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.

Outpatient Robotic Unilateral Extravesical Ureteral Reimplantation in the Pediatric Population: Short-Term Assessment of Safety.

PURPOSE: Robotic extravesical ureteral reimplantation has been established as a viable option for surgical management of vesicoureteral reflux. Typically this procedure is associated with a hospital stay for routine postoperative care. We assessed the short-term safety of robotic unilateral extravesical ureteral reimplantation as a scheduled outpatient procedure in a pediatric population. MATERIALS AND METHODS: We retrospectively studied a cohort of patients who underwent robotic extravesical ureteral reimplantation between June 2012 and January 2018. No regional blocks were performed. Patients were discharged from the postanesthesia care unit as part of a scheduled outpatient procedure without an extended stay. Postoperative outcomes included 30-day emergency room visits, readmissions to the hospital and Clavien-Dindo grade I to V complications. RESULTS: Four male and 23 female patients were identified. Median age was 85 months (range 27 to 210) and median weight was 26 kg (13 to 97). Median robotic console time was 140 minutes (range 84 to 257). No patient required a hospital stay for management of pain. Two patients (9%) required unplanned antibiotic therapy postoperatively for bacterial cystitis and pneumonia (Clavien-Dindo grade II complications). The patient with pneumonia was diagnosed during a subsequent emergency room visit. One patient was rehospitalized on postoperative day 4 because of constipation. No Clavien-Dindo grade III or higher complication was observed in any patient. CONCLUSIONS: Robotic unilateral extravesical ureteral reimplantation is safe as an outpatient procedure in the pediatric population. Further evaluation is warranted to assess its short and long-term outcomes on a larger scale.

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.

Fertility preservation options in pediatric and adolescent patients with cancer.

The incidence of childhood cancer has steadily increased since the 1950s, with approximately 16,000 children diagnosed each year. However, with the advent of more effective multimodal therapies, childhood cancer survival rates have continued to improve over the past 40 years, with >80% of patients now surviving into adulthood. Fertility preservation (FP) has become an important quality-of-life issue for many survivors of childhood cancer. As a result, the therapeutic options have become less gonadotoxic over time and more patients are being offered FP options. This review examines the indications for consultation, male and female FP options both in the prepubertal patient and adolescent patient, and the unique ethical issues surrounding FP in this vulnerable population. Cancer 2018;124:1867-76. © 2018 American Cancer Society.

Assessing health related benefit after reconstruction for urinary and fecal incontinence in children: a parental perspective.

PURPOSE: We sought to evaluate health related benefit in children undergoing surgical reconstruction for urinary and fecal incontinence from a parental perspective. MATERIALS AND METHODS: A health related benefit instrument was mailed to the parents or guardians of 300 consecutive patients who had undergone reconstruction for urinary and/or fecal incontinence at our institution between 1997 and 2011. We assessed parent reported health related benefit using the validated Glasgow Children's Benefit Inventory and satisfaction with 6 supplemental questions. One-sample t-tests as well as exploratory univariate and multivariate linear regressions were performed for statistical analysis. RESULTS: Response rate was 40.0% at a mean of 5.5 years (range 0.6 to 13.8) after reconstruction. Spina bifida was the most common primary diagnosis (48 patients, 56.5%). Mean total Glasgow Children's Benefit Inventory score and subscores for each domain were positive, indicating an improved health related benefit after reconstruction (all p <0.0001). Certain patients, possibly based on diagnosis and gender, may particularly benefit from reconstruction (p ≤0.04). Parents believed that the bladder augmentation and/or urinary continent catheterizable channel most changed the life of their child but that achievement of stool continence was most important to themselves. Only 17 families (16.2%) required more than 1 month to become comfortable with catheterizations, and 69 patients (68.8%) required less care or no assistance with daily activities after reconstruction. Only 2 parents (1.9%) would be unwilling to consent to the procedure again. CONCLUSIONS: We observed moderate parental satisfaction and parent reported improvement in health related quality of life for children undergoing surgical reconstruction for urinary and fecal incontinence.

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.

Time to therapy and safety of testicular tissue cryopreservation in children undergoing gonadotoxic treatment or hematopoietic stem cell transplant.

BACKGROUND: With the use of multimodal treatments and hematopoietic stem cell transplant, the majority of children diagnosed with malignancies and hematologic diseases are now surviving into adulthood. Due to the gonadotoxic effects and potential for future infertility associated with many of these treatments, fertility counseling with sperm cryopreservation prior to starting therapy is the standard of care for post-pubertal males. Unfortunately, the options are limited for pre-pubertal patients or those unable to provide a specimen. Testicular tissue cryopreservation (TTC) is an investigational method to surgically obtain germ cells from testicular tissue and potentially restore future spermatogenesis. While TTC has been shown to be safe, little is reported on the time to treatment following the procedure to ensure adequate wound healing and avoid delays in definitive therapy. OBJECTIVES: The primary outcome was the time to initiation of treatment following TTC. Secondary outcomes were complication rates, delays in treatment due to TTC, and presence of germ cells. METHODS: We conducted a single-institution retrospective cohort study of patients undergoing TTC between 2017 and 2023. Patients at significant risk for treatment related infertility based on established criteria were eligible for TTC. Patients were excluded if they received their oncology or hematology care elsewhere. All patients were enrolled in an IRB approved research protocol with 75% of the tissue submitted for cryopreservation and 25% for research purposes. Time to therapy was defined as the first receipt of gonadotoxic treatment following TTC. RESULTS: A total of 122 patients (53 = malignant, 69 = non-malignant) underwent TTC with a median age of 5.9 years (IQR 2.3-9.35). Germ cells were identified in 115 (94%) specimens. A total of 109 (89%) patients underwent concomitant procedures. The median time to initiation of therapy was 5 (IQR 1.0-7.0) and 7 days (IQR 6.0-13.0) for malignant and non-malignant disease, respectively. The 30-day surgical complication rate was 2.5% and was similar between malignant vs non-malignant diagnoses (p = 0.58). All surgical complications were managed non-operatively. No patients had a delay in definitive treatment due to concern for wound healing or complications. DISCUSSION: Our surgical complication rates are similar to previous studies and are not affected by the time to treatment following TTC. Limitations of the study are its retrospective design, single institution, and short-term follow up. CONCLUSION: TTC can be performed safely, efficiently, and in conjunction with other necessary procedures without resulting in delays of definitive treatment. TTC affords the opportunity for fertility preservation in children who have no other options.

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.

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.

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.

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.

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.

Inadvertent cryoablation of exophytic upper tract urothelial carcinoma without pelvicalyceal involvement.

Ablative therapy has recently emerged as an option for the treatment of small renal masses (SRMs). Benign tumors and indolent renal cell carcinoma (RCC) represent a majority of these masses, although an additional but often unappreciated consideration is upper tract urothelial carcinoma (UC). We report the case of a 74-year-old man with upper tract UC presenting as a SRM without any apparent involvement of the pelvicalyceal system, leading to its inadvertent cryoablation. We also discuss the role of renal biopsy in the management of SRMs undergoing an ablative procedure.

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.

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.

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.

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.