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.

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.

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 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.

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 ultimate challenge of cloacal exstrophy.

Purpose: Our review addresses the various system abnormalities associated with cloacal exstrophy and revisits the question of gender assignment. Gender assignment decisions and psychological aspects of gender issues have become the "ultimate challenge." Exploration of gender identity, gender role behavior and sexual orientation has just begun.