Simultaneous second-stage Fowler-Stephens Orchiopexy and microvascular testicular autotransplantation in a patient with prune belly syndrome with cryptorchidism.

We present a case of simultaneous second-stage Fowler-Stephens Orchiopexy (FSO) with microvascular testicular autotransplantation for cryptorchidism and in a patient with prune belly syndrome. At 5 months old, the patient underwent laparoscopic bilateral first-stage FSO with the right testicle located 1 cm from the liver and the left slightly more caudal. An ultrasound on postoperative Day 72 following second-stage FSO and microvascular autotransplantation showed patent testicular vasculature.

3-Dimensional Magnetic Resonance Imaging Guided Pelvic Floor Dissection for Bladder Exstrophy: A Single Arm Trial.

Purpose: We determined the safety and efficacy of intraoperative magnetic resonance imaging guided surgical reconstruction of bladder exstrophy for the identification of the urogenital diaphragm fibers and the thickened muscular attachments between the posterior urethra, bladder plate and pubic rami.

Materials And Methods: Institutional review board and U.S.

Perioperative Analgesic Management of Newborn Bladder Exstrophy Repair Using a Directly Placed Tunneled Epidural Catheter with 0.1% Ropivacaine.

Effective multimodal analgesia and sedation reduce the postoperative morbidity and mortality associated with newborn bladder exstrophy repair. Epidural analgesia is safe and effective for major surgery in neonates and infants, reducing the need for muscle relaxants, opioids, and ventilator support postoperatively. The risk of epidural catheter colonization typically dictates removal after 3 to 5 days.

Pediatric surgical complications of major genitourinary reconstruction in the exstrophy-epispadias complex.

Purpose: Urinary continence is the goal of exstrophy-epispadias complex (EEC) reconstruction. Patients may require a continent urinary diversion (CUD) if they are a poor candidate for bladder neck reconstruction or are receiving an augmentation cystoplasty (AC) or neobladder (NB). This study was designed to identify the incidence of surgical complications among various bowel segments typically used for CUD.

Duplicated pelvic floor musculature and diastematomyelia in a cloacal exstrophy patient.

Cloacal exstrophy is the most severe and rare form of the exstrophy-epispadias complex, presenting with exposed bladder halves extruding through an abdominal wall defect and variable genitourinary, gastrointestinal, musculoskeletal, and neurological defects. The authors report magnetic resonance imaging findings of a neurologically-intact, 24-month-old female with cloacal exstrophy who presented with anterior spinal dysraphism and diastematomyelia and duplicate pelvic floor musculature. The constellation of defects suggests a common genetic, biochemical, and embryological origin for duplication of the bladder, spinal cord, and pelvic floor muscles occurring in the fourth week of gestation.

Urologic complications of major genitourinary reconstruction in the exstrophy-epispadias complex.

Objective: To present the authors' experiences with urologic complications associated with various techniques used to create a continent stoma (CS), augmentation cystoplasty (AC), and neobladder in the exstrophy-epispadias complex (EEC) population.

Methods: Retrospective review of medical records of patients who underwent CS with or without bladder augmentation were identified from an institutional review board-approved database of 1208 EEC patients. Surgical indications, tissue type, length of hospital stay, age, preoperative bladder capacity, prior genitourinary surgeries, postoperative urological complications, and continence status were reviewed.

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.

Perioperative management of classic bladder exstrophy.

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall muscles, and pelvic structures. Modern surgical repairs focus on reconstruction of the bladder and its adjacent structures, with the goal of achieving urinary continence, a satisfactory cosmetic result, and a high quality of life. Complex surgery in neonates and young children, as well as a prolonged postoperative course require close collaboration between surgeons, anesthesiologists, intensivists, pediatricians, and an experienced nursing staff.

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.

Utility of screening ultrasound after first febrile UTI among patients with clinically significant vesicoureteral reflux.

Objective: To assess the sensitivity and negative predictive value (NPV) of screening renal and bladder ultrasound (RBUS) after initial febrile urinary tract infection (UTI) among patients with clinically significant vesicoureteral reflux (VUR).

Methods: A retrospective review was performed of all children <2 years of age who presented with a febrile UTI between 2004 and 2011. The sensitivity and NPV of initial RBUS was calculated among patients who were found to have high-grade (IV-V) VUR.

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.

Review and management of 46,XY disorders of sex development.

Disorders of sex development (DSD) among 46,XY individuals are rare and challenging conditions. Abnormalities of karyotype, gonadal formation, androgen synthesis, and androgen action are responsible for the multiple disorders that result in undervirilization during development. Phenotypic appearance and timing of presentation are quite variable.

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.

Technique for preservation of penile skin in genital reconstruction: free graft to the scrotum.

Objective: To describe a new technique to preserve the penile epithelium with good cosmetic results when additional surgery is a significant possibility. Complex genital reconstruction can require multiple procedures.

Methods: Seven patients underwent grafting of the penile epithelium to the scrotum during genital reconstruction to bank the tissue for potential future use.

Sexual function after male radical cystectomy in a sexually active population.

Objectives: To assess sexual dysfunction in sexually active men after radical cystectomy (RC) and to determine whether sildenafil citrate can improve erectile dysfunction after surgery.

Methods: The baseline and follow-up data from 49 sexually active male patients (mean age 57.8 +/- 9.

Female sexual dysfunction after radical cystectomy: a new outcome measure.

Objectives: To assess, in our contemporary radical cystectomy (RC) series, female sexual dysfunction and whether the type of diversion affected the occurrence of sexual dysfunction. Outcome data after RC with and without orthotopic diversion has focused primarily on cure, urethral recurrence, and continence.

Methods: The baseline and follow-up data from 27 sexually active female patients (mean age 54.