Urodynamics is pivotal in understanding lower urinary tract pathophysiology. While essential, its utility in pediatric and transitional patient populations has been challenged by evolving paradigms and ongoing controversies. This review elucidates the fundamental principles of urodynamics, its worldwide variations, confounding factors, and most importantly, its critical role in the management of genitourinary conditions. Multiple articles have demonstrated advances and challenges in performing, interpreting, and comparing urodynamic studies. Urodynamics serves as an invaluable tool for assessing lower urinary tract function. Technologies such as needle EMG and machine learning modeling can further enhance its utility, especially in scenarios calling for nuanced approaches to neuro-urologic management.

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. Materials and Methods:A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded. Results:CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant's traction, and 2 in external fixation with Buck's traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals. Conclusions:Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.

Cloacal exstrophy is the most severe congenital anomaly of the exstrophy-epispadias complex and is characterized by gastrointestinal, genitourinary, neurospinal, and musculoskeletal malformations. Individualized surgical reconstruction by a multidisciplinary team is required for these complex patients. Not infrequently, patients need staged surgical procedures throughout childhood and adolescence. Following significant improvements in medical care and surgical reconstructive techniques, nearly all patients with cloacal exstrophy now survive, leading to an increased emphasis on quality of life. Increased attention is given to gender identity and the implications of reconstructive decisions. Long-term sequelae of cloacal exstrophy, including functional continence and sexual dysfunction, are recognized, and many patients require ongoing complex care into adulthood.

Objective: Pelvic osteotomies relieve tension of the bladder and fascial closures during bladder exstrophy repair. Multiple techniques for postoperative immobilization of the pelvis and lower extremities have been described. The primary aim of this study was to assess differences in short and long-term changes in pubic rami diastasis when comparing Bryant traction to spica cast immobilization. Secondary aims included a comparison of length of stay, skin-related complications, and urologic outcomes. Methods: We performed a single-institutional retrospective review of bladder exstrophy patients younger than 18 months of age who underwent posterior pelvic osteotomy and bladder exstrophy closure from April 2005 to April 2020. Short-term and long-term pubic rami diastasis were defined as postoperative measurements <= 6 months and >= 12 months, respectively. Secondary outcomes included length of stay, pressure ulcer, skin rash/abrasion, urethrocutaneous fistula, and bladder or fascial dehiscence rates. Multivariable logistic regression assessed for an association between immobilization type and degree of diastasis while controlling for age at the time of diastasis measurement and sex. Results: Fifteen patients underwent Bryant traction and 36 patients underwent spica cast immobilization. In both the short-term and long-term, there was a greater reduction in pubic diastasis in the spica cast group (P= 0.002 and P= 0.05, respectively). After adjustments, there were higher odds of having a greater reduction in pubic rami diastasis in both the short-term (odds ratio: 2.71, 95% CI: 1.52-4.86, P= 0.001) and long-term (odds ratio: 2.41, 95% CI: 1.00-5.80, P= 0.05). Length of stay was significantly higher in Bryant's traction group (26 vs 19 d, P< 0.001). Rates of pressure ulcers were higher in the Bryant traction group (26.7% vs 0%, P= 0.005). Rates of skin rash/abrasions, urethrocutaneous fistula, and bladder/fascial dehiscence did not differ. Conclusions: Spica cast immobilization is a safe and effective immobilization method. Compared with Bryant traction, spica cast immobilization was associated with a greater reduction in postoperative pubic diastasis both short and long-term, along with a shorter length of hospitalization and reduced rate of pressure ulcers.

Boston Children’s Hospital has established a genomic sequencing and analysis research initiative to improve clinical care for pediatric rare disease patients. Through the Children’s Rare Disease Collaborative (CRDC), the hospital offers CLIA-grade exome and genome sequencing, along with other sequencing types, to patients enrolled in specialized rare disease research studies. The data, consented for broad research use, are harmonized and analyzed with CRDC-supported variant interpretation tools. Since its launch, 66 investigators representing 26 divisions and 45 phenotype-based cohorts have joined the CRDC. These studies enrolled 4653 families, with 35% of analyzed cases having a finding either confirmed or under further investigation. This accessible and harmonized genomics platform also supports additional institutional data collections, research and clinical, and now encompasses 13,800+ patients and their families. This has fostered new research projects and collaborations, increased genetic diagnoses and accelerated innovative research via integration of genomics research with clinical care.

Introduction Epispadias, which occurs on the more mild end of the Bladder Exstrophy Epispadias Complex (BEEC) spectrum, presents still with a wide range of severity in boys, from mild glanular epispadias to penopubic epispadias with severe urethral and bladder neck defects. Surgical management ranges from isolated epispadias repair to epispadias repair with bladder neck reconstruction (BNR) with or without pelvic osteotomies. Objectives We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration. In addition, we sought to delineate outcomes based on anatomic severity at time of diagnosis, and initial procedure performed in cases of penopubic epispadias. Methods IRB approved databases were retrospectively queried at three institutions for patients who underwent repair of epispadias between 1/1993 and 1/ 2013. Degree of epispadias, age and technique at initial repair, and self -reported continence status at last follow-up were recorded. Continence was categorized as: wet, intermediate (dry 2-3 h), or dry, while also distinguishing those who void and those who require clean intermittent catheterization (CIC). Those not seen since 1/1/2015, younger than 10 years at last follow up, or in whom continence data were not recorded were excluded. Results A total of 48 boys were identified; 36 met inclusion criteria. The epispadias cohort consisted of 8 glanular epispadias (GE) (22%); 8 penile epispadias (PE) (22%), and 20 penopubic epispadias (PPE) (56%) with a median follow-up of 11.3 years (3.2-26.2 years). Overall, 33 of 36 (92%) boys void per urethra. Within the group that voids, 19/33 (58%) are completely dry, while 6/33 (18%) are wet. Among patients who underwent initial epispadias repair without concurrent or subsequent bladder neck reconstruction, continence rates were: GE 63% (5/8); PE 75% (6/8); PPE 71% (5/7). Among the 9 boys with PPE who underwent initial epispadias repair with concurrent BNR, 22% (2/9) were dry with no further surgeries. Overall, 8/20 (40%) of boys with PPE void with complete dryness. Discussion This multi -center retrospective review of continence in epispadias demonstrates that even some boys with glanular and penile epispadias can have challenges with continence, and boys with penopubic epispadias may remain wet despite careful preoperative assessment of bladder neck functionality and concurrent BNR. Conclusion Continence outcomes in boys with all degrees of epispadias can be variable. Even boys with more distal defects may have significant bladder neck deficiency. And those with the most severe form of epispadias may require bladder neck reconstruction to achieve continence.

Lower urinary tract dysfunction (LUTD) can manifest as a spectrum of voiding symptoms in childhood, including urinary urgency, frequency, hesitancy, and incontinence. In severe cases, it can lead to frequent urinary tract infections, hydronephrosis, kidney scarring, and chronic kidney disease. Non-neurogenic neurogenic bladder (NNNB) is a diagnosis of exclusion in which children develop discoordination between the detrusor smooth muscle and external urethral sphincter in the absence of neurological or obstructive lesions, resulting in severe LUTD. Historically, such disorders of voiding were thought to result from behavioral maladaptation. However, it is now increasingly recognized that some individuals may have an underlying genetic etiology for their symptoms. Here, we performed exome sequencing for five probands with NNNB or other forms of severe LUTD, and we identified two individuals with monogenic etiologies for their symptoms. One individual had a homozygous exon 9 deletion in HPSE2 and another had a homozygous single amino acid deletion (p.Gly167del) in ARL6. We performed PCR experiments to identify the breakpoints of the HPSE2 exon 9 deletion and implicate microhomology-mediated end joining as a potential mechanism by which the deletion arose. These findings suggest that genetic testing should be considered for children with severe LUTD.

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. Prevalence was tabulated using a multi-institutional retrospective review. RESULTS Mean rise in RPP was + 26.0 mmHg in the PEK and + 10.3 mmHg in the orthotopic kidney (P = .55). One patient had immediate increase in RPP of 66 mmHg in the PEK (7 mmHg in the orthotopic kidney) which decreased to + 17 mmHg in the PEK upon release of the pubic approximation stitch. Mean change in PSV was + 67.7 cm/s in the PEK compared to - 25.7 cm/s in the orthotopic kidney (P = .09). Mean change in renal RI was + 0.06 in PEK compared to - 0.01 in the orthotopic kidney (P = .29). Among 80 OEIS patients, 24 (30%) had a PEK. 3 (4%) had a solitary PEK. CONCLUSION During second-stage OEIS closure, we witnessed patterns of higher change in RPP, PSV, and RI of the PEK compared to the orthotopic kidney. Furthermore, PEKs were found in nearly a third of our OEIS patients. Real-time RPP monitoring, especially those with solitary PEK, may aid in the management of OEIS patients during the perioperative period.

A noninvasive test that can longitudinally assess renal parenchymal status would be incredibly valuable for a wide range of conditions, including neurogenic bladder, renal transplantation, and upper and lower urinary tract anomalies. To address this need, enormous amounts of time, effort, and resources have been invested to identify biologic molecules that signal the pathologic processes of renal parenchymal defects. In this comprehensive narrative review, the authors summarize biomarkers that have previously been investigated while highlighting the key pitfalls and barriers that have impeded biomarker discovery and translation.

Background:Due to racial, cultural, and linguistic marginalization, some populations experience disproportionate barriers to genetic testing in both clinical and research settings. It is difficult to track such disparities due to non-inclusive self-reported race and ethnicity categories within the electronic health record (EHR). Inclusion and access for all populations is critical to achieve health equity and to capture the full spectrum of rare genetic disease.Objective:We aimed to create revised race and ethnicity categories. Additionally, we identified racial and ethnic under-representation amongst three cohorts: (1) the general Boston Children's Hospital patient population (general BCH), (2) the BCH patient population that underwent clinical genomic testing (clinical sequencing), and (3) Children's Rare Disease Cohort (CRDC) research initiative participants.Design and Methods:Race and ethnicity data were collected from the EHRs of the general BCH, clinical sequencing, and CRDC cohorts. We constructed a single comprehensive set of race and ethnicity categories. EHR-based race and ethnicity variables were mapped within each cohort to the revised categories. Then, the numbers of patients within each revised race and ethnicity category were compared across cohorts.Results:There was a significantly lower percentage of Black or African American/African, non-Hispanic/non-Latine individuals in the CRDC cohort compared with the general BCH cohort, but there was no statistically significant difference between the CRDC and the clinical sequencing cohorts. There was a significantly lower percentage of multi-racial, Hispanic/Latine individuals in the CRDC cohort than the clinical sequencing cohort. White, non-Hispanic/non-Latine individuals were over-represented in the CRDC compared to the two other groups.Conclusion:We highlight underrepresentation of certain racial and ethnic populations in sequencing cohorts compared to the general hospital population. We propose a range of measures to address these disparities, to strive for equitable future precision medicine-based clinical care and for the benefit of the whole rare disease community.

Purpose: ERAS (enhanced recovery after surgery) protocols are designed to optimize perioperative care and expedite recovery. Historically, complete primary repair of bladder exstrophy has included postoperative recovery in the intensive care unit and extended length of stay. We hypothesized that instituting ERAS principles would benefit children undergoing complete primary repair of bladder exstrophy, decreasing length of stay. We describe implementation of a complete primary repair of bladder exstrophy-ERAS pathway at a single, free-standing children's hospital. Materials and Methods: A multidisciplinary team developed an ERAS pathway for complete primary repair of bladder exstrophy, which launched in June 2020 and included a new surgical approach that divided the lengthy procedure into 2 consecutive operative days. The complete primary repair of bladder exstrophy-ERAS pathway was continuously refined, and the final pathway went into effect in May 2021. Post-ERAS patient outcomes were compared with a pre-ERAS historical cohort (2013-2020). Results: A total of 30 historical and 10 post-ERAS patients were included. All post-ERAS patients had immediate extubation (P =.04) and 90% received early feeding (P <.001). The median intensive care unit and overall length of stay decreased from 2.5 to 1 days (P =.005) and from 14.5 to 7.5 days (P <.001), respectively. After final pathway implementation, there was no intensive care unit use (n=4). Postoperatively, no ERAS patient required escalation of care, and there was no difference in emergency department visits or readmissions. Conclusions: Applying ERAS principles to complete primary repair of bladder exstrophy was associated with decreased variations in care, improved patient outcomes, and effective resource utilization. Although ERAS has typically been utilized for high-volume procedures, our study highlights that an enhanced recovery pathway is both feasible and adaptable to less common urological surgeries.

Exstrophy-epispadias complex encompasses a spectrum of disorders with lower abdominal midline malformations, including epispadias, bladder exstrophy, and cloacal exstrophy, also known as Omphalocele-Exstrophy-Imperforate Anus-Spinal Anomalies Complex. In this review, the authors discuss the epidemiology, embryologic cause, prenatal findings, phenotypic characteristics, and management strategies of these 3 conditions. The primary focus is to summarize outcomes pertaining to each condition.

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 currentmedical 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 2019.15 patients underwent cephalotrigonal BUR-CPRE and 49 patients underwent CPRE alone. Our primary outcome was >1 episode of pyelonephritis as documented in the medical record. Secondary outcomes were persistent vesicoureteral reflux (VUR), with a sub-analysis of number of refluxing renal units and presence of dilating VUR, and the need for subsequent ureteral surgery. Descriptive statistics in addition to standard, two tailed univariate statistics, were used to compare the groups where appropriate. Results BUR-CPREwas associatedwith a significant decrease in the rates of post-operative VUR, number of refluxing renal units, and need for subsequent ureteral surgery (p=0.002, p=0.001, and p=0.048 respectively). There was a reduction in the rates of recurrent pyelonephritis and dilating reflux in patients undergoing BUR-CPRE, though it did not reach significance. Female gender was significantly associated with recurrent pyelonephritis regardless of BUR-CPRE status (p=0.005). There were no reports of distal ureteral obstruction or other complications following BURCPRE. Themean post-operative follow up for the BURCPRE groupwas 46.33 (10.26) months vs. 53.76 (26.05) months for CPRE (p=0.11). Discussion Recurrent pyelonephritis following bladder closure in patients with BE is a well-documented surgical complication, with centers performing CPRE reporting rates of post-operative pyelonephritis from 22 to 50%. Our series demonstrates similar efficacy of BUR-CPRE compared to other contemporary series and provides additional detail about need for subsequent ureteral surgeries and increased long term follow-up of these complex patients. Limitations of the study include male predominance of the cohort and lack of randomization of BUR-CPRE. Conclusions BUR-CPRE decreases postoperative VUR and the need for additional ureteral surgery in select BE patients; it should be considered when technically feasible. While results continue to suggest a trend toward decreased recurrent pyelonephritis and dilating reflux, further longitudinal follow-up in our cohort will be needed.

Background Assessment of the ureter is a fundamental part of the radiologic evaluation of the urinary tract. Abnormal ureteral dilation warrants further investigation to assess the etiology, which includes obstruction and/or reflux. Despite this fundamental need, there are no established normative values in children based on imaging. Objective To provide normative values for ureteral diameter in pediatric patients with age-related ranges. Materials and methods We retrospectively reviewed all magnetic resonance (MR) urography studies and chose only normal ureters for assessment. The images were analyzed on commercially available software to assess maximum internal diameter. Manual measurements were done in cases where the images were below the resolution for automated assessment. Maximum intraluminal ureteral diameters were measured in upper, mid and lower thirds and the average of the three maximum ureteral diameters was used to obtain the average widest internal ureteral diameter. Multivariable linear regression was performed to test the association between the calculated diameter and gender. Differences in sizes between the left and right ureter were assessed using paired Wilcoxon signed rank test. Results One hundred twenty-one MR urography studies were selected, which included 160 ureter units. The diameter increases progressively with age, ranging from 3.2 mm during infancy to 5.0 mm in patients older than 16 years of age. After 9 years of age, the average widest internal ureteral diameter is slightly larger in males compared to females (odds ratio [OR]=1.91, 95% confidence interval [CI] [1.63, 2.25], P<0.0001). The right ureter was slightly larger than the left (3.9 mm vs. 3.7 mm, P=0.004) among 39 patients in whom both right and left ureter units were included. The average mid ureteral diameter is widest, followed by the distal third then proximal third. Conclusion We present the normative values for the average widest internal ureteral diameter based on laterality and different segments. In the pediatric population, 3.8 mm should be considered the average widest internal ureteral diameter.

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. We retrospectively determined prenatal or postnatal diagnosis. Those with unknown prenatal history were excluded. Multivariable logistic regression was used to investigate temporal pattern in rate of prenatal diagnosis while adjusting for sex and treating institution. RESULTS Among 197 BE and 52 OEIS patients, 155 BE and 45 OEIS patients had known prenatal history. Overall prenatal diagnosis rates of BE and OEIS were 47.1% (73/155) and 82.2% (37/45), respectively. Prenatal diagnosis rate was significantly lower in BE compared to OEIS (P <.0001). The prenatal diagnosis rate for BE significantly increased over time (OR 1.10; [95%CI: 1.03-1.17]; P =.003). Between 2000 and 2005, the prenatal diagnosis rate of BE was 30.3% (10/33). Between 2015 and 2020, prenatal diagnosis rate of BE was 61.1% (33/54). Prenatal diagnosis rate for OEIS did not change over time. Rates of prenatal diagnosis did not differ by sex or treating institution. CONCLUSION Rates of prenatal diagnosis of BE and OEIS are higher than previously reported. Prenatal diagnosis rate of BE doubled in the last 5 years compared to the first 5 years of the study period. Nonetheless, a significant proportion of both BE and OEIS patients remain undiagnosed prior to delivery. (c) 2022 Elsevier Inc.

You have accessJournal of UrologyCME1 May 2022MP08-19 FACTORS AFFECTING ORCHIOPEXY GUIDELINE ADHERENCE Andrea Balthazar, Ted Lee, Tanya Logvinenko, Caleb Nelson, Hsin-Hsiao Scott Wang, and Caleb Nelson Andrea BalthazarAndrea Balthazar More articles by this author , Ted LeeTed Lee More articles by this author , Tanya LogvinenkoTanya Logvinenko More articles by this author , Caleb NelsonCaleb Nelson More articles by this author , Hsin-Hsiao Scott WangHsin-Hsiao Scott Wang More articles by this author , and Caleb NelsonCaleb Nelson More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002530.19AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Cryptorchidism affects 3%-5% of term males. AUA guidelines for evaluating and treating cryptorchidism recommend surgical repair before 18 months of age (MoA) in boys with unilateral or bilateral cryptorchidism. Our objective was to understand the factors associated with cryptorchidism guideline adherence. METHODS: Pediatric Health Information System (PHIS) is a database of freestanding children’s hospitals. Using PHIS, we performed a multicenter retrospective study of boys ages 18 years and younger undergoing orchiopexy between 1/1/2009-12/31/2019. Categorized subjects by race/ethnicity(R/E) (Hispanic defined as any subject who identifies as Hispanic regardless of race), insurance type, income quintile, and presence of comorbid conditions. PHIS hospitals without outpatient surgery information were excluded. We fitted multivariate mixed-effects logistic regression models to evaluate the association of sociodemographic factors with the odds of undergoing surgery after 18 MoA (hospital-level as random effect). Multiple imputations were used for missing observations. Sensitivity analysis (SA) was performed for 18 and 24 MoA. RESULTS: 89114 cryptorchid males had orchiopexy between 2009-2019; 59093 (66.3%) surgeries occurred ≥18 MoA. 45303 (50.8%) were White, and 42213 (47.4%) had public insurance. Increased odds of orchiopexy after 18 MoA correlates with Black or Hispanic (P < 0.001), self-pay/charity (P=0.007), higher-income quintile (P <0.001), and lower comorbidity level (P <0.001). Black (OR=1.22; 95% CI, 1.16-1.28; P <0.001) and Hispanic (OR=1.19; 95% CI, 1.14-1.24; P <0.001) were at increased odds of delayed orchiopexy compared to Whites. We did not attribute the associations of R/E, insurance, or income with the outcome to single hospitals. The highest income compared to lowest was associated with increased odds of surgery after 18 MoA at 29/34 hospitals (85%). Compared to private insurance, public correlates with increased odds of surgical delay at 13/34 hospitals (38%). SA with 24 MoA with similar results. CONCLUSIONS: Consistent with prior studies, orchiopexy remains delayed despite guidelines. In addition, Black, Hispanic, self-pay/charity, and lower comorbidity were significantly associated with delay. This highlights a need to refine provider education & outreach efforts to reduce disparities in care. Source of Funding: 5T32DK060442 © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e133 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Andrea Balthazar More articles by this author Ted Lee More articles by this author Tanya Logvinenko More articles by this author Caleb Nelson More articles by this author Hsin-Hsiao Scott Wang More articles by this author Caleb Nelson More articles by this author Expand All Advertisement PDF downloadLoading ...

You have accessJournal of UrologyCME1 May 2022MP11-12 PRENATAL DIAGNOSIS OF BLADDER AND CLOACAL EXSTROPHY OVER 20 YEARS Ted Lee, Dana Weiss, Elizabeth Roth, Sahar Eftekharzadeh, Saafia Masoom, Susan Jarosz, John Kryger, Aseem Shukla, Travis Groth, Michael Mitchell, Richard Lee, Douglas Canning, and Joseph Borer Ted LeeTed Lee More articles by this author , Dana WeissDana Weiss More articles by this author , Elizabeth RothElizabeth Roth More articles by this author , Sahar EftekharzadehSahar Eftekharzadeh More articles by this author , Saafia MasoomSaafia Masoom More articles by this author , Susan JaroszSusan Jarosz More articles by this author , John KrygerJohn Kryger More articles by this author , Aseem ShuklaAseem Shukla More articles by this author , Travis GrothTravis Groth More articles by this author , Michael MitchellMichael Mitchell More articles by this author , Richard LeeRichard Lee More articles by this author , Douglas CanningDouglas Canning More articles by this author , and Joseph BorerJoseph Borer More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002533.12AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Bladder exstrophy (BE) and cloacal exstrophy (CE) presents significant challenges during the newborn period. In contrast to the unexpected diagnosis in the newborn, prenatal diagnosis of BE and CE affords valuable opportunities for education and preparation for caregivers and healthcare providers. Based on small sample populations, previously reported prenatal diagnosis rates of BE and CE were 13-25% and 17%, respectively. In this study, we aimed to examine the prenatal diagnosis rates of BE and CE in a large cohort of patients over a 20-year period. We hypothesized that prenatal diagnosis rates improved over time in both BE and CE due to evolving techniques and technologies in prenatal ultrasonography. METHODS: We queried a multi-institutional exstrophy-epispadias complex database of three centers to identify patients with BE or CE who underwent primary closure between 2000 and 2020. Retrospective review was performed to assess whether BE or CE diagnosis was made prenatally versus postnatally. Those with unknown prenatal history were excluded from analysis. Univariate analysis of categorical data was performed using Pearson’s Chi-square test. Multivariable logistic regression was used to investigate temporal pattern in the rate of prenatal diagnosis while adjusting for sex and institution. RESULTS: Among 197 BE and 52 CE patients that were identified, 155 BE and 45 CE patients had known prenatal history. The overall prenatal diagnosis rates of BE and CE were 47.1% (73/155) and 82.2% (37/45), respectively. Prenatal diagnosis rate was significantly lower in BE compared to CE (p<0.0001) across the 20-year period. The prenatal diagnosis rate for BE significantly increased over time (OR 1.10; [95% CI: 1.03, 1.17]; p = 0.003). Between 2000 and 2005, the prenatal diagnosis rate of BE was 30.3% (10/33). Between 2015 and 2020, the prenatal diagnosis rate of BE was 61.1% (33/54). The prenatal diagnosis rate for CE did not change over time. Rates of prenatal diagnosis did not differ by sex or institution in both BE and CE. CONCLUSIONS: The rates of prenatal diagnosis of BE and CE are higher than previously reported. Prenatal diagnosis rate of BE doubled in the last 5 years compared to the first 5 years of the study period. Nonetheless, a significant proportion of both BE and CE patients remain undiagnosed prior to delivery. Source of Funding: None © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e161 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Ted Lee More articles by this author Dana Weiss More articles by this author Elizabeth Roth More articles by this author Sahar Eftekharzadeh More articles by this author Saafia Masoom More articles by this author Susan Jarosz More articles by this author John Kryger More articles by this author Aseem Shukla More articles by this author Travis Groth More articles by this author Michael Mitchell More articles by this author Richard Lee More articles by this author Douglas Canning More articles by this author Joseph Borer More articles by this author Expand All Advertisement PDF downloadLoading ...

Background Acute pain after surgery is one of the most frequent indications for opioid prescribing in children. Opioids are often not stored or disposed of safely after their use, placing children and others in the home at risk for accidental ingestion or intentional misuse. We currently lack evidence-based guidelines for post-operative pain management after common ambula-tory pediatric urologic procedures. Thus, each sur-geon must decide if and how much opioid to prescribe based on his/her own assumptions of perceived post-operative pain. Objectives As part of an effort to establish opioid prescribing guidelines across two academic centers, the objec-tives of this study were to evaluate current vari-ability in pediatric urologists' opioid prescribing factors and identify patients at greatest risk of being prescribed high doses of opioids after common ambulatory pediatric urologic procedures. Methods We retrospectively evaluated post-operative opioid prescribing patterns after common ambulatory pe-diatric urology procedures (circumcision, orchi-opexy, and hernia/hydrocele) at two major childre's hospitals. Specifically, we evaluated if and how much opioid was prescribed for all children (18 years or younger) between 2016 and 2017. Bivariate analysis was performed using Kruskal-Wallis Test and Wilcoxon Rank Sum. Multi-variable logistic regression was performed to determine patient, surgeon, and procedural factors that predicted the prescription of a high dose of opioids (greater than the median number of doses prescribed for that procedure). Results Over the two-year period, 811 circumcisions and 883 inguinal surgeries (inguinal orchiopexy and hernia/ hydrocele) were performed. 94% of patients under -going circumcision and 97% of those undergoing inguinal surgery were prescribed opioid analgesia. The median number of doses prescribed for circumcision was 20; for inguinal surgeries, 23.75% of patients received 15 opioid doses or more. Pa-tients ages 0-2 years, who represented the largest age group (41% of all patients), received significantly more opioid doses than all other age groups, fol-lowed by those >10 years (p < 0.01). There was significant variation in opioid prescribing patterns by provider (p < 0.01) (Figure 1) On multivariable lo-gistic regression, younger age, pill form, and earlier year were all associated with a greater number of opioid doses prescribed for all surgeries. Conclusions Across two institutions without a formal post-operative opioid prescribing policy for ambulatory pediatric urologic procedures, we observed consid-erable variability in provider prescribing patterns, with nearly all patients receiving an opioid, and those 0-2 years receiving the highest number of doses. This highlights the need for evidence-based guidelines for post-operative pain management after ambulatory pediatric urologic surgeries.