Chromophobe Renal Cell Carcinoma in a Pediatric Patient With Neurofibromatosis Type 1: A Case Report and Review of the Literature.

Renal Cell Carcinoma is rare in the pediatric population, making up only 2%-6% of all pediatric renal tumors. Literature on pediatric Chromophobe Renal Cell Carcinoma (chRCC) is exceptionally limited. In this report, we describe the case of a 12-year-old patient with Neurofibromatosis Type 1 (NF1), incidentally found to have a kidney lesion with pathology revealing chRCC.

Multi-Institutional Bladder Exstrophy Consortium After 8 Years: The Short- and Intermediate-Term Outcomes.

Purpose: Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real-time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long-term protection of the upper tracts. This study reports on our short-term complications and intermediate-term continence outcomes.

Pelvic Ectopic Kidney Prevalence and Pressure Changes During Cloacal Exstrophy (Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome) Closure.

Objectives: To assess the prevalence of pelvic ectopic kidneys (PEK) and compare renal parameters of the PEK to the orthotopic kidney following pubic bone approximation.

Methods: In four Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome (OEIS) patients undergoing second-stage repair with known pelvic and orthotopic kidneys, changes in the renal pelvis pressure (RPP), peak systolic velocity (PSV), and resistive index (RI) were measured in the pelvic and orthotopic kidneys following pubic bone approximation. A paired t-test was performed for analysis.

Early urodynamic findings after complete primary repair of exstrophy.

Introduction: Detrusor contraction in bladder exstrophy (BE) patients following reconstruction is poorly understood as there are few published studies assessing urodynamic findings in this population. Understanding the ability of the detrusor to contract in BE patients early after closure may be able to inform the longer-term management and potential for the development of future continence in this population.

Objective: We sought to evaluate early detrusor contraction using urodynamic studies (UDS) in children who had previously undergone complete primary repair of bladder exstrophy (CPRE).

Prenatal Diagnosis of Bladder Exstrophy and OEIS over 20 Years.

Objective: To examine the prenatal diagnosis rates of bladder exstrophy (BE) and Omphalocele-Exstrophy-Imperforate anus-Spinal Defect Syndrome (OEIS) in a large cohort of patients over a 20-year period. We hypothesized that prenatal diagnosis rates improved over time due to evolving techniques in fetal imaging.

Methods: A multi-institutional database was queried to identify BE or OEIS patients who underwent primary closure between 2000 and 2020.

Technical details and long-term outcomes of P.A.D.U.A. for congenital urethral narrowing; a case series and review of the literature.

Introduction: The P.A.D.

Repurposing tools: A peña stimulator as a tool to map the limits of dissection and continence zone during bladder exstrophy repair.

Introduction: The complete primary repair of bladder exstrophy (CPRE) aims to restore normal anatomy through complete mobilization and reapproximation of the bladder neck and proximal urethra.

Methods: The Peña stimulator has previously been used to identify musculature in the pelvis. The device is now used to distinguish intersymphyseal bands from pelvic floor and urethral sphincteric musculature during CPRE.

Bilateral ureteral reimplantation at complete primary repair of exstrophy: Post-operative outcomes.

Background And Study Objective: The value of bilateral ureteral reimplant (BUR) at the time of complete primary repair of bladder exstrophy (CPRE) has been suggested, however, outcomes are poorly characterized in current medical literature. We hypothesize that BUR at time of CPRE will decrease the rate of recurrent pyelonephritis, post-operative vesicoureteral reflux (VUR), and the need for subsequent ureteral surgery.

Study Design: We analyzed 64 consecutive patients with a diagnosis of classic bladder exstrophy (BE) who underwent CPRE at three institutions from 2013 to 2019.

The Richard Grady Monsplasty: A vertical Z-plasty technique.

Classic bladder exstrophy in the female results in an exstrophic bladder and urethra, an anterior introitus with a bifid clitoris and short labia minora. During closure, the lower abdominal wall is closed and the bifid clitori are brought into close apposition, but are often not completely closed to prevent injury to the clitoral bodies, thus leaving a persistent gap between the clitoral bodies that grows over time. We demonstrate a vertical z plasty closure to provide a 2 layer closure of the mons that decreases tension and improves cosmetic appearance by recreating a clitoral hood that provides a more normal appearance of the external genitalia for girls with bladder exstrophy.

Timing of inguinal hernia following complete primary repair of bladder exstrophy.

Introduction/background: Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder exstrophy patient is under greatest risk of developing an inguinal hernia following bladder closure is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity if not addressed in a timely fashion. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood.

Key anatomic findings on fetal ultrasound and MRI in the prenatal diagnosis of bladder and cloacal exstrophy.

Introduction: Prenatal determination of bladder exstrophy (BE) or cloacal exstrophy (CE), known also as the omphalocele-exstrophy-imperforate anus-spinal anomaly complex (OEIS), is challenging. Distinguishing between BE and CE is important because children with CE have many more challenges initially and during their lifetime. An accurate diagnosis is critical when counselling expectant parents.

Evaluation of outcomes following complete primary repair of bladder exstrophy at three individual sites prior to the establishment of a multi-institutional collaborative model.

Purpose: The Multi-Institutional Bladder Exstrophy Consortium (MIBEC) was established in 2013 to refine technical aspects of the complete primary repair of bladder exstrophy (CPRE), to decrease complications, and to improve outcomes. In order to place outcomes from the consortium into context of historic outcomes, we evaluated continence and dry intervals in children who were repaired prior to the beginning of the consortium at these institutions. We hypothesized that continence (voiding with dryness) is rarely achieved after primary CPRE and surgery following CPRE improves dryness but may hinder voiding per urethra.

A comparison of clinical outcomes of acute testicular torsion between prepubertal and postpubertal males.

Short Introduction/background: Surgical intervention for acute testicular torsion can require either orchiopexy or orchiectomy. The decision of which surgery to perform is dependant on the amount of time that the testicle experienced ischemia and the viability of the testicle after reperfusion.

Objective: It is hypothesized that (1) there is a difference in orchiectomy and orchiopexy rates between prepubertal and postpubertal males with acute testicular torsion and (2) presenting symptoms may vary between the two age groups as prepubertal males may present with atypical symptoms, which could result in delayed presentation and diagnosis.

The implications of fellowship expansion on future pediatric urologist surgical volumes.

Introduction: Over the past 25 years, Pediatric Urology fellowship programs accredited by the Accreditation Council for Graduate Medical Education (ACGME) have more than doubled. This increase may lead to a significant decrease in the number of operative cases per surgeon and therefore impact the current practice of pediatric urology.

Objective: The objective in conducting this study is to try and predict the effect of the current number of pediatric urology fellowship training positions on future case volume per surgeon using a mathematical model and to discuss future management of the pediatric urology workforce.

Antibiotic Prophylaxis with Trimethoprim-Sulfamethoxazole versus No Treatment after Mid-to-Distal Hypospadias Repair: A Prospective, Randomized Study.

Purpose: To evaluate the impact of prophylactic antibiotics after distal hypospadias repair on postoperative bacteriuria, symptomatic urinary tract infection, and postoperative complications in a prospective, randomized trial.

Materials And Methods: Consecutive patients aged 6 months to 2 years were enrolled at our institution between June 2013 and May 2017. Consenting patients were randomized to antibiotic prophylaxis with trimethoprim-sulfamethoxazole versus no antibiotic.

Postoperative Immobilization and Pain Management After Repair of Bladder Exstrophy.

Surgical repair of bladder exstrophy is an ongoing challenge for pediatric urologists. Postoperative immobilization is a mainstay of care to decrease tension on the repair site and is often utilized in conjunction with pelvic osteotomies performed in the same operative setting by pediatric orthopedic surgeons. Multiple pelvic immobilization techniques have been developed in conjunction with repair techniques including special techniques for neonates.

Genetic Evaluation of E. coli Strains Isolated from Asymptomatic Children with Neurogenic Bladders.

This study was conducted to describe the genetic profiles of E. coli that colonize asymptomatic pediatric neurogenic bladders. E.

Clinical pathway for early discharge after complete primary repair of exstrophy and epispadias by using a spica cast.

Introduction/objective: Secure closure of the pubic diastasis during bladder exstrophy and epispadias repair decreases the abdominal wall tension at the time of reconstruction. Pelvic osteotomies are routinely performed at the time of abdominal wall and bladder reconstruction in order to more easily facilitate pubic symphyseal diastasis approximation. Postoperative pelvic immobilization is performed by methods that include modified Buck's traction, modified Bryant's traction, and spica casting.

An initial report of a novel multi-institutional bladder exstrophy consortium: a collaboration focused on primary surgery and subsequent care.

Purpose: For bladder exstrophy repair it is universally accepted that successful initial surgery is paramount to achieve the optimal outcome. Gaining the necessary surgical experience is challenging due to the rarity of bladder exstrophy. We report preliminary findings of a multi-institutional collaboration created to increase experience and proficiency with the care of bladder exstrophy.

Urinary proteome analysis in patients with stable SFU grade 4 ureteropelvic junction obstruction differs from normal.

Objective: To evaluate and analyze the urinary proteome in infants with stable grade 4 ureteropelvic junction obstruction (UPJO) and compare to age-matched normal controls.

Methods: Bladder urine specimens were obtained from 21 healthy infants with normal maternal/fetal ultrasound and 25 infants with grade 4 unilateral UPJO. All patients had >40% ipsilateral individual kidney function by renal scanning and the anteroposterior (AP) diameter of the hydronephrotic kidney ranged from 1.

Technique for Resonance® stent exchange in patients with extrinsic obstruction: description of a novel approach and literature review.

Malignant ureteral obstruction is a morbid diagnosis and can be difficult to manage. Ureteral stents often fail as the disease progresses. Metallic stents have provided a longer-term option for decompression as they inherently resist compression and require changes left often.

Delayed onset ureteral obstruction following Deflux® injection for vesicoureteral reflux.

Endoscopic injection treatment of vesicoureteral reflux is an increasingly common and successful option. Obstruction is an infrequent postoperative complication, occurring in 1% of patients; delayed onset of obstruction is even rarer. There is a paucity of literature describing possible treatments.

Nonsurgical management of the neurogenic bladder in children.

The neurogenic bladder can have many serious consequences. However, with proper understanding of the disease spectrum, and with proper awareness of the surveillance and management tools, adverse outcomes can be minimized. The incidence of renal failure is uncommon.