Search

The opioid epidemic response led to increased use of postoperative, non-opioid analgesia. Some pediatric urologists do not routinely use non-steroidal anti-inflammatory drugs (NSAIDs) for fear of causing acute kidney injury (AKI). While previous studies have demonstrated the safety and efficacy of NSAIDs in children, safety after lower urinary tract reconstruction has not been well characterized. ptUsing the Kidney Disease: Improving Global Outcomes (KDIGO) criteria for AKI (increase in creatinine ≥0.3 mg/dL or increase in creatinine ≥1.5x baseline or urine output <0.5 mL/kg/hr for 6 h), we hypothesized there would be a difference in the incidence of postoperative AKI between patients who did and did not receive NSAIDs following surgery. Patients 2–18 years old who underwent lower urinary tract reconstruction (i.e., bladder augmentation and/or creation of a catheterizable channel) from 2009 to 2021 and had documented urine output were retrospectively reviewed. Chronic kidney disease (CKD) stage was calculated from creatinine and cystatin C within 6 months of surgery using the CKiD U25 equations. Patients who received NSAIDs were propensity matched on 11 characteristics with patients undergoing similar surgeries who did not receive NSAIDs. The primary outcome was incidence of AKI within 48 h of surgery. The unmatched cohorts included 243 patients. Propensity matching identified 166 patients in the NSAID arm and 41 in the no NSAID arm. 26 patients with CKD stage 2–3 were included. There was no significant difference in the incidence of postoperative AKI based on any KDIGO criteria (17.1% no NSAID versus 16.3% NSAID, p = 0.87). Median postoperative opioids fell from 0.88 mg/kg in the no NSAID arm to 0.37 mg/kg morphine equivalents in the NSAID arm, although this was not statistically significant. Log-rank testing by Kaplan–Meier analysis demonstrated no difference in time to incidence of low urine output between the groups (p = 0.32). In the whole population not stratified by NSAID use, no differences were seen in AKI between those with and without CKD (16.7% with versus 17.9% without CKD). There was no difference in the incidence of postoperative AKI among patients who did and did not receive NSAIDs after lower urinary tract reconstruction, excluding those with advanced CKD. These results support that postoperative NSAIDs were an unlikely source of AKI. However, AKI remained a risk following these surgeries, regardless of NSAID use, likely owing to underlying disease, longer operations, and fluid shifts. Summary Table Incidence of postoperative AKI among patients who did and did not receive NSAIDs in the operating room or within 6 h after surgery. Summary table n No NSAID NSAID P value 41 patients 166 patients Acute kidney injury (any criteria) 207 7 (17.1%) 27 (16.3%) 0.875 Acute kidney injury (criteria 1) 38 3 (23.1%) 3 (12.0%) 0.895 Acute kidney injury (criteria 2) 41 4 (28.6%) 10 (37.0%) 0.380 Acute kidney injury (criteria 3) 207 2 (4.9%) 17 (10.2%) 0.618 Median length of stay (IQR), days 207 5.3 (4.2–7.1) 4.5 (3.4–6.3) 0.970 [ABSTRACT FROM AUTHOR]

Ureteral stents are commonly used during pyeloplasty to ensure drainage and anastomotic healing. Antibiotic prophylaxis is often used due to concerns for urinary tract infection (UTI). Although many surgeons prescribe prophylactic antibiotics following pyeloplasty, practices vary widely due to lack of clear evidence-based guidelines. We hypothesize that the rate of stent UTI does not significantly vary between children who receive antibiotics and those who do not. We reviewed the medical records of 741 patients undergoing pyeloplasty between January 2010 and July 2018 across seven institutions. Exclusion criteria were: age older than 22 years, no stent placed, externalized stents used, and incomplete records. Surgical approach, age, antibiotic use, stent duration, Foley duration, and urine culture results were recorded. Patients were categorized into two groups, those younger than four years of age and those four years and older as proxy for likely diaper use. Univariate logistic regression was conducted to identify variables associated with UTI. Multivariable backward stepwise logistic regression was used to identify the best model with Akaike information criterion as model selection criteria. The selected model was used to calculate odds ratios and 95% confidence intervals summarizing the association between prophylactic antibiotics and stent UTI while controlling for age, gender, and intra-operative urine cultures. 672 patients were included; 338 received antibiotic prophylaxis and 334 did not. These groups differed in mean age (3.91 vs. 6.91 years, P <.001), mean stent duration (38.5 vs. 35.32 days, P <.001), and surgical approach (53.25% vs. 32.04% open vs. laparoscopic, P <.001). The incidence of stent UTI was low overall (7.59%) and similar in both groups: 31/338 (9.17%) in the prophylaxis group and 20/334 (5.99%) in the non-prophylaxis group (P =.119). Although female gender, likely diaper use, and positive intra-operative urine culture were each associated with significantly higher odds of stent UTI, prophylactic antibiotic use was not associated with significant reduction in stent UTI in any of these groups. Surgical approach, stent duration, and Foley duration were not associated with stent UTI. Incidence of stent UTI is low overall following pyeloplasty. Prophylactic antibiotics are not associated with lower rates of stent UTI following pyeloplasty even after controlling for risk factors of female gender, likely diaper use, and positive intra-operative urine culture. Routine administration of prophylactic antibiotics after pyeloplasty does not appear to be beneficial, and may be best reserved for those with multiple risk factors for UTI. [Display omitted] [ABSTRACT FROM AUTHOR]

The purpose of this study was to understand pediatric urologists' perceived role of patient characteristics on discussions about treatment of infants with suspected UPJ obstruction. We conducted semi-structured interviews with pediatric urologists from three geographically diverse sites. Interview domains included: clinical indications for surgery, discussions with parents, and consideration of parent socioeconomic factors. Transcribed data and field notes were analyzed using a team-based, inductive grounded theory approach. Thirteen physicians were interviewed. Physicians reported a standardized approach to discussions to facilitate parental understanding. While they did not report overt consideration of demographics, they tailored discussions based on educational and cultural background and language barriers. Physicians also reported that concerns about risk of loss to follow up contributed to their treatment recommendations. Most physicians recognized that the lack of clear data often led to use of personal experience to guide recommendations. Physicians recognize a gap in data to guide surgical decisions and utilize personal experience to augment this gap. They also recognize the influence of educational and language barriers on discussions with families and consider risk of loss to follow up when making recommendations, suggesting an implicit consideration of demographics. These findings suggest that development of evidence-based guidelines may reduce treatment variations. Not applicable (qualitative research study written in compliance with COREQ guidelines). [ABSTRACT FROM AUTHOR]

Summary Background The electronic health record (EHR) was designed as a clinical and administrative tool to improve clinical patient care. Electronic healthcare systems have been successfully adopted across the world through use of government mandates and incentives. Methods Using electronic health record, health information system, electronic medical record, health information systems, research, outcomes, pediatric, surgery, and urology as initial search terms, the literature focusing on clinical documentation data capture and the EHR as a potential resource for research related to clinical outcomes, quality improvement, and comparative effectiveness was reviewed. Relevant articles were supplemented by secondary review of article references as well as seminal articles in the field as identified by the senior author. Findings US federal funding agencies, including the Agency for Healthcare Research and Quality, the Patient-Centered Outcomes Research Institute, the National Institutes of Health, and the Food and Drug Administration have recognized the EHR's role supporting research. The main approached to using EHR data include enhanced lists, direct data extraction, structured data entry, and unstructured data entry. The EHR's potential to facilitate research, overcoming cost and time burdens associated with traditional data collection, has not resulted in widespread use of EHR-based research tools. Conclusion There are strengths and weaknesses for all existing methodologies of using EHR data to support research. Collaboration is needed to identify the method that best suits the institution for incorporation of research-oriented data collection into routine pediatric urologic clinical practice. [ABSTRACT FROM AUTHOR]

Initial management of pediatric patients with neurogenic bladder is focused on clean intermittent catheterization and medical therapies. Those with more hostile or small capacity bladders require surgical intervention including bladder augmentation that can result in significant clinical sequelae. This study examines a rarely described approach wherein the bladder reconstruction is extraperitonealized by bringing bowel segments through a peritoneal window and then closed. The aim of this study was to determine if the rate of bladder rupture and subsequent morbidity differed between patients who have undergone an intraperitoneal versus extraperitoneal bladder augmentation. We hypothesized that an extraperitoneal approach reduced the risk of intraperitoneal bladder perforation, downstream Intensive Care Unit (ICU) admission, small bowel obstruction (SBO) requiring exploratory laparotomy, and ventriculoperitoneal (VP) shunt-related difficulties as compared to the standard intraperitoneal technique. A retrospective chart review was conducted to assess surgical approach and outcomes in patients who underwent bladder augmentation performed between January 2009 and June 2021. Patients were identified through an existing database and manual chart review was conducted to extract data through imaging studies, operative notes, and clinical documentation. The primary outcome was bladder perforation. Secondary outcomes were ICU admission, exploratory laparotomy, and VP shunt externalization, infection, or revision for any cause. Nonparametric statistical analyses were performed. A total of 111 patients underwent bladder augmentation with 37 intraperitoneal and 74 extraperitoneal procedures. Median follow up was 5.8 years [IQR 3.0–8.6 years] and did not vary between groups (P = 0.67). Only one patient was found to have a bladder perforation in the intraperitoneal group (log-rank P = 0.154). There were no significant differences in time to post-augmentation ICU admission, exploratory laparotomy, or VP shunt events between the two groups (log-rank P = 0.294, log-rank P = 0.832, and log-rank P = 0.237, respectively). Furthermore, a Kaplan–Meier analysis assessing time to composite complication demonstrated no significant difference between the two techniques (log-rank P = 0.236). This study provides important data comparing the rate of bladder perforation and subsequent morbidity between intraperitoneal and extraperitoneal bladder augmentation. As expected, with a complex procedure, both groups suffered complications, but these data showed no difference between the two procedures. Rates of prior (abdominal) surgery may influence the decision to perform this procedure extraperitoneal. Outcomes related to bladder perforation and secondary consequences do not differ significantly between patients who had bladder augmentation performed with an intraperitoneal versus extraperitoneal approach. Given the low number of adverse events in this study, larger studies are warranted. [Display omitted] [ABSTRACT FROM AUTHOR]

Although multi-center research is needed in pediatric urology, collaboration is impeded by differences in physician documentation and research resources. Electronic health record (EHR) tools offer a promising avenue to overcome these barriers. To assess the accuracy, completeness, and utilization of structured data elements across multiple practices. A standardized template was developed and implemented at five academic pediatric urology practices to document clinic visits for patients with congenital hydronephrosis and/or vesicoureteral reflux. Data from standardized elements in the template and from pre-existing EHR fields were extracted into a secure database. A 20% random sample of infants with data from structured elements from 1/1/2020 and 4/30/2021 were identified and compared to manual chart review at sites with >100 charts; all other sites reviewed at least 20 charts. Manual chart review was standardized across sites and included: clinic and operative notes, orders linked to the clinic encounter, radiology results, and active medications. Accuracy of data extraction was evaluated by computing the kappa statistic and percentage agreement. For sites that had adopted the templates prior to 6/1/2019 (early adopters), a list of eligible patients with an initial clinic visit from 1/1/2020–7/27/2020 was generated using standardized reporting techniques and confirmed by manual chart review. Physician utilization of the template was then calculated by comparing patients with data obtained from the note template to the generated list of eligible patients. 230 patient records met study criteria. Agreement between manual chart review and data extracted from the EHR was high (>85%). Race, ethnicity and insurance data were misclassified in about 10–15% of cases; this was due to site-specific differences in how these fields were coded. Renal ultrasound was misclassified 12% of the time; this was primarily due to outside images documented in radiology results but not included in the clinical note. All other data elements had >90% agreement (Figure). Template utilization for early adopters was >75% (75.5–87.5%). This is the first study in urology to demonstrate that use of structured data elements can support multi-center research. Limitations include: inclusion of only academic sites with the Epic EHR and lack of data on utilization and sustainability at sites without a prior history of structured template use. Multi-center research collaboration using EHR-based data collection tools is feasible with generally high accuracy compared to manual chart review. Additionally, sites with a long history of template adoption have high levels of provider utilization. [Display omitted] [ABSTRACT FROM AUTHOR]

Objective: To identify geographic variability in the imaging of infants with congenital hydronephrosis at initial pediatric urologic evaluation. Methods: We performed a retrospective review of infants aged ≤12 months with congenital hydronephrosis seen as new patients from October 2010 to September 2011 at 3 regionally diverse pediatric urology practices: University of Virginia Hospital, Rady Children's Hospital, and Children's Hospital Colorado. Primary outcomes measured were the type and number of tests ordered at initial evaluation. Independent variables collected included the following: patient age, location, and initial ultrasound findings. Ultrasound findings were manually extracted from the attending pediatric urologist's clinic note. All other data were automatically extracted from the electronic medical record. Proportions were analyzed using Pearson's goodness of fit and Fisher exact tests. Medians were compared using the Kruskal-Wallis test. Results: Two hundred forty-one patients met the study criteria. Median patient age was 2 months and did not differ across sites. Most patients (64.7%) had Society for Fetal Urology grade 0-2 hydronephrosis; prevalence of high-grade hydronephrosis varied across sites (P = .002). Use of voiding cystourethrography also varied across sites (17.6%-88.9%); this difference persisted when controlling for age and hydronephrosis grade (P <.05). Use of other imaging studies did not significantly differ across sites. Conclusion: Use of screening voiding cystourethrography for infants with congenital hydronephrosis varies across practices. This variation persists when controlling for differences in age and ultrasound findings, suggesting that regional differences in patient demographics, provider/parental preferences, or referral patterns might contribute to practice variations in the evaluation of these patients. [Copyright &y& Elsevier]

Management of the neurogenic bladder is variable, complex, and often requires a demanding bladder care regimen which may present caregiver burdens that are unique among chronic disease. While research into patient quality of life is increasing, parallel study of the caregiver experience is scant. Existing research primarily comprises survey data using validated instruments originally developed for non-urologic conditions, such as dementia. These surveys may detect high caregiver burden and decreased quality of life amongst caregivers but are limited in their ability to understand the underlying causes. To characterize the experience of those caring for children with neurogenic bladders, with a focus on unexpected burdens and challenges. In light of limited existing research, a qualitative research methodology was selected to explore the caregiver experience. Semi-structured phone interviews were conducted with primary caregivers of children with neurogenic bladder, all of whom were patients in the pediatric urology department of a single tertiary pediatric referral center. Purposive sampling was used to ensure diverse representation. Interviews were recorded, transcribed, and professionally translated if needed. Transcripts were analyzed using a team-based inductive grounded-theory approach, facilitated by ATLAS. ti software. Member-checking focus groups were held to validate the results. Twenty-five caregivers were interviewed (20 in English, 5 in Spanish), at which point thematic saturation was reached. Three primary themes emerged surrounding the topic of unexpected challenges: 1. High caregiver burden, 2. Challenges with catheterization and supplies, 3. Urinary tract infections. Member-checking focus groups validated the thematic analysis and provided additional insights into mitigating factors for these challenges. A child's independence with his or her health care regimen was cited as particularly important for decreasing caregiver burden. Caregivers of children with neurogenic bladder report their role is more difficult than they anticipated it would be. Catheterization represents a particularly burdensome task, and recurrent infections are an unexpected and persistent medical challenge. Understanding unexpected challenges that caregivers face will help pediatric urologists target modifiable factors to decrease caregiver burden, address current gaps in counseling and expectation-setting, and set the stage for more complete shared decision-making. This study represents an initial qualitative characterization of the experience caring for a child with neurogenic bladder. This is a key first step in understanding how caregivers make decisions for their children and their families. This initial study is foundational to a larger project to create a decision aid for caregivers of children with neurogenic bladder. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: To evaluate management patterns of lower urinary tract (LUT) dysfunction and establish a treatment algorithm to guide pediatric healthcare providers. Methods: 390 children with non-neurogenic LUT dysfunction were followed over 7 months; 115 patients were excluded due to incomplete data. Children were categorized based on presenting complaints and pelvic ultrasound into three groups: daytime urinary incontinence (UI) with complete emptying (CE), UI with incomplete emptying (IE), or IE without UI. Every child underwent behavioral modification (BM) including timed voiding, double voiding, deep breathing, and treatment of constipation if present. BM failures received secondary treatment including medications (alpha blockers, anticholinergics), physical therapy, and/or botulinum toxin type A injection of the external sphincter at a dose of 100 units. Results: BM improved symptoms in 152 (55%): 68% (46% dry), 49% (27% dry), and 59% (29% dry) from the three groups, respectively. Of the 45% who showed no change in symptoms, 98 (80%) improved with addition of medication, the majority (89) after starting alpha blocker therapy. Children with IE responded better to alpha blockers, 83 (77%) compared to 38% with CE, whereas those with CE demonstrated more symptom resolution with anticholinergics, 6 (38%) compared to 13% of those with IE. Only 6 (2%) patients were refractory to non-operative treatment with all showing improvement after injection of botulinum toxin type A, 4 (67%) of whom became completely dry. Conclusion: Diagnosis of UI and/or IE with stratification of children into particular symptom groups appears beneficial in determining the appropriate therapy for children with LUT dysfunction. [Copyright &y& Elsevier]

Surgical intervention in youth with differences of sex development (DSD) is a controversial topic. Historically, evidence suggests that genital surgery in DSD is associated with mixed patient satisfaction. However, less is known about surgical outcomes under therapeutic advancements in the past several decades. The purpose of the current study is to provide an updated and empirical qualitative examination of the surgical experiences and responses to care of adolescents and young adults (AYA) with DSD in order to fill this gap in the literature and inform patient care. Qualitative interviews were conducted with 37 AYA (ages 12–26) with DSD. Interviews were transcribed, coded, and analyzed thematically. Three major themes were identified: 1) knowledge related to surgery; 2) surgical and medical experience; and 3) psychosocial factors related to surgery. Results demonstrated that most AYA were not involved in the decision to pursue surgery and were observed to have varying levels of knowledge regarding their surgeries. Most participants in the current study had received a DSD-related surgery and the majority described having positive surgical experiences and few regrets. Nonetheless, AYA described both medical and psychosocial challenges related to their surgeries and recoveries and offered feedback on ways to improve the surgical process. The current study provides a qualitative examination of the surgical experiences of 37 AYA with DSD. Findings highlight the importance of regular and ongoing communication with providers to improve knowledge related to surgery during the decision-making process as well as after surgical intervention. Results underscore the benefits of multidisciplinary teams and the value of patient handouts and decision aids in assisting AYA in the decision to pursue surgical intervention. Other specific recommendations for providers include increased patient privacy during genital exams, greater emphasis on psychoeducation and pain management strategies, and the use of behavioral health services to assist with challenges and social support. The decision to pursue surgical intervention in youth with DSD remains a complex and controversial issue, and more information regarding patients' perspectives on surgery is needed. The current study provides novel insights into patient experiences of surgical intervention and highlights the need for psychosocial support throughout the shared decision-making process. [Display omitted] [ABSTRACT FROM AUTHOR]

Abstract: The effects of mechanoreceptor stimulation and subsequent ATP release in cyclophosphamide evoked chronic bladder inflammation was examined to demonstrate: (1) whether inflammation modulates ATP release from bladder urothelium and (2) whether intravesical botulinum toxin A administration inhibits urothelial ATP release, a measure of sensory nerve activation. ATP release was measured from rat bladders in a Ussing chamber, an apparatus that allows one to separately measure resting and mechanoreceptor evoked (e.g. hypoosmotic stimulation) ATP release from urothelial and serosal sides of the bladder. Cystometry was utilized to correlate changes in ATP release with alterations in the frequency of voiding and non-voiding bladder contractions, in vivo measures of bladder afferent activity. The resting urothelial release of ATP was not significantly affected by either cyclophosphamide or botulinum toxin A treatment. However, evoked ATP release following hypoosmotic stimulation was significantly increased (i.e. 94%) in chronic cyclophosphamide treated bladder urothelium compared to control bladders. In addition, botulinum toxin A treatment significantly reduced hypoosmotic shock induced ATP release in cyclophosphamide treated animals by 69%. Cystometry revealed that cyclophosphamide and botulinum toxin A treatments altered non-voiding (i.e. cyclophosphamide increased, botulinum toxin A decreased) but not voiding contraction frequency suggesting that alterations in urothelial ATP release selectively diminished underlying bladder C-fiber nerve activity. Finally, intravesical instillation of botulinum toxin A did not affect ATP release from the serosal side implying that its effects were confined to the urothelial side of the bladder preparation. [Copyright &y& Elsevier]

An Institutional Review Board-exempt REDCap survey was distributed through the Society of Academic Urologists to all 508 applicants registered for the 2023 Urology Match following the rank list submission deadline on January 10, 2023. The survey closed on February 1, 2023. Responses were anonymized, aggregated, and characterized using descriptive statistics. Thematic mapping of open text comments was performed by 2 reviewers. The response rate was 42% (215/508). Eighty-eight percent of respondents disapproved of the Dobbs ruling. Twenty percent of respondents (15% male/24% female) eliminated programs in states where abortion is illegal. Fifty-nine percent (51% male/70% female) would be concerned for their or their partner's health if they matched in a state where abortion was illegal, and 66% (55% male/82% female) would want their program to assist them or their partner if they required abortion care during residency. Due to the competitive nature of Urology, 68% of applicants reported feeling at least somewhat obligated to apply in states where abortion legislation conflicts with their beliefs. Of the 65 comments provided by respondents, 4 common themes emerged: (1) avoidance of states with restrictive abortion laws; (2) inability to limit applications because of the competitiveness of urology; (3) impacts on personal health care; and (4) desire for advocacy from professional urology organizations. The Dobbs ruling will impact the urology workforce by affecting urology applicants' decision-making regarding residency selection and ranking. Although the competitiveness of the Urology Match pressures applicants to apply broadly, many are taking reproductive health care access into consideration. [ABSTRACT FROM AUTHOR]

Summary Introduction Randomized controlled trials (RCTs) are considered the gold standard for assessing treatment efficacy. However, pediatric surgical RCTs have been limited in their ability to recruit patients. The purpose of this study was to identify barriers and motivators to pediatric participation in surgical RCTs. Methods We conducted a series of two focus groups with parents and one focus group with urology providers for children aged <2 years of age with a diagnosis of Society for Fetal Urology grade 3 or 4 hydronephrosis. We then administered a survey to referring pediatricians based on the initial analysis of focus group findings. Theme analysis was used for all qualitative transcribed text data obtained from focus groups and open-ended survey questions using team-based inductive approaches. Descriptive statistics were obtained for the remainder of the provider survey. Results Using qualitative text from stakeholders ( n = 38) we identified four key themes across the data : responsibility to my child; responsibility to my patient; responsibility to the field; and irreversibility of surgery . Participants felt there was an obligation to be informed of relevant scientific research within a clinic research culture. However, there remains a disconnect for parents between randomized research studies that may ultimately benefit their child, depending on their age and concern their child is being treated as a ‘guinea pig’. Some parents were willing to participate in RCTs but all were more open to participate in an observational study where the treatment decisions were felt to be under their control even when there was no “right answer” or multiple equivalent options for treatment. There was mixed opinion across the parents and providers whether research trial education and enrollment should be provided by the pediatrician or urologist. Active physician decisions were seen as critical within the context of a long term clinical relationship and provision of information of risks and benefits without pressure were considered essential for ethical research by both parents and providers. Conclusion While some parents are open to participation in surgical RCTs, providers and parents of children with hydronephrosis feel discomfort with the element of chance in surgical randomized trials. Parents and providers are more likely to participate in observational studies where treatment decisions may be made jointly by the physician and the parent. These findings suggest that pragmatic trial strategies with the option for participation in an observational cohort may improve recruitment of pediatric patients into surgical clinical trials. Table Perceived barriers to participation in surgical randomized controlled trials. Parents Pediatric urologists Pediatricians Counseling and decision-making Loss of control over treatment decision Child's inability to participate in decision Discomfort with admission of uncertainty in treatment Time and effort needed for education regarding RCTs Ethics of randomization in young children/lack of patient input into decision Complications and risks Stigma of child being labeled as “sick” Concerns about cost and long term complications Adequacy of safeguards and justification of potential risks Potential risk of sham surgery/anesthesia exposure Risk of side effects, unnecessary tests, and unknown long term consequences Unique attributes of surgery Irreversibility of surgery No difference between medical and surgical RCTs Lack of true equipoise between surgical and non-surgical treatment arms Irreversibility of surgery [ABSTRACT FROM AUTHOR]

Although anhydramnios due to in utero renal failure has traditionally been considered lethal, in utero interventions offer the potential for pulmonary survival. As fetal interventions become more common, questions arise about how to identify and counsel eligible candidates.  In this report we describe the presentation and management of a 17-year-old pregnant female who presented from out-of-state with severe lower urinary tract obstruction (LUTO) with associated anhydramnios, focusing on the ethical questions that this case raised. [ABSTRACT FROM AUTHOR]

Fetal megacystis, or an enlarged fetal bladder, is most often attributed to embryological defects, occurring early in gestation. Recent investigations have demonstrated that the underlying etiology of megacystis may be more myriad than originally thought. We present the third reported patient with megacystis due to an ACTA2 Arg179 substitution variant causing Multisystemic Smooth Muscle Dysfunction Syndrome. We also provide a description of pediatric evaluation and follow up. The growing number of cases in which this ACTA2 variant has been identified in fetal megacystis suggests that molecular sequencing is an appropriate consideration, particularly prenatally, when other features of Multisystemic Smooth Muscle Dysfunction Syndrome cannot be detected. [ABSTRACT FROM AUTHOR]

Objective: To describe the annual volume of pediatric urology cases in an academic, tertiary care setting.Methods: A retrospective review was performed of all patients operated on by 4 pediatric urologists (total of 2.5 full-time equivalents) at an academic, tertiary care center with a free-standing children's hospital from 2016 to 2017 (24 months). Basic case information was collected from operative reports. Descriptive statistics are reported using nonparametric methods. "Uncommon" was defined a priori as occurring <10% of the time.Results: During the entire study period, 2718 patients underwent 4580 procedures. This equated to 1088 patients and 1832 procedures per full-time equivalent. Median age at surgery was 3.2 years (IQR 0.8-10) and 757 (16.5%) of patients were female. Most procedures were elective (4406, 96.2%) and did not require postoperative admission (3842, 83.9%). Urgent and emergent cases were uncommon (174, 3.8%). Most cases were classified as general pediatric urology (3894, 85%) with 319 (7%) classified as major reconstruction, 275 (6%) as laparoscopy/endourology and 92 (2%) as oncology. The most common cases involved the groin/scrotum (1415, 30.9%), prepuce (809, 17.7%), phallus (802, 17.5%), and endoscopy (652, 14.2%). All other case types were uncommon.Conclusion: This description of an academic pediatric urology practice at a tertiary care center with a free-standing children's hospital noted a high volume of elective, outpatient procedures that are largely general pediatric urology. Uncommon cases include urgent/emergent interventions, major reconstruction, laparoscopy/endourology, and oncology procedures. [ABSTRACT FROM AUTHOR]

Cloacal exstrophy (CE) is a rare congenital disorder with a number of significant associated anomalies. Fetal ultrasound has been used to identify CE; however, accurate diagnosis is challenging. Recently, magnetic resonance imaging has been reported to improve prenatal diagnosis and better characterize the associated defects. We report a case of CE accurately diagnosed using magnetic resonance imaging after fetal ultrasound was nondiagnostic and review the literature comparing the use of each modality. [Copyright &y& Elsevier]