal exstrophy were reviewed retrospectively. Gastrointestinal and spinal anomalies were documented.

Long-term outcome of Fowler-Stephens orchiopexy in boys with prune-belly syndrome.

Purpose: Intra-abdominal testes in boys with prune-belly syndrome have been conventionally managed by 1 or 2-stage orchiopexy with division of the gonadal vessels. We reviewed a series of adults with prune-belly syndrome to assess the morphological and functional outcome of orchiopexy in childhood with specific reference to the spontaneous onset of puberty, hormonal profiles and sexual function.

Materials And Methods: A total of 41 boys were divided into 3 groups depending on the type of orchiopexy performed, namely group 1-20 with bilateral 1-stage orchiopexy, group 2-10 with unilateral 1-stage and contralateral 2-stage orchiopexy, and group 3-11 with bilateral 2-stage orchiopexy.

A novel, inexpensive, double lumen suprapubic catheter for urodynamics.

Purpose: We describe a novel, double lumen, intravesical, suprapubic catheter designed to meet the requirements of pediatric urodynamics that is easy to use and has minimal complications.

Materials And Methods: A commercially available 10Fr pediatric suprapubic pigtail catheter forms the outer lumen for instilling filling media. A 16 gauge epidural catheter is inserted through the outer catheter providing an inner lumen for measuring intravesical pressures.

[The effect of enterocystoplasty in childhood on linear growth].

Objective: The assumption that enterocystoplasty in children has a detrimental effect on linear growth has been based almost exclusively upon a chance finding in a retrospective study 10 years ago. We re-evaluated the same research question in a larger cohort and with a longer follow-up.

Patients And Methods: Between 1982 and 1997, 242 children and adolescents underwent enterocystoplasty.

The effect of enterocystoplasty on bone strength assessed at four different skeletal sites in a rat model.

The objective of the study was to investigate bone strength at four different skeletal sites in a chronic animal model of urinary diversion. Young male Wistar rats (120) were allocated randomly to four groups undergoing ileocystoplasty; ileocystoplasty and resection of the ileocecal segment; colocystoplasty; or sham operation (controls). After 8 months the lumbar vertebrae, femora, and tibiae were harvested at necropsy.

Long-term gynecological outcome of patients with persistent cloaca.

Purpose: Persistent cloaca is a complex malformation that remains a difficult reconstructive challenge, and data on long-term outcome are scarce. Gynecological abnormalities are common with cloaca but may remain asymptomatic until puberty or adult life. We evaluate long-term gynecological sequelae in these patients with persistent cloaca.