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.

Cloacal Exstrophy Closure Without Osteotomy and Immobilization: A Recipe for Failure.

Background: Cloacal exstrophy (CE) remains one of the most severe birth defects compatible with life with a constellation of anomalies involving the bladder, genitalia, hindgut, and spinal cord. Pelvic osteotomy and immobilization have been utilized to facilitate bladder closure, yet their role as adjuncts remains a topic of debate. The authors sought to evaluate the outcomes of CE closure without the use of osteotomy or lower extremity (LE)/pelvic immobilization.

A Double-blinded Randomized Controlled Trial Assessing the Efficacy of Opioid Disposal Instructions with Parental Education on Proper Opioid Disposal Rates Following Ambulatory Pediatric Urologic Surgery.

Objectives: To determine if the administration of standardized opioid disposal instructions with focused parental education improves proper disposal of leftover opioid medication among families of children undergoing ambulatory urologic surgery compared to routine postoperative instructions.

Methods: A prospective, double-blinded, single-center randomized controlled trial was conducted in children 6-18 years undergoing ambulatory urology procedures between October 2021 and April 2023. Patients were randomized (1:1) to receive either the Food and Drug Administration (FDA) opioid disposal best practices worksheet plus nursing parental education or routine postoperative instructions alone.

A Rocky Road: Bladder Stones in the Augmented Exstrophy-Epispadias Complex Patient.

Objective: To determine the rate of stone formation amongst patients of the exstrophy-epispadias complex with augmentation cystoplasty. We hypothesize that bowel segment choice influences the rate of stone formation after bladder augmentation and the rate of complications from bladder stone surgery.

Methods: An IRB-approved institutional database of 1512 exstrophy-epispadias patients was reviewed retrospectively.

Stomal Stenosis After Continent Urinary Diversion in Bladder Exstrophy: Risk Factors and Management.

Objective: To identify risk factors for stenosis and compare management strategies for stenosis etiology and to examine the efficacy of each approach. Patients with classic bladder exstrophy (CBE), a rare genitourinary malformation, may require construction of a continent urinary stoma (CUS) if incontinence persists. Stomal stenosis is a challenging complication as it is common, progressive, and recurrent.

Perioperative management of primary classic bladder exstrophy: A single institutional pathway to success.

Purpose: Appropriate perioperative management is crucial in patients undergoing classic bladder exstrophy closure (CBE). Therefore, the authors sought to review their intra and postoperative management of patients with CBE undergoing primary closure and examine the impact of this pathway on patient outcomes.

Method: A prospectively maintained institutional approved exstrophy-epispadias complex database was reviewed for patients with CBE who had undergone primary closure between 2016 and 2022 and whose closure was performed within one year of age.

Clinical patterns and implications of prescription opioid use in a pediatric population for the management of urolithiasis in the emergency room.

Extrapolations from the adult population have suggested that opioids should be avoided in the management of pediatric urolithiasis, but the literature is sparse with regards to actual practice patterns and the downstream implications. We sought to investigate the rate of oral opioid administration for children presenting to the emergency room (ER) with urolithiasis and to identify associations between opioid administration and return visits and persistent opioid use. The TriNetX Research and Diamond Networks were used for retrospective exploratory and validation analyses, respectively.

De novo variants identified by trio whole exome sequencing of bladder exstrophy epispadias complex.

Bladder exstrophy epispadias complex (BEEC) encompasses a spectrum of conditions ranging from mild epispadias to the most severe form: omphalocele-bladder exstrophy-imperforate anus-spinal defects (OEIS). BEEC involves abnormalities related to anatomical structures that are proposed to have a similar underlying etiology and pathogenesis. In general, BEEC, is considered to arise from a sequence of events in embryonic development and is believed to be a multi-etiological disease with contributions from genetic and environmental factors.

Higher prevalence of benign tumors in men with testicular tumors and history of treated cryptorchidism.

Objectives: To identify if surgically treated cryptorchidism correlated with testicular tumor pathology at presentation.

Materials And Methods: An institutional database of patients treated for testicular cancer between 2003 and 2020 was reviewed. Inclusion criteria included orchiectomy patients.

Effectiveness of Liposomal Bupivacaine With Bupivacaine Hydrochloride vs Bupivacaine Hydrochloride Alone as a Local Anesthetic for Children Undergoing Ambulatory Urologic Surgery: The Baby ORIOLES Randomized Clinical Trial.

Purpose: We sought to determine if the addition of liposomal bupivacaine to bupivacaine hydrochloride improves opioid-free rate and postoperative pain scores among children undergoing ambulatory urologic surgery.

Materials And Methods: A prospective, phase 3, single-blinded, single-center randomized trial with superiority design was conducted in children 6 to 18 years undergoing ambulatory urologic procedures between October 2021 and April 2023. Patients were randomized 1:1 to receive dorsal penile nerve block (penile procedures) or incisional infiltration with spermatic cord block (inguinal/scrotal procedures) with weight-based liposomal bupivacaine plus bupivacaine hydrochloride or bupivacaine hydrochloride alone.

Achieving goal capacity for continence surgery: A cumulative event analysis of bladder exstrophy patients.

Background: Following successful closure of patients with classic bladder exstrophy (CBE), the next major milestone is the establishment of urinary continence. Prior to determining the most appropriate continence surgery, it is imperative to reach an adequate bladder capacity minimum of 100 cc in order to make the decision between bladder neck reconstruction (BNR) or continent stoma, with or without augmentation cystoplasty (AC).

Objective: To examine the timing of when patients achieve threshold bladder capacity for BNR eligibility.

Bladder capacity and growth in classic bladder exstrophy: A novel predictive tool.

Introduction: Bladder capacity (BC) is an important metric in the management of patients with classic bladder exstrophy (CBE). BC is frequently used to determine eligibility for surgical continence procedures, such as bladder neck reconstruction (BNR), and is associated with the likelihood of achieving urinary continence.

Objective: To use readily available parameters to develop a nomogram that could be used by patients and pediatric urologists to predict BC in patients with CBE.

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.

Safety and Efficacy of Long-Acting Liposomal Bupivacaine Plus Bupivacaine Hydrochloride for Dorsal Penile Block During Ambulatory Pediatric Urologic Surgery.

This study evaluates the tolerability and efficacy of preoperative dorsal penile nerve block with Exparel plus bupivacaine hydrochloride in children>6 years old undergoing ambulatory urologic surgery. We demonstrate that the drug combination is well-tolerated, with appropriate analgesic efficacy in the recovery room as well as at 48-hour and 10-14 day follow-up periods. These preliminary data justify the need to perform a prospective, randomized trial comparing Exparel plus bupivacaine hydrochloride to other common local anesthetic regimens used in pediatric urologic surgery.

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).

Complications of delayed and newborn primary closures of classic bladder exstrophy: Is there a difference?

Introduction: The authors aim to compare single institutional 30-day complication rates between delayed and neonatal closure of classic bladder exstrophy (CBE).

Materials And Methods: An institutional database of 1415 exstrophy-epispadias patients was reviewed retrospectively for CBE patients who underwent primary closures at the authors' institution between 1990 and 2020. Patients were identified as having received either neonatal or delayed (at age >28 days) closures.

Abdominal Mass in a Phenotypic Female with 46,XY Differences in Sex Development.

We present a case of a large intra-abdominal mass found to be localized pure seminoma within a retained gonad of a 53-year-old phenotypic female with 46,XY differences in sex development (DSD) and androgen insensitivity syndrome (AIS). Our management included extirpation of the mass with contralateral gonadectomy. Historically, patients with AIS would undergo gonadectomy to mitigate the lifetime risk of testicular germ cell tumor development; however, growing evidence suggests safety in retention and surveillance of these gonads into adulthood.

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.