Bladder exstrophy in adulthood: A narrative review of transitional health.

Background: Exstrophy-epispadias complex (EEC) classically presents as bladder exstrophy (BE) and requires lifetime urologic care. As men and women with BE age, there is an often difficult period of transition to adulthood in terms of addressing urologic and general health challenges. BE can lead to many urinary and sexual health issues as these patients age, which is often made more complex given their past surgical history and anatomy.

Prevalence of opioid and benzodiazepine use in adult patients with the exstrophy-epispadias complex.

Introduction: Bladder exstrophy (BE), cloacal exstrophy (CE), and epispadias (E) are variants of the exstrophy-epispadias complex (EEC). These children require opioids and benzodiazepines to achieve pain management and immobilization for a lifetime of surgeries. It is hypothesized that these children would be sensitized to opiates and benzodiazepines as adults.

Bladder exstrophy-epispadias complex related litigation: A legal database review.

Objective: To review the nature and extent of bladder exstrophy-epispadias related malpractice litigation in the United States.

Methods: Two legal databases (Nexis Uni, WestLaw) were reviewed for state and federal cases using the terms "bladder exstrophy", "cloacal exstrophy", "epispadias", in combination with "medical malpractice", or "negligence", or "medical error", or "complication", or "malpractice", or "tort". Databases were queried from 1948 to 2022 and reviewed for medical and legal details.

Inflammatory Expression Profiles in Bladder Exstrophy Smooth Muscle: Normalization Over Time.

Objective: To test the hypothesis that phenotypes in bladder exstrophy result from alterations in detrusor smooth muscle cell (SMC) gene expression.

Methods: We generated primary human bladder smooth muscle cell lines from patients with classic bladder exstrophy (CBE) undergoing newborn closure (n = 6), delayed primary closure (n = 5), augmentation cystoplasty (n = 6), and non-CBE controls (n = 3). Gene expression profiles were then created using RNA sequencing and characterized using gene set enrichment analysis (GSEA).

The exstrophy experience: A national survey assessing urinary continence, bladder management, and oncologic outcomes in adults.

Introduction And Objective: The bladder exstrophy-epispadias complex (BEEC) is a rare spectrum of congenital genitourinary malformations with an incidence of 1:10,000 to 1:50,000. Advances in reconstructive surgical techniques have improved clinical outcomes, but there is a paucity in data about disease sequela in adulthood. This is the largest survey to date in the United States exploring the urinary continence, bladder management, and oncologic outcomes in adults with BEEC.

Postphalloplasty urinary function test: an observational study of novel outcome instrument to capture urinary dysfunction and quality of life after phalloplasty.

Due to growing social acceptance, there has been an increasing number of gender-affirmation surgeries performed in North America. Most research in this patient population focuses on surgical outcomes and advancing techniques. However, little work has been done to study functional outcomes.

Parent Experience of Communication about Children's Surgery: A Qualitative Analysis.

Unlabelled: Parent experience is a core component of the quality of pediatric care and an increasingly common focus of quality improvement initiatives. However, the parent experience of communication in the pediatric surgical setting remains unexplored.

Methods: We conducted semi-structured interviews with 20 parents of children undergoing surgery.

Osteotomy in the newborn classic bladder exstrophy patient: A comparative study.

Introduction: Pelvic osteotomy is indicated in classic bladder exstrophy (CBE) patients with a wide pubic diastasis or non-malleable pelvis. While the safety of pelvic osteotomy in delayed and failed closures is established, there remains less clarity on their safety in newborns. The authors herein sought to present their experience with CBE patients who underwent pelvic osteotomy for assistance with bladder closure during both the newborn and delayed time periods.

Understanding Bias in Surgery: Perceived Cultural Similarity Between Surgeons and Patient Families.

Objective: We describe surgeon and parent perceptions of similarity toward each other and evaluate differences in the perceptions of similarity by race.

Study Design: Observational cohort analysis.

Setting: Three outpatient sites.

Antireflux Surgery at National Surgical Quality Improvement Program-Pediatric Hospitals.

Purpose: This study aims to examine contemporary practice patterns and compare short-term outcomes for vesicoureteral reflux procedures (ureteral reimplant/endoscopic injection) using National Surgical Quality Improvement Program-Pediatric data.

Materials And Methods: Procedure-specific variables for antireflux surgery were developed to capture data not typically collected in National Surgical Quality Improvement Program-Pediatric (eg vesicoureteral reflux grade, urine cultures, 31-60-day followup). Descriptive statistics were performed, and logistic regression assessed associations between patient/procedural factors and outcomes (urinary tract infection, readmissions, unplanned procedures).

Practice patterns in classic bladder exstrophy: A global perspective.

Introduction: While evaluation and management options for classic bladder exstrophy (CBE) patients are numerous and varied, little is known regarding the relative utilization of these different methods throughout the world. A large group of exstrophy surgeons practicing globally was surveyed, seeking to document their methods of care.

Methods: A list of international exstrophy surgeons' email addresses was compiled using professional contacts and referral networking.

Exploration of Practice Patterns in Exstrophy Closures: A Comparison Between Surgical Specialties Using a National and Institutional Database.

Objective: To compare the surgical subspecialties performing bladder exstrophy closures and characterize their practice patterns using both a national and institutional database.

Methods: The National Surgical Quality Improvement Program Pediatric (NSQIPP) database was reviewed for all bladder exstrophy closures performed from 2012 to 2017. A single institutional exstrophy-epispadias complex database of 1337 patients was reviewed for patients with a bladder closure at a referring institution from 1975 to 2018.

Novel Observations of Female Genital Anatomy in Classic Bladder Exstrophy Using 3-Dimensional Magnetic Resonance Imaging Reconstruction.

Purpose: Understanding the distinct female anatomy in classic bladder exstrophy is crucial for optimal reconstructive and functional outcomes. We present novel quantitative anatomical data in females with classic bladder exstrophy before primary closure.

Materials And Methods: 3-Dimensional reconstruction was performed in patients undergoing pelvic magnetic resonance imaging, and pelvic anatomy was characterized, including measurements of the vagina, cervix and erectile bodies.

Cystectomy in the Pediatric Exstrophy Population: Indications and Outcomes.

Objective: To explore the long-term outcomes and indications for cystectomy in patients with bladder exstrophy. Although rare, cystectomy is the final surgical alternative to bladder repair among these patients with a poor quality bladder template.

Methods: A prospectively maintained database of 1298 patients with exstrophy-epispadias complex was reviewed for patients who underwent cystectomy between 1970 and 2015 at the authors' institution.

The Surgical Management of Bladder Polyps in the Setting of Exstrophy Epispadias Complex.

Objective: To investigate the surgical course for treating bladder polyps in patients with exstrophy epispadias complex (EEC). EEC bladder polyps are unique pathologic entities, with a distinct difference between polyps discovered at birth and polyps developed after failed exstrophy closure.

Methods: A prospectively maintained database of 1300 patients with EEC was reviewed for bladder exstrophy patients with bladder polyps.

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.

Impact of nephrectomy on long-term renal function in non-syndromic children treated for unifocal Wilms tumor.

Objective: The present study is designed to assess the long-term renal function of children who underwent radical nephrectomy for unifocal Wilms tumor.

Methods: A single institution retrospective cohort study of non-syndromic children treated with radical nephrectomy for unifocal Wilms tumor between 1995 and 2011 was performed to identify risk factors for decreased glomerular filtration rate (GFR). The primary endpoint was decrease in age-adjusted GFR below normal published ranges.

Alemtuzumab with corticosteroid minimization for pediatric deceased donor renal transplantation: a seven-yr experience.

Alemtuzumab is a monoclonal antibody targeting CD52 receptors on B and T lymphocytes and is an effective induction agent in pediatric renal transplantation. We report a seven-yr experience using alemtuzumab induction and steroid-free protocol in the pediatric population as safe and effective. Twenty-one pediatric deceased donor renal transplants were performed at a single academic institution.