Impact of pelvic osteotomy on the incidence of inguinal hernias in classic bladder exstrophy.

Background/purpose: The high prevalence of inguinal hernias in the bladder exstrophy population is well documented. The authors' aim is to determine whether pelvic osteotomy reduces the incidence of primary and recurrent inguinal hernias in patients with classic bladder exstrophy.

Methods: Using an institutionally-approved database, patients who underwent immediate or delayed primary bladder closure between 1974 and 2012 were identified and stratified by the use of pelvic osteotomy at the time of closure.

Safety and efficacy of staged pelvic osteotomies in the modern treatment of cloacal exstrophy.

Introduction And Objective: Staged pelvic osteotomy (SPO) prior to bladder closure has been shown to be a safe and effective method for achieving pubic approximation in cloacal exstrophy (CE) patients with extreme diastasis. However, SPO outcomes have never been compared to those for combined pelvic osteotomy (CPO) at the time of closure in CE patients.

Methods: A prospectively maintained database of 1208 exstrophy-epispadias complex patients was reviewed for CE patients treated with pelvic osteotomies.

Inflatable penile prosthesis technique and outcomes after radial forearm free flap neophalloplasty.

The aim of this study was to describe the technical aspects and short-term outcomes of inflatable penile prosthesis (IPP) implantation after neophallus reconstruction at a single institution. Nine men with previously constructed radial forearm neophalli underwent IPP implantation. The etiologies of their penile anomaly were bladder exstrophy complex in five, disorder of sexual differentiation in two and genital obliteration secondary to ballistic trauma in two.

Initial bladder closure of the cloacal exstrophy complex: outcome related risk factors and keys to success.

Purpose: This study examines a large single-institution experience with cloacal exstrophy patients, analyzing patient demographics and surgical strategies predictive of bladder closure outcomes.

Methods: One hundred patients with cloacal exstrophy were identified. Complete closure history including demographics, operative history, and outcomes was available on 60 patients.

Bladder capacity as a predictor of voided continence after failed exstrophy closure.

Objective: To evaluate potential predictors of voided continence among bladder exstrophy patients with a history of a failed closure.

Patients And Methods: The authors reviewed all patients who underwent a bladder neck reconstruction (BNR) with a history of one or more failed exstrophy closures between 1979 and 2007. The following data were collected for each patient: number of failures, site of surgery, mode of failure, presence of osteotomy, bladder capacity, need for additional procedures, and continence status.

Role of urethral bulking agents in epispadias-exstrophy complex patients.

Objective: Injection of urethral bulking agents (UBA) has been used to increase bladder capacity prior to bladder neck reconstruction (BNR) or as an adjuvant therapy following BNR to improve continence. The purpose of this study was to determine the effectiveness of urethral injections in the exstrophy population.

Materials And Methods: A review was performed of patient characteristics, bladder capacity, and continence status of all patients with the exstrophy-epispadias complex who underwent injection of UBA between 1980 and 2008.

Modern management of bladder exstrophy repair.

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall musculature, and pelvic bones. Historically, surgical outcomes in patients with classic bladder exstrophy, the most common presentation of the exstrophy-epispadias complex, were poor. However, modern techniques have increased the success of achieving urinary continence, satisfactory cosmesis, and improved quality of life.

Novel mutation among two sisters with 17β hydroxysteroid dehydrogenase type 3 deficiency.

The clinical presentations of 17β hydroxysteroid dehydrogenase type 3 (17β-HSD3) deficiency, 5α-reductase type 2 deficiency, and complete androgen insensitivity syndrome can be similar. However, those with 17β-HSD3 deficiency and 5α-reductase type 2 deficiency will develop virilization and should undergo gonadectomy after genetic testing before the age of puberty if reared in the female sex. Two sisters were initially diagnosed with complete androgen insensitivity syndrome as young children after testes were discovered during hernia surgery.

Radial forearm free flap phalloplasty for penile inadequacy in patients with exstrophy.

Purpose: Male patients with bladder and cloacal exstrophy are born with demure genitalia and often desire phalloplasty during late adolescence or early adulthood. Radial forearm free flap phalloplasty was used successfully for congenital aphallia in a few small series. We present a series of patients treated with phalloplasty using a radial forearm free flap after the repair of bladder and cloacal exstrophy.

Persistent vesicocutaneous fistula after repair of classic bladder exstrophy: a sign of failure?

Objective: To describe the potential predisposing clinical characteristics at initial closure, the treatments of and outcomes associated with vesicocutaneous fistula (VCF) after primary bladder closure.

Materials And Methods: Eighteen patients were referred for VCF after primary bladder exstrophy closure. Aspects from the primary closure such as gender, timing of diagnosis and repair, osteotomies, pelvic immobilization, layers of closure, use of tissue adjuncts, and complications in addition to details from their required treatment of VCF were retrospectively extracted from the medical record.

Developing a pediatric pain service.

Pediatric pain services were first established in larger pediatric centers over two decades ago. Children's acute pain was poorly managed at the time owing to misconceptions, safety concerns, and variability in practice. While many larger pediatric centers now have acute pain services, there remains a need for better pain management in facilities and geographic locations with fewer resources.