Feasibility of Enhanced Recovery After Surgery (ERAS) implementation in Pediatric Urology: Pilot-phase outcomes of a prospective, multi-center study.

INTRODUCTION/BACKGROUND: Enhanced Recovery After Surgery (ERAS) is a fundamental shift in perioperative care that has consistently demonstrated an improved outcome for a wide variety of surgeries in adults but has only limited evidence in the pediatric population. OBJECTIVE: We aimed to assess the success with and barriers to implementation of ERAS in a prospective, multi-center study on patients undergoing complex lower urinary tract reconstruction. STUDY DESIGN: Centers were directed to implement an ERAS protocol using a multidisciplinary team and quality improvement methodologies. Providers completed pre- and post-pilot surveys. An audit committee met after enrolling the first 5 patients at each center. Pilot-phase outcomes included enrollment of ≥2 patients in the first 6 months of enrollment, completion of 90 days of follow-up, identification of barriers to implementation, and protocol adherence. RESULTS: A total of 40 patients were enrolled across 8 centers. The median age at surgery was 10.3 years (IQR 6.4-12.5). Sixty five percent had a diagnosis of myelomeningocele, and 33 % had a ventriculoperitoneal shunt. A bladder augmentation was performed in 70 %, Mitrofanoff appendicovesicostomy in 52 %, Monti ileovesicostomy in 15 %, and antegrade continence enema channel in 38 %. The most commonly perceived barriers to implementation on the pre-pilot survey were "difficulty initiating and maintaining compliance with care pathway" in 51 % followed by a "lack of time, money, or clinical resources" in 36 %. The pre-pilot study experience, implementation, and pilot-phase outcomes are provided in the Table. All primary and secondary outcomes were achieved. DISCUSSION: The findings of the present study were similar to several small comparative studies with regard to the importance of a multidisciplinary team, strong leadership, and continuous audit for successful implementation of ERAS. Similar barriers were also encountered to other studies, which primarily related to a lack of administrative support, leadership, and buy-in from other services. The limitations of the present study included a relatively small heterogeneous cohort and absence of a comparative group, which will be addressed in the larger exploratory phase of the trial. The findings may also not be generaziable due to the need for sustainable processes that were unique to each center as well as an absence of adequate volume or resources at smaller centers. CONCLUSIONS: ERAS was successfully implemented for complex lower urinary tract reconstruction across 8 centers through a multidisciplinary team, structured approach based on the local context, and focus on a continuous audit.

One and done: Feasibility and Safety of Primary Ureteroscopy in a Pediatric Population.

BACKGROUND: Pediatric urolithiasis has been increasing at rate of 4-10 % annually in the United States, most notably within adolescents and females. A significant number of patients will require surgical management of their stones. Primary ureteroscopy (URS) affords the opportunity to treat stones under a single anesthetic with lower re-treatment rates or anatomical and stone characteristic limitations compared to shockwave lithotripsy. Previous studies evaluating primary URS have been largely underpowered, are limited by stone location, and/or are not representative of the stone population in the United States. OBJECTIVES: Primary study outcomes were the success of primary URS and patient characteristics associated with success. Secondary outcomes were the stone-free rate (SFR), 30-day emergency department (ED) visits, 30-day readmissions, and complications. METHODS: We performed a retrospective cohort study of patients less than 18 years of age from 2011 to 2023 who underwent primary URS. Patients were excluded if a ureteral stent was placed prior to URS or diagnostic URS was performed. A successful primary URS was considered if access to the ureter was obtained and treatment of the stone(s) completed. In failed primary URS, a ureteral stent was placed for staged management. RESULTS: A total of 196 patients were included and primary URS was performed or attempted on 224 renal units. The median age was 15.8 (IQR 13.4-16.9) years and median follow up 8.4 (IQR 1.1-24.6) months. The success rate of primary URS was 79 %. No significant characteristics were appreciated for successful primary URS based on: overall age, <14 vs > 14 years of age, sex, body mass index, history of stones, history of endourologic procedures, preoperative alpha blockade, location of stone(s), multiple stones, type of URS, or acute treatment. In successful primary URS, the SFR was 88 % with stone size (p = 0.0001) the only predictor of having residual stones. The 30-day ED rate was 21.4 %, 30-day unplanned readmission rate was 12.5 %, and complication rate was 7.5 %. No long-term complications were appreciated. DISCUSSION: Our success of primary URS compares favorably to previously published literature. Our SFR rate, 30-day ED visits, 30-day unplanned readmission, and complication rates are similar to other studies. Limitations of the study are its retrospective design, selection bias, and intermediate follow-up. CONCLUSIONS: Primary URS can be completed safely in the majority of pediatric patients without any patient characteristics associated with success. We advocate for primary URS when possible due to the excellent SFR and potential of treating stones under a single anesthetic.

Long-Term Male Sexual Function and Fecal Incontinence Outcomes for Adult Patients with Hirschsprung Disease or Anorectal Malformation.

BACKGROUND: This study sought to characterize sexual function and fecal incontinence related quality of life (QOL) outcomes for adult males with anorectal malformation (ARM) or Hirschsprung's Disease (HD). METHODS: We conducted a cross-sectional survey study of male patients ≥18 years with ARM or HD. Patients were identified from our institutional database, contacted and consented by telephone, and sent a REDCap survey via email. The International Index of Erectile Function (IIEF-5) and Male Sexual Health Questionnaire (MSHQ) evaluated erectile dysfunction (ED) and ejaculatory dysfunction (EjD), respectively. The Cleveland Clinic Incontinence Score (CCIS) and the Fecal Incontinence Quality of Life Scale (FIQLS) assessed fecal incontinence-related outcomes. A linear regression analysis of IIEF-5 scores compared to CCIS scores was used to evaluate for an association between ED and incontinence. RESULTS: Of 63 patients contacted, 48 completed the survey. The median age for respondents was 22.5 years (IQR 20-25). There were 19 patients with HD and 29 patients with ARM. On the IIEF-5 survey, 35.3% report some level of ED. On the MSHQ-EjD survey, the median score was 14 out of 15 (IQR 10.75-15), indicating few EjD concerns. The median CCIS was 5 (IQR 2.25-7.75) and the median FIQL scores ranged from 2.7 to 3.5 depending on the domain assessed, demonstrating some QOL challenges secondary to fecal incontinence. On linear regression analysis, IIEF-5 and CCIS scores were weakly associated (B = -0.55, p = 0.045). CONCLUSIONS: Male adult patients with ARM or HD may have ongoing concerns with sexual function and fecal incontinence. LEVEL OF EVIDENCE: Level 4. TYPE OF STUDY: Cross-Sectional Survey Study.

Reoperative Anorectal Procedures in Patients with Anorectal Malformations - Is Bladder Function Affected?

BACKGROUND: Anorectal malformations (ARM) are associated with neurogenic bladder. The traditional surgical ARM repair is a posterior sagittal anorectoplasty (PSARP), which is believed to have a minimal effect on bladder dynamics. However, little is known about the effects of reoperative PSARP (rPSARP) on bladder function. We hypothesized that a high rate of bladder dysfunction existed in this cohort. METHODS: We performed a retrospective review of ARM patients undergoing rPSARP at a single institution from 2008 to 2015. Only patients with Urology follow-up were included in our analysis. Data was collected regarding original level of ARM, coexisting spinal anomalies and indications for reoperation. We assessed urodynamic variables and bladder management (voiding, CIC or diverted) before and after rPSARP. RESULTS: A total of 172 patients were identified, of which 85 met inclusion criteria with a median follow-up of 23.9 months (IQR, 5.9-43.8 months). Thirty-six patients had spinal cord anomalies. Indications for rPSARP included mislocation (n = 42), posterior urethral diverticulum (PUD; n = 16), stricture (n = 19) and rectal prolapse (n = 8). Within 1 year following rPSARP, 11 patients (12.9%) had a negative change in bladder management, defined as need for beginning intermittent catheterization or undergoing urinary diversion, which increased to 16 patients (18.8%) at last follow-up. Postoperative bladder management changed in rPSARP patients with mislocation (p < 0.0001) and stricture (p 0.005) but not for rectal prolapse (p 0.143). CONCLUSIONS: Patients who undergo rPSARP warrant especially close attention for bladder dysfunction as we observed a negative postoperative change in bladder management in 18.8% of our series. LEVEL OF EVIDENCE: Level IV.

Reduced-Intensity Conditioning Mitigates Risk for Primary Ovarian Insufficiency but Does Not Decrease Risk for Infertility in Pediatric and Young Adult Survivors of Hematopoietic Stem Cell Transplantation.

Hematopoietic stem cell transplantation (HSCT) is a curative therapy for many pediatric malignant and nonmalignant conditions. Gonadal insufficiency or infertility is present in almost all HSCT survivors who received a myeloablative conditioning (MAC) regimen. Reduced-intensity conditioning (RIC) regimens are being increasingly used in medically fragile patients or in patients with nonmalignant diagnoses to limit the toxicities associated with HSCT; however, the short-term and long-term gonadal toxicity of RIC regimens in pediatric and young adult survivors remains unknown. In this study, we compared the prevalence of gonadal insufficiency and infertility among pubertal and postpubertal pediatric and young adult survivors of HSCT who received a RIC regimen versus those who received a MAC regimen. Twenty-three females (RIC, n = 8; MAC, n = 15) and 35 males (RIC, n = 19; MAC, n = 16) were included in this single-center, retrospective cross-sectional study. Eligible patients were those with available laboratory results who were ≥1 year post-HSCT, age <40 years, and pubertal or postpubertal as assessed by an endocrinologist. Follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, and anti-Müllerian hormone (AMH) levels were measured in females, and FSH, LH, total testosterone, and inhibin B (InhB) levels were measured in males. Twenty-one males (RIC, n = 11; MAC, n = 10) underwent semen analysis through a separate consent. Parametric and nonparametric analyses were undertaken to compare the RIC and MAC groups. Female patients who received RIC were less likely than those who received MAC to develop primary ovarian insufficiency, as demonstrated by elevated FSH (P = .02) and low estradiol (P = .01) or elevated LH (P = .09). Most females in the RIC (75%) and MAC (93%) groups had low AMH levels, indicating low or absent ovarian reserve, with no significant difference between the groups (P = .53). In males, there were no significant differences between the 2 groups in the prevalence of abnormal FSH, LH, testosterone, or InhB levels. Ten of 11 RIC males (91%) and 10 of 10 MAC males (100%) had azoospermia or oligospermia, at a median time to semen analysis from HSCT of 3.7 years (range, 1.3 to 12.2 years). RIC may pose less risk than MAC for primary ovarian insufficiency among female survivors of HSCT; however, both female and male recipients of either RIC or MAC regimens are at high risk for infertility. In the largest reported series of semen analyses of pediatric and young adult male recipients of RIC, azoospermia or oligospermia was found in nearly all (91%) RIC survivors. All patients undergoing HSCT should receive counseling about the high risk of gonadal toxicity, and efforts should be made to preserve fertility in patients undergoing either RIC or MAC.

Urinary and bowel management in cloacal exstrophy: A long-term multi-institutional cross-sectional study.

BACKGROUND: We sought to evaluate long-term surgical urinary and bowel management in cloacal exstrophy (CE) in a multi-institutional study. METHODS: We performed a cross-sectional study of people with CE and covered variants managed at five participating institutions. Those with <1 year follow-up or born with variants without hindgut involvement were excluded. Primary outcomes were methods of urinary and bowel management. Urinary management included: voiding via urethra, clean intermittent catheterizations (CIC), incontinent diversion and incontinent in diaper. Bowel management included: intestinal diversion (colostomy/ileostomy) and pull-through (with/without MACE). We evaluated three age groups: children (<10 years), older children (10 to <18) and adults (≥18). We assessed if management varied by age, institution or time (born≤2000 vs. >2000). RESULTS: A total of 160 patients were included (40% male). Median follow-up was 15.2 years (36% children, 22% older children, 43% adults). While 42% of children were incontinent in diapers, 73% of older children and adults managed their bladder with CIC, followed by incontinent urinary diversion (21%) (p < 0.001, Table). CIC typically occurred after augmentation (88%) via a catheterizable channel (89%). Among older children and adults, 86% did not evacuate urine per urethra and 28% of adults had an incontinent urinary diversion. No child or adult voided per urethra. Age-adjusted odds of undergoing incontinent diversion was no different between institutions (p = 0.31) or based on birthyear (p = 0.08). Most patients (79%) had an intestinal diversion, irrespective of age (p = 0.99). Remaining patients had a pull-through, half with a MACE. The probability of undergoing bowel diversion varied significantly between institutions (range: 55-91%, p = 0.001), but not birth year (p = 0.85). SUMMARY: We believe this large long-term data presents a sobering but realistic view of outcomes in CE. A limitation is our data does not assess comorbidities or patient-reported outcomes. Rarity of volitional urethral voiding in CE forces the question of whether is a potentially unachievable goal. We advocate thoughtful surgical decision making and thorough counseling about appropriate expectations, distinguishing between volitional voiding and urinary and fecal dryness. CONCLUSIONS: In this long-term, multi-institutional study of patients with CE, 94% of older children and adults manage their bladder with incontinent diversion or CIC. Nearly 80% of patients, regardless of age, have an intestinal diversion. Given that no patients were dry and voided via urethra and 86% of older patients do not evacuate urine per urethra, these data bring into question what functional goals are achievable when performing reconstructive surgery for these patients.

Evaluating the role for regional analgesia in children with spina bifida: a retrospective observational study comparing the efficacy of regional versus systemic analgesia protocols following major urological surgery.

INTRODUCTION: Regional techniques are a key component of multimodal analgesia and help decrease opioid use perioperatively, but some techniques may not be suitable for all patients, such as those with spina bifida. We hypothesized peripheral regional catheters would reduce postoperative opioid use compared with no regional analgesia without increasing pain scores in pediatric patients with spina bifida undergoing major urological surgery. METHODS: A retrospective review of a multicenter database established for the study of enhanced recovery after surgery was performed of patients from 2009 to 2021 who underwent bladder augmentation or creation of catheterizable channels. Patients without spina bifida and those receiving epidural analgesia were excluded. Opioids were converted into morphine equivalents and normalized to patient weight. RESULTS: 158 patients with pediatric spina bifida from 7 centers were included, including 87 with and 71 without regional catheters. There were no differences in baseline patient factors. Anesthesia setup increased from median 40 min (IQR 34-51) for no regional to 64 min (IQR 40-97) for regional catheters (p<0.01). The regional catheter group had lower median intraoperative opioid usage (0.24 vs 0.80 mg/kg morphine equivalents, p<0.01) as well as lower in-hospital postoperative opioid usage (0.05 vs 0.23 mg/kg/day morphine equivalents, p<0.01). Pain scores were not higher in the regional catheters group. DISCUSSION: Continuous regional analgesia following major urological surgery in children with spina bifida was associated with a 70% intraoperative and 78% postoperative reduction in opioids without higher pain scores. This approach should be considered for similar surgical interventions in this population. TRIAL REGISTRATION NUMBER: NCT03245242.

Factors Associated With Fertility Preservation in a Pediatric, Adolescent and Young Adult Population.

OBJECTIVE: To determine the patient characteristics associated with pursuing fertility preservation (FP) before gonadotoxic therapy in a pediatric, adolescent and young adult patient population. METHODS: This is a retrospective cohort study of patient data at Cincinnati Children's Hospital Medical Center. Demographics, clinical diagnoses, and treatment characteristics were compared between participants that selected FP versus those that declined. Variables were analyzed separately for males and females by logistic regression. RESULTS: Patients with a hematologic cancer were less likely to be eligible for preservation: 53.9% of ineligible males, P <0.001, and 51.8% of ineligible females, P <0.0001. Among patients who were candidates for FP, those receiving high-risk therapy were more likely to elect for FP (65.3% males, P <0.0001, and 87.5% of females, P <0.0001). Pubertal males were more likely to undergo preservation than prepubertal males (70.5% vs. 29.5%, P <0.0001; however, this trend was not demonstrated among female patients. In both males and females, race, ethnicity, religion, primary language, and insurance status were not shown to be statistically significant factors in predicting utilization of FP. CONCLUSION: Risk of infertility, type of cancer, and developmental status influenced decisions on pursuing FP in pediatric, adolescent and young adult patients facing iatrogenic infertility.

Validation of the modified Bosniak classification system to risk stratify pediatric cystic renal masses: An international, multi-site study from the pediatric urologic oncology working group of the societies for pediatric urology.

BACKGROUND: Pediatric cystic renal lesions are challenging to manage as little is known about their natural course. A modified Bosniak (mBosniak) classification system has been proposed for risk stratification in pediatric patients that takes ultrasound (US) and/or computed tomogram (CT) characteristics into account. However, literature validating this system remains limited. OBJECTIVE: To determine if the mBosniak classification system correlates with pathologic diagnoses. The hypothesis is that mBosniak classification can stratify the risk of malignancy in children with renal cysts. STUDY DESIGN: Patients treated for cystic renal masses with available imaging and pathology between 2000 and 2019 from five institutions were identified. Clinical characteristics and pathology were obtained retrospectively. Characteristics from the most recent US, CT, and/or magnetic resonance imaging (MRI) were recorded. Reviewers assigned a mBosniak classification to each scan. mBosniak scores 1/2 were considered low-risk and 3/4 high-risk. These groups were compared with pathology (classified as benign, intermediate, malignant). Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (+LR), and negative likelihood ratio (-LR) were calculated to assess this categorization as a screening tool to guide surgical intervention. Agreement between imaging modalities was also explored. RESULTS: 99 patients were identified. High-risk imaging findings were correlated with malignant or intermediate pathology with a sensitivity of 88.3%, specificity of 84.6%, PPV of 89.8%, NPV of 82.5%, +LR of 5.7, and -LR of 0.14. The sensitivity for detecting malignant lesions only was 100%. There was substantial agreement between US/CT (n = 55; κ = 0.66) and moderate agreement between US/MRI (n = 20; κ = 0.52) and CT/MRI (n = 13; κ = 0.47). DISCUSSION: The mBos classification system is a useful tool in predicting the likelihood of benign vs. intermediate or malignant pathology. The relatively high sensitivity and specificity of the system for prediction of high-risk lesions makes this classification applicable to clinical decision making. In addition, all malignant lesions were accurately identified as mBosniak 4 on imaging. This study adds substantial data to the relatively small body of literature validating the mBosniak system for risk stratifying pediatric cystic renal lesions. CONCLUSIONS: Pediatric cystic renal lesions assigned mBosniak class 1/2 are mostly benign, whereas class 3/4 lesions are likely intermediate or malignant pathology. We observed that the mBosniak system correctly identified pathology appropriate for surgical management in 88% of cases and did not miss malignant pathologies. There is substantial agreement between CT and US scans concerning mBos classification.

Diagnosis, management and recurrence rates of Mitrofanoff polyps.

BACKGROUND: Intraluminal polyps are a known complication following creation of a urinary continent catheterizable channel (Mitrofanoff). These polyps can lead to difficult catheterizations in addition to symptomatic bleeding. However, there is limited data available regarding management and outcomes of these polyps. We aim to describe clinical presentation and management of a large series of polyps occurring in a Mitrofanoff channel. METHODS: We performed a retrospective review of all patients that were treated for polyps in a Mitrofanoff at our institution. Information was collected regarding presenting symptoms, management and recurrence rates of the polyps. RESULTS: A total of 24 patients were identified that fulfilled inclusion criteria. The majority of these polyps developed in channels composed of appendix (87%), while only 3 patients (13%) had polyps develop in an ileal composed channel. Thirteen (54%) of these polyps were incidentally diagnosed while 11 patients presented with a variety of symptoms such as difficulty in catheterization, bleeding with catheterization or both difficulty catheterizing and bleeding. For management of the polyps, a cystoscopy was performed and snaring the polyp with stone basket was performed in 37%, energy was applied to base to remove polyps in 33%, 16% were fulgurated and only 13% were left in situ. All procedures were performed under general anesthesia and all of the pathology was benign showing chronic inflammatory tissue. Eight polyps (33%) recurred after initial treatment. DISCUSSION: We did not observe an asymptomatic channel polyp convert to a symptomatic during our follow up period. Our experience has led us to not intervene on all asymptomatic Mitrofanoff polyps encountered during cystoscopy under assumption they will inevitably become symptomatic. Although we admit our follow up period may not be long enough to make this a universal declaration of best practice when any Mitrofanoff polyp is diagnosed. Endoscopic treatment was effective minimally invasive method to address the symptomatic polyp rather than excision and construction of new channel. CONCLUSIONS: This is the largest series to date of polyps developing in urinary continent catheterizable channels. The majority of these polyps were encountered incidentally however symptomatic polyps presented with difficulty with catheterizations. Symptomatic polyps can be managed endoscopically but recurrence of the polyp can occur.

Comparison of risk of anticholinergic utilization for treatment of neurogenic bladder between in utero or postnatal myelomeningocele repair.

INTRODUCTION: In utero myelomeningocele (MMC) repair has resulted in significant decrease in need for shunt-dependent hydrocephalus, however its impact on bladder function remains less clear. Neurogenic detrusor overactivity (NDO) due to MMC can be addressed with combination of clean intermittent catheterization (CIC) and anticholinergic (AC) therapy to minimize its effect on bladder function and upper urinary tract. The aim of this study was to compare the risk of postnatal AC utilization for in patients that underwent either postnatal or in utero MMC repair related to neurogenic bladder (NGB) management. We hypothesized that postnatal MMC repair would be associated with increased risk of postnatal AC utilization compared to in utero MMC repair. MATERIAL AND METHODS: All newborns with MMC in our hospital undergo prospective surveillance radiographic and urodynamic testing as part of institutional MMC protocol. Those MMC patients born between 2013 and 2018 at our institution, who underwent in utero or postnatal MMC repair were retrospectively analyzed. We identified postnatal AC utilization from electronic medical records and recorded indications for AC therapy according to the urodynamic, radiographic and clinical findings related to NGB management. RESULTS: 97 patients fulfilled the inclusion criteria. 56 patients underwent a postnatal and 41 an in utero repair. Median follow-up for the in utero and postnatal MMC repair groups was the same at 37 months (p = 0.53). More newborns from the postnatal group were discharged from birth hospital performing CIC (91.1% vs. 58.5%, p < 0.0001), however upon last follow up no difference existed between the groups on use of CIC (83.9% postnatal group % vs. 82.9% in utero group, p = 0.78). At last follow up, postnatal AC utilization was observed in 75% of postnatal MMC group compared to 78.1% of the in utero MMC repair group, p = 0.81 (Figure). The median time to AC utilization was 6.9 months and 8.8 months in the in utero and postnatal groups, respectively (p = 0.28). DISCUSSION: We observed no reduction in risk of AC utilization with in utero repair which refuted the hypothesis of our study. Indications for AC utilization were urodynamic abnormalities such as NDO (81.3% in utero vs. 81% postnatal) or impaired bladder compliance (53.1% in utero vs. 64.3% postnatal). CONCLUSIONS: We found no difference in risk of postnatal AC utilization between in utero or postnatal MMC repair. CIC rates were higher at birth hospital discharge in the postnatal repair group, however at last follow up CIC rates did not differ between groups.

Outcomes according to channel type for continent catheterizable channels in patients undergoing simultaneous urinary and fecal reconstruction.

INTRODUCTION: When creating a continent catheterizable channel (CCC) the choice of bowel segment used as the conduit should be tempered with the morbidity associated with it. The split-appendix technique allows the creation of both a urinary and fecal CCC without the need for a bowel anastomosis. However, there is concern that by splitting the appendix there is compromise to its blood supply and may affect outcomes. We aim to compare what affect the bowel segment used for urinary and fecal CCCs has on perioperative and long-term outcomes in patients undergoing simultaneous urinary and fecal reconstruction. METHODS: A retrospective review was performed analyzing all patients that underwent simultaneous continent catheterizable urinary and fecal CCC between the years 2010-2016. Patient demographics, channel characteristics, perioperative complications and clinical success rate were analyzed. RESULTS: A total of 106 patients were identified that had simultaneous fecal and urinary CCC created at time of reconstruction. For urinary CCC, there were 64 patients (60.4%) that underwent a split-appendix technique, 27 patients (25.4%) underwent a Monti, and 15 patients (14.2%) had the appendix used only for the urinary channel. Those patients undergoing a split-appendix technique had median operative time of 447 min compared to 619 min when a Monti channel was created. The median length of hospital stay was 9 days for the split-appendix technique compared to 12 and 13 day median hospital stay when the appendix was used only for the urinary channel or a Monti was created, respectively. There was no difference seen in revision free survival of the channel following surgery of any of the channels with median follow-up of 44.5 months. However, there were more subfascial revisions of urinary CCC in those that underwent a Monti (5 patients, 18.5%) compared to other bowel segments (0 patients). CONCLUSIONS: Utilizing a split-appendix approach for creation of urinary and fecal CCCs does not affect 30-day complications or long-term revision rates compared to other established techniques. This technique minimizes the potential surgical morbidity of a bowel anastomosis and provide shorter operative times, when feasible, at time of simultaneous creation of fecal and urinary CCC.

Design and development of the Pediatric Urology Recovery After Surgery Endeavor (PURSUE) multicentre pilot and exploratory study.

INTRODUCTION: Lower urinary tract reconstruction in paediatric urology represents a physiologically stressful event that is associated with high complication rates, including readmissions and emergency room visits. Enhanced recovery after surgery (ERAS) protocol is a set of multidisciplinary, perioperative strategies designed to expedite surgical recovery without adversely impacting readmission or reoperation rates. Early paediatric urology data demonstrated ERAS reduced complications in this population. METHODS AND ANALYSIS: In 2016, a working group of paediatric urologists and anaesthesiologists convened to develop an ERAS protocol suitable for patients undergoing lower urinary tract reconstruction and define study process measures, patient-reported outcomes and clinically relevant outcomes in paediatric and adolescent/young adult patients. A multicentre, prospective, propensity-matched, case-control study design was chosen. Each centre will enrol five pilot patients to verify implementation. Subsequent enrolled patients will be propensity matched to historical controls. Eligible patients must be aged 4-25 years and undergoing planned operations (bladder augmentation, continent ileovesicostomy or appendicovesicostomy, or urinary diversion). 64 ERAS patients and 128 controls will be needed to detect a decrease in mean length of stay by 2 days. Pilot phase outcomes include attainment of ≥70% mean protocol adherence per patient and reasons for protocol deviations. Exploratory phase primary outcome is ERAS protocol adherence, with secondary outcomes including length of stay, readmissions, reoperations, emergency room visits, 90-day complications, pain scores, opioid usage and differences in Quality of Recovery 9 scores. ETHICS AND DISSEMINATION: This study has been registered with authors' respective institution review boards and will be published in peer-reviewed journals. It will provide robust insight into the feasibility of ERAS in paediatric urology, determine patient outcomes and allow for iteration of ERAS implementations as new best practices and evidence for paediatric surgical care arise. We anticipate this study will take 4 years to fully accrue with completed follow-up. TRIAL REGISTRATION NUMBER: NCT03245242; Pre-results.

XY Gonadal Dysgenesis in a Phenotypic Female Identified by Direct-to-Consumer Genetic Testing.

We report a 16-year-old phenotypic female with 46,XY complete gonadal dysgenesis and metastatic dysgerminoma, unexpectedly discovered through direct-to-consumer (DTC) commercial genetic testing. This case underscores the importance of timely interdisciplinary care, including psychosocial intervention and consideration of gonadectomy, to optimize outcomes for individuals with differences of sex development. Her unique presentation highlights the implications of DTC genetic testing in a new diagnostic era and informs general pediatricians as well as specialists of nongenetic services about the value, capabilities, and limitations of DTC testing.

Clinical urologic and urodynamic outcomes in patients with anorectal malformation and absent vagina after vaginal replacement.

INTRODUCTION AND OBJECTIVES: Anorectal malformations (ARMs) represent a complex spectrum of anorectal and genitourinary anomalies and a paucity of evidence is available on long-term urologic outcomes in all ARM subtypes. It was our subjective bias from being a referral center for ARM patients that the subtype of rectovestibular fistula and absent vagina had higher risk of renal and bladder abnormalities than typical rectovestibular fistula patients. Therefore, to confirm or refute our clinical suspicions, the purpose of this study was to review this specific cohort of ARM patients and describe both the clinical urological and urodynamic outcomes. METHODS: A retrospective cohort study was performed for 120 patients who were treated for ARM and vaginal replacement at our institution between 1991 and 2017. Fifteen patients with rectovestibular fistula and absent vagina were included in our review. Demographic and clinical data were abstracted from their medical records, including urodynamic findings, need for clean intermittent catheterization (CIC), urinary continence, and renal function. RESULTS: Vaginal replacement surgery was undertaken concomitantly with ARM repair in 10 of the 15 patients (67%). One patient was lost to follow up, and mean follow up postoperatively was 39 months. In all but one patient, rectum or colon was used as the substrate for vaginal replacement. Of the 15 patients, 13 had continence data available. A total of 10 patients (77%) were able to achieve social continence. Overall six patients used CIC to manage their bladder and 40% of continent patients used CIC. Urinary continence outcomes in patients who had partial vaginal replacement compared to those with total vaginal replacement did not reveal a clinically significant difference. Continence was achieved in 3/4 patients (75%) with a history of tethered cord compared to 7/9 patients (78%) without a history of tethered cord release. Urodynamics were performed postoperatively in 7 of the 157 patients (47%). Uninhibited detrusor contractions (UDCs) were present in 3 out of 7 patients, and a cystometric capacity greater than expected was noted in 4 patients. Additionally, 2 patients had end filling detrusor pressure greater than 40 cm H2O. GFR data were available for 13 of the 15 patients and (85%) were classified as chronic kidney disease (CKD) stage I or not having any significant loss of renal function. CONCLUSIONS: In this cohort of rectovestibular fistula and absent vagina, 77% reported achieving urinary continence. However CIC was employed in 40% of the patients which is higher than prior published noncloaca female ARM patient population. Urodynamic abnormalities were noted when performed and led to change in bladder management. Renal function measured with GFR was normal in 85%. Patients with rectovestibular fistula and absent vagina benefit from urologic screening given higher rates of lower urinary tract dysfunction that can require CIC to protect the upper urinary tract and achieve urinary continence. TYPE OF STUDY: Case series. LEVEL OF EVIDENCE: Level IV.

Need for botulinum toxin injection and bladder augmentation after isolated bladder outlet procedure in pediatric patients with myelomeningocele.

INTRODUCTION: In patients with neurogenic bladder outlet incompetence, a bladder outlet procedure (BOP) may be required to achieve urinary continence. However, when performed in isolation, a BOP can be associated with bladder deterioration and upper-tract injury. In the event of bladder deterioration, additional procedures such as bladder augmentation (BA) or botulinum toxin injection (BTI) may be pursued. OBJECTIVE: The aim of this study was to assess long-term outcomes after isolated BOP in a pediatric myelomeningocele (MMC) population, including the need for additional surgical intervention in the form of BTI or BA. MATERIALS AND METHODS: A retrospective cohort study was performed for patients with MMC who underwent an isolated BOP between 2004 and 2017. Primary outcomes included the need for postoperative BTI or BA. Secondary outcomes included the association between preoperative urodynamic parameters and need for BTI or BA. RESULTS: BTI or BA was performed in 18 of 36 (50%) patients at a median of 17.8 months (IQR 11.2-29.3) after an isolated BOP. A median of 1 (IQR 1-3) BTI was performed in 11 (30.6%) patients. BA was performed in 9 (25%) patients, including 2 patients who previously underwent BTI. Patients who did not undergo BTI or BA after BOP had a slightly increased percentage estimated bladder capacity at the end of follow-up (107% versus 95%, p=0.42). By contrast, patients who underwent BTI or BA had a post-BOP percentage estimated bladder capacity that decreased from 112 to 70% (p < 0.001), increased maximum detrusor leak point pressure from 43 to 67 cm H2O (p = 0.01), and higher rate of de novo upper-tract changes. Unfortunately, no preoperative clinical, radiographic, or urodynamic factors predicted the need for BTI or BA. DISCUSSION: On time-to-event analysis, the risk of BTI or BA was 53% at 5 years in our cohort. Risk of these procedures was highest in the first two years after BOP. 9 of 11 (82%) patients who underwent BTI had improvement in bladder dynamics and BA was not pursued. These findings suggest that BTI provides a less-morbid alternative to BA in patients with MMC and de novo adverse bladder storage changes after an isolated BOP. CONCLUSION: The need for BTI or BA after an isolated BOP is significant in patients with MMC. BTI offers a less-invasive alternative to BA in this population.

Probability of Bladder Augmentation, Diversion and Clean Intermittent Catheterization in Classic Bladder Exstrophy: A 36-Year, Multi-Institutional, Retrospective Cohort Study.

PURPOSE: We assessed the probability of bladder augmentation/diversion and clean intermittent catheterization in classic bladder exstrophy in a multi-institutional cohort. MATERIALS AND METHODS: We included children born from 1980 to 2016 with bladder exstrophy and treated across 5 centers (exclusion criteria less than 1 year followup after birth, isolated epispadias, bladder exstrophy variants etc). Outcomes were probability of bladder augmentation/diversion after bladder closure and proportion of patients performing clean intermittent catheterization at last followup. Survival analysis was used. RESULTS: Of 216 patients 63.4% were male (median followup 14.4 years). Overall 4 patients (1.9%) underwent primary diversion and 212 underwent primary closure (72.6% in first week of life). After primary closure 50.9% underwent augmentation, 4.7% diversion and 44.8% neither. By age 18 years 88.5% underwent a bladder neck procedure (synchronous augmentation 27.3%). On survival analysis the probability of bladder augmentation/diversion was 14.9% by age 5 years, 50.7% by 10 years and 70.1% by 18 years. Probability of bladder augmentation/diversion varied significantly between centers (p=0.01). Probability of bladder augmentation/diversion was 60.7% 10 years after bladder neck procedure. At last followup of the entire cohort 67.4% performed clean intermittent catheterization. Among 95 patients with intact native bladders 30.5% performed clean intermittent catheterization (channel 72.4%). Among 76 adults without a diversion 85.5% performed clean intermittent catheterization (augmented bladder 100.0% clean intermittent catheterization, native bladder 31.3%). Fifteen patients underwent diversion (continent 8, ureterosigmoidostomy 5, incontinent 2). CONCLUSIONS: On long-term followup probability of bladder augmentation/diversion increased with age, with 1 in 2 patients by age 10 years and the majority in adulthood. Probability of bladder augmentation/diversion differed among institutions. Almost a third of patients, including adults, with a closed native bladder performed clean intermittent catheterization. Considering all adults only 14% did not perform clean intermittent catheterization.

Development of a Pediatric Fertility Preservation Program: A Report From the Pediatric Initiative Network of the Oncofertility Consortium.

Infertility is known to decrease quality of life among adults. In some cases, infertility is caused by medical conditions and/or treatments prescribed in childhood, and using methods to protect or preserve fertility may expand future reproductive possibilities. Structured programs to offer counseling about infertility risk and fertility preservation options are essential in the care of pediatric patients facing fertility-threatening conditions or treatments, yet multiple barriers to program development exist. This report was developed from the institutional experiences of members of the Pediatric Initiative Network of the Oncofertility Consortium, with the intent of providing guidance for health care providers aiming to establish programs at institutions lacking pediatric fertility preservation services. The mechanics of building a fertility preservation program are discussed, including essential team members, target populations, fertility preservation options (both established and experimental), survivorship issues, research opportunities, and ethical considerations. Common barriers to program development and utilization, including low referral rates and financial concerns, are also discussed, and recommendations made for overcoming such barriers.

Pediatric Partial Nephrectomy for Upper Urinary Tract Duplication Anomalies: A Comparison Between Different Surgical Approaches and Techniques.

OBJECTIVE: To review and compare 4 different surgical approaches for partial nephrectomy of a nonfunctioning moiety in children with upper urinary tract duplication anomalies. MATERIALS AND METHODS: A retrospective review of all pediatric patients who underwent open partial nephrectomy (OPN), laparoscopic partial nephrectomy (LPN), robotic partial nephrectomy (RPN), or laparoendoscopic single site partial nephrectomy (LESS-PN) for the treatment of a nonfunctioning moiety in a duplicated collecting system at 2 medical centers between 2007 and 2017. Patient demographics, perioperative data, surgical techniques, complications, and results were compared. RESULTS: A total of 59 pediatric patients underwent partial nephrectomy for an upper urinary tract duplication anomaly during a 10-year period: 24 OPN, 7 LPN, 18 RPN, and 10 LESS-PN. Median age was 16 months (interquartile range 9-49.7). Median weight was 10.7 Kg (interquartile range 8.8-16.4). Median estimated blood loss was comparable between all minimally invasive approaches, but significantly increased in the open approach. OPN required more narcotics (0.554 mg Morphine equivalent/Kg/day, range 0.03-6.13) and Acetaminophen (72.12 mg/Kg/day, range 0-209.06) than all other groups in the study. Median operating time in OPN (154.5 minutes, range 108-413) and LESS-PN (140 minutes, range 65-245) were found to be significantly shorter in comparison to LPN (190 minutes, range 159-355), and RPN (256 minutes, range 163-458); (P = .03, .005, .02, and .005). CONCLUSION: Minimally invasive approaches (LPN, RPN, and LESS-PN) for partial nephrectomy in upper urinary tract duplication anomalies may be associated with decreased postoperative analgesia requirements, shorter hospital stay, less blood loss, and less use of drains in comparison to the open approach, while demonstrating efficacy and safety.