Prenatal hydrocolpos: imaging findings and differential diagnosis.

Prenatal hydrocolpos is characterized by fluid distension of the vagina. Hydrocolpos can be caused by multiple underlying etiologies and often demonstrates overlapping imaging features compared to other cystic abdominal and pelvic lesions. The purpose of the current pictorial essay is to provide a systematic prenatal magnetic resonance imaging (MRI) approach to differentiating the primary etiologies leading to hydrocolpos. After discussing the fundamental embryological processes involved in vaginal development, the current essay discusses the most common causes of hydrocolpos with their associated prenatal and postnatal imaging features. An approach to distinguishing the more common differential diagnoses is provided. Given the implications of parental counseling and postnatal management, this essay provides an important approach for narrowing differential diagnoses based on prenatal imaging.

Parental decision regret after pediatric urologic surgeries compared to decisions of everyday life.

INTRODUCTION: Parents are at risk of decision regret (DR) for decisions affecting their children. The Decision Regret Scale (DRS) measures medical DR but lacks context outside of healthcare. OBJECTIVE: To compare parental DR 1) between common pediatric urologic surgeries and everyday decisions and 2) with preference to make a different choice. METHODS: We conducted a cross-sectional online survey of randomly selected parents >1year (y) after their children underwent: orchiopexy (males ≤10y), open ureteral reimplant (OUR, females 2-6y), open pyeloplasty (OP, ≤2y), or robotic pyeloplasty (RP, 5-17y) (2017-2021). Higher DRS scores indicate increased DR (none: 0, mild: 1-25, moderate: 30-50, strong: 55-75, very strong: 80-100). Parents completed DRS on four decisions: their child's surgery, most recent/current romantic relationship, most recent leased/purchased car, and most recent purchased meal. Parents reported if they would make the same choice (yes/no). Nonparametric statistics were used. RESULTS: We surveyed 191 parents (orchiopexy n = 52, OUR n = 50, OP n = 51, RP n = 38). The median parent age was 36y (mothers: 86%). Some DR was reported for all decisions, but with significant differences in DR severity. The lowest median DRS score was seen with surgery (orchiopexy 0 [IQR 0-10], OUR 0 [IQR 0-5], OP 0 [IQR 0-0], RP 0 [IQR 0-0]), with no difference between surgery groups (p = 0.78). This was followed by relationship (0, IQR 0-20), car (15, IQR 0-25), and meal (20, IQR 0-30, p < 0.001). Most parents did not report any DR regarding surgery (orchiopexy 69%, OUR 74%, OP 76%, RP 76%, with no difference between surgery groups p = 0.85, Summary Figure). Comparatively, 59% of parents did not have any regret about their relationship, 37% their car, and 28% their meal (p < 0.001). All surgical DR was mild or moderate. No parent (0%) would have chosen differently for their child's surgery versus 4-12% for non-surgical decisions (p < 0.001). Overall, increasing DR corresponded to increasing desire to have made a different choice (DRS≤10: 0%, DRS 45-50: 32%, DRS 55-60: 66%, DRS≥75: 100%, p < 0.001). CONCLUSION: Parental DR varied between urological surgical and non-surgical decisions. It was lowest after surgery. Some regret was reported after every decision, but the subset of parents with regret was smallest after surgical decisions. Positive DRS scores do not necessarily correspond to parents wishing they made a different choice.

Surgical simulation of hypospadias repair - High-fidelity, reproducible and affordable animal tissue model.

INTRODUCTION: Teaching methods in hypospadias repair are still traditional. Available simulators often lack authenticity in terms of haptic feedback and realistic tissue handling. OBJECTIVE: Our aim was to develop a high-fidelity, easily reproducible, and affordable animal tissue model for the advanced surgical simulation of hypospadias repair with realistic haptic feedback and tissue handling. MATERIAL AND METHODS: A regular-sized chicken leg and a lamb tenderloin are used to assess the feasibility of simulating hypospadias correction by using the example of a Snodgrass Tubularized Incised Plate-Repair. The model preparation is incorporated into the training process. RESULTS: A detailed description of the high-fidelity model is provided. All steps of a hypospadias repair can be trained while providing realistic anatomy, adequate size, and multilayer tissue properties. Haptic conditions highly resemble human tissue properties. Fine tissue handling corresponds to intraoperative conditions. Limitations to this surgical model apply as in other animal tissue models. CONCLUSION: We developed a high-fidelity, easily reproducible, and affordable hypospadias animal tissue model. Due to the multilayer animal tissue properties, this model provides realistic haptic feedback and thus an inexpensive and reproducible model for hypospadias simulation. External validation is mandatory prior to implementation into urological training.

People with spina bifida use their MACE on long-term follow-up: A single institutional retrospective cohort study.

OBJECTIVE: While the Malone antegrade continence enema (MACE) facilitates bowel movements in patients with spina bifida (SB) and neuropathic bowel, little is known about its long-term use. We aimed to assess long-term MACE use and potential risk factors for disuse. METHODS: All patients with SB who underwent MACE procedures at our institution were retrospectively reviewed. Main outcome was MACE disuse (no longer catheterizing the MACE for antegrade enemas) based on self-report on a clinic questionnaire, or medical record for patients last seen before introducing the questionnaire 5 years ago. Survival analysis used two timeframes: time after surgery (Analysis 1) and chronological age: accounting for older children reaching adulthood earlier (Analysis 2). RESULTS: Overall, 411 patients (54% female, 78% shunted, 65% augmented) underwent a MACE procedure at median 7.9 years old (median follow-up: 8.4 years). Thirty-three (8%) patients no longer used their MACE. Most common reasons for doing so were channel/stomal stenosis (61%) and excision at colostomy or other abdominal surgery (12%). Bowel management afterwards included oral agents ± enemas (55%), Chait tube (30%), colostomy (12%). After correcting for differential follow-up, 90% of participants used their MACE at 10 years and 87% at 15 years after surgery. Based on chronological age, 97% used their MACE at 15 years old, 92% at 20 and 81% at 30 (Summary Figure). On multivariate analysis, umbilical MACEs were 2.4 times more likely to be disused than right lower quadrant MACEs (p = 0.04). Without correcting for chronological age (Analysis 1), patients undergoing MACE surgery at older ages were more likely to stop MACE use (p = 0.03). However, after accounting for chronological age (Analysis 2), patients undergoing a MACE procedure at older ages were no more likely to stop its use (p = 0.47, Figure). Gender, SB type, shunt status, mobility status, bladder augmentation or a urinary catheterizable channel were not associated with stopping MACE use (p ≥ 0.10). COMMENT: Participants were regularly followed in multi-disciplinary SB clinics. We did not assess continence, satisfaction or long-term urinary channel use, making it premature to recommend optimal stomal locations. CONCLUSIONS: Most patients with SB followed by a multi-disciplinary team continue using their MACE; 1% stopped MACE use annually, particularly after adolescence. This strongly suggests it is an effective bowel management method and transitioning to self-care plays a role in maintaining long-term MACE use. Umbilical MACEs may be at high risk of disuse, but all people with a MACE can benefit from support as they transition to adult care.

Age-based risk of end-stage kidney disease in patients with myelomeningocele.

OBJECTIVE: We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals with myelomeningocele (MMC) followed by urology in the modern medical era and to assess if ESKD risk was higher after surgery related to a hostile bladder. METHODS: We retrospectively reviewed patients with MMC followed by urology at our institution born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of age (when mortality is highest, sometimes before establishing urology care). ESKD was defined as requiring permanent peritoneal/hemodialysis or renal transplantation. Early surgery related to hostile bladder included incontinent vesicostomy, bladder augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy for recurrent urinary tract infections. Survival analysis and proportional hazards regression were used. Sensitivity analyses included: risk factor analysis with only vesicostomy, timing of surgery, including the entire population without minimal follow-up (n = 1054) and only patients with ≥ 5 years of follow-up (n = 925). RESULTS: Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female, 76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis, 1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was 0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old, p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender, birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16). Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had a higher risk of ESKD. No other statistically/clinically significant differences were noted. COMMENT: Patients with MMC remain at risk of progressive renal damage throughout life. We relied on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent documentation of early urodynamic findings and indications for bladder-related surgery. CONCLUSIONS: While ESKD is relatively uncommon in the MMC population receiving routine urological care, affecting 2.1% of individuals in the first 3 decades, it is significantly higher than the general population. Children with poor bladder function are likely at high risk, underlining the need for routine urological care, particularly in adulthood.

Ask the parents: Testing the acceptability and usability of a hypospadias decision aid.

INTRODUCTION: In previous work, we engaged key stakeholders to create a web-based decision aid (DA) prototype to facilitate shared decision making about hypospadias. OBJECTIVE: The study's objective was to use a human-centered design approach to assess the DA's acceptability and usability and revise it prior to pilot testing. METHODS: We recruited English-speaking parents (≥18 years old) of sons with hypospadias (≤5 years) for a two-phase process of semi-structured phone/video interviews to obtain feedback about our DA prototype. DA webpages included: "Hypospadias," "Surgery Basics," "No Surgery," "Family Stories," "Help Me Decide," and "FAQs." In both phases, participants viewed the DA using the "think aloud" technique and completed several validated scales to evaluate its acceptability and usability. In phase 1, we collected feedback about the "Homepage" organization, values clarification methods (VCM), and webpage content. In phase 2, participants searched the DA for answers to hypospadias-related questions, provided feedback on testimonial videos and VCM, and shared their preferences about data visualizations. All interviews were audio recorded. After each phase, transcripts were qualitatively analyzed to identify key areas for revision. Revisions were made between phase 1 and 2 to improve the DA's acceptability and usability. RESULTS: We interviewed 20 participants (10/phase): median age 33.7 years, 60% female, 80% White. Mean score on the Preparation for Decision Making Scale: 86.8 (out of 100). We revised: 1) VCM, focusing on pros/cons of surgery and question prompts, 2) "Homepage," adding webpage descriptions (Extended Summary Figure), 3) menu organization, 4) "Surgery Day" webpage, adding general anesthesia risk information, and 5) "Hypospadias" webpage, adding an icon bar graph to help participants visualize statistics. Participants thought the testimonial videos were relatable and the VCMs would prepare them for their visit with their child's urologist. DISCUSSION: Ours is the first parent-centered DA developed and pre-tested for hypospadias. Using validated usability and acceptability scales, participants highly rated the DA in helping them arrive at a decision about surgery. Study limitations include the sample's lack of diversity (i.e., educated, health literate) and participants already decided about their son's hypospadias management before enrolling. To learn more about the DA's usability and acceptability, we plan to pilot test it in a clinical setting. CONCLUSIONS: Participants found our DA informative in understanding hypospadias. There was a high perceived level of preparation for hypospadias decision making. Participatory research methods, such as "think aloud," may be helpful when testing DAs as they privilege the patient's experience.

Genetics: The diagnostic frontier in pediatric urology.

In the course of human history few scientific breakthroughs can rival the importance of the discovery of DNA. Our ever increasing ability to unravel the secrets contained in this molecule allow new insight in to the etiology and eventual treatment of human conditions ranging from congenital structural disorders to cancer. It is the focus of this article to touch upon sequencing technologies and their potential to provide information that can revolutionize healthcare.

Ultrasound of retained gonads in children and young women with androgen insensitivity syndrome.

INTRODUCTION: Gonadal management in androgen insensitivity syndrome (AIS) patients has been controversial due to low risk of testicular cancer. Our study evaluated the role of ultrasound (US) in screening for malignancy in retained gonads in AIS patients. METHODS: This was a retrospective study (2001-2020) of gonadal US in patients with AIS. Demographics and clinical information were retrieved from the medical records. US studies were reviewed for nodule presence, size, echotexture, and change on follow-up studies. When available, pathology correlation was performed. Two-tailed t-test was used to compare age and development of nodules clinically or on US examination. RESULTS: 13 patients were included with a median age was 9.9 years (range 3.8-18.4 years). In 11 patients, gonads were in the inguinal canals on either initial or follow-up US. No nodules were palpable on physical examination, but nodules were detected in ten testicles by US in five of 13 patients (41.7%). Presence of nodules was significantly (p = 0.0038) associated with older age. The largest nodule size varied from 0.4 to 2.2 cm (average 0.9 ± 0.5 cm) and most (7/10, 70%) were hypoechoic. Finding testicular nodules on US led to change in management in three patients; bilateral gonadectomies, unilateral gonadectomy, and gonadal excisional biopsies (Figure). Pathology demonstrated Sertoli hamartoma in these patients, and in an additional two patients who underwent post-puberty gonadectomy. No malignancy was found in any specimen. DISCUSSION: Preservation of the gonads in children with AIS is associated with low risk for malignant transformation. The role of US surveillance of the gonads is unknown. In our series on 13 patients, most of the visualized 24 gonads (22/24, 91.7%) were localized in the groins in either the first or follow-up US studies. Nodules were detected in ten gonads in five of 13 patients (41.7%). Most of these gonads (8/10) had numerous nodules, most (7/10) were hypoechoic with average diameter of the largest nodule of 0.9 ± 0.5 cm. Pathology in 5 patients demonstrated Sertoli hamartomas in all of the gonads. No malignancy was found. In our series, gonadal nodules led to either gonadectomies or excisional biopsies in three patients. Our study has several limitations, related to the retrospective nature of the study and the small size of our series. CONCLUSION: Multiple testicular nodules were commonly detected by US in AIS patients and were not associated with malignancy. Therefore, we are concerned that US screening can lead to unnecessary excisional biopsies and orchiectomies.

Split appendix catheterizable urinary channels are at no higher risk of undergoing revision compared to channels made with the intact appendix.

OBJECTIVE: To assess long-term APV and split-appendix MACE durability and to compare split and intact appendix APVs in a large patient cohort. METHODS: This retrospective cohort study included consecutive patients ≤21 years old undergoing an APV at our institution (1990-2019). Main outcomes were stomal and subfascial revisions. Kaplan Meier survival and Cox proportional hazards analysis were used. RESULTS: A total of 339 patients underwent APV creation at a median 7.4 years old (41% female vs. 59% male; 37% umbilical stoma vs. 63% other). In total, 36 patients underwent a stomal revision and 19 a subfascial revision (median channel follow-up 6.3 years). On survival analysis, the risk of stomal revision of the APV was 9.1% at 5 years, 12.6% at 10 years and 16.5% at 15 years. Risk of subfascial revision of the APV was 5.1% at 5 years, 7.0% at 10 years and 8.2% at 15 years. A split-appendix APV was performed in 118 (34.8%) of 339 patients. They had a shorter follow-up compared to those with an intact APV (5.1 vs. 7.0 years, p = 0.03). After correcting for differential follow-up time, there was no significant difference between groups for stomal revisions (HR 1.11, p = 0.76) or subfascial revisions (HR 0.80, p = 0.67, Figure). Risk of APV stomal revision was independent of stomal location and age at surgery (p ≥ 0.37). Similarly, risk of subfascial APV revision was independent of stomal location and age at surgery (p ≥ 0.18). Risk of stomal revision for split-appendix MACE channels was 16.2% at 5, 10 and 15 years (similar to split-appendix APV and all APVs, p ≥ 0.26). Risk of MACE subfascial revision was 5.5% at 5 years, 5.5% at 10 years and 14.7% at 15 years (similar to split-appendix APV and all APVs, p ≥ 0.36). COMMENT: We focused on surgical complications, as these entail the highest morbidity, however, we did not assess non-surgical, percutaneous or endoscopic management which also impact long-term outcome and patient quality of life. We did not compare the outcomes of the split-appendix MACE to an intact-appendix MACE cohort, as this patient population was not captured in this review. CONCLUSIONS: The split-appendix technique has durable long-term results for both the APV and MACE channels, which are comparable to the technique utilizing the intact appendix. Channel complications occur over the channel's lifetime, as 1 in 8 APVs in the entire cohort underwent a stomal revision and 1 in 14 APVs underwent a subfascial revision at 10 years after surgery.

Incidence of nephrolithiasis after bladder augmentation in people with spina bifida.

INTRODUCTION: Risks of nephrolithiasis after bladder augmentation in people with spina bifida (SB) remain unclear. Annual incidence of nephrolithiasis in the general population is 0.01% for 10-14 years old, 0.07% for 15-19 years old and 0.2% for 20-24 years old. Our aim was to assess the incidence and risk factors of nephrolithiasis in SB patients after augmentation. METHODS: Patients with SB and augmentation followed at our institution were retrospectively reviewed (born ≥1972, surgery 1979-2019). Patients were screened annually with renal bladder ultrasound and abdominal radiograph. Main outcome was nephrolithiasis treatment. Kaplan-Meier survival and Cox proportional hazards analysis were used. Possible predictors were assessed using stepwise forward selection (variables with p < 0.1 on univariate analysis included in multivariate analysis). RESULTS: 427 patients with SB and augmentation were included (51.8% female, 74.9% shunted). Median age at augmentation was 8.5 years (median follow-up: 12.4 years, ileum segment: 81.0%, bladder neck procedure: 60.7%, urinary channel: 74.2%) and 28.8% developed bladder stones. Overall, 47 (11.0%) patients were treated for nephrolithiasis. After correction for differential follow-up, nephrolithiasis was treated in 7.3% at 10 years, 13.2% at 15 years, and 18.0% at 20 years (Figure). Patients presented with either a urinary tract infection (46.8%), on screening (44.7%), or pain (8.5%). Stones were treated percutaneously, endoscopically or by ESWL (63.8%/34.0%/10.7%, respectively). Most were calcium stones (58.3%). On multivariate analysis, compared to younger patients, patients augmented at ≥10 years of age had 1.84 times the risk of nephrolithiasis (p = 0.01). Nephrolithiasis was more common in those who developed bladder stones (HR = 3.00, p < 0.0001). Among those with both renal and bladder stones, bladder stones typically preceded nephrolithiasis (55.2%), were treated concurrently (31.0%) and 13.8% occurred after nephrolithiasis. Gender, wheelchair use, bowel segment used, MACE and skeletal fractures were not associated with higher nephrolithiasis risk (p ≥ 0.11). DISCUSSION: This study of a large cohort of SB patients with long-term follow-up highlights that the risk of nephrolithiasis is cumulative and related to bladder stone formation, age at augmentation and time since augmentation. An association with bladder stones suggests potential shared metabolic causes. The study's retrospective design likely led to underestimating the risk of nephrolithiasis by not capturing spontaneously passed stones. CONCLUSION: Approximately 1% of patients with SB develop nephrolithiasis annually after augmentation. Close long-term surveillance after augmentation is strongly indicated, as nephrolithiasis incidence in augmented patient with SB is at least 10 times higher than general population. Patients with bladder stones are especially at risk.

Provider perspectives on shared decision-making regarding hypospadias surgery.

INTRODUCTION: Many parents experience decisional conflict and decisional regret around hypospadias surgery. The utilization of a shared decision-making (SDM) process may mitigate these issues, however addressing the principal components of the SDM process is a complex task that requires the investment of providers. OBJECTIVE: The purpose of this study was to facilitate a discussion about SDM anchored on hypospadias with pediatric urology and general pediatric providers to explore perspectives, clinical applications and barriers to adopting SDM in clinical practice. STUDY DESIGN: We conducted two focus groups in order to engage pediatric urology and general pediatric providers in guided discussions about SDM anchored on hypospadias. All activities were audio recorded and professionally transcribed. The transcripts were analyzed by three coders using directed qualitative content analysis techniques to identify themes and relationships between themes to inform the development of an affinity diagram (Extended Summary Figure). RESULTS: Two focus groups were held; one with seven pediatric urology providers in November 2018 and one with ten general pediatric providers in January 2019 (median age 51 years, 88.2% Caucasian, 58.8% female, 70.6% physicians and 29.4% nurse practitioners). Both groups identified some of the key components of SDM including engaging families in decision-making, informing them about treatment options and clarifying values/preferences (Extended Summary Figure). They thought that SDM was useful for discussing preference-sensitive conditions (e.g. hypospadias) and addressing parental compliance. General pediatric providers also suggested that SDM helped them avoid unnecessary referrals to specialists. Both groups identified parental, provider and systemic barriers to the adoption of SDM: a) desire for paternalism, b) misperceptions about medical evidence, c) completion of parental decision-making prior to the clinical visit, d) provider bias/lack of interest and e) time constraints/productivity pressures. DISCUSSION: Providers who care for hypospadias patients are knowledgeable about SDM and its potential clinical applications. They identified several potentially modifiable barriers to the adoption of a SDM process about hypospadias surgery in a pediatric clinical setting. CONCLUSIONS: Based on feedback from providers, we plan to implement a hypospadias decision aid early in the parental decision-making process about hypospadias such as in the postpartum unit and at well-child visits in the newborn period and provide a provider training session about SDM to address the identified knowledge gaps.

Additional Surgeries after Bladder Augmentation in Patients with Spina Bifida in the 21st Century.

PURPOSE: We determined the long-term risks of additional surgery after bladder augmentation in a modern spina bifida cohort accounting for differential followup. MATERIALS AND METHODS: We retrospectively reviewed patients with spina bifida who were born after 1972 and were followed at our institution after augmentation surgery performed between 1979 and 2018. Outcomes included diversion, bladder stones, perforation, reaugmentation, laparotomy for bowel obstruction, and benign and malignant bladder tumors. Survival analysis was used for the entire cohort and the modern cohort (detubularized and reconfigured ileocystoplasty beginning in 2000). RESULTS: A total of 413 patients were included in the study. At a median followup of 11.2 years 80.9% of the patients had undergone ileocystoplasty and 44.1% had undergone 370 additional surgeries. Ten-year risk of any reoperation was 43.9%, with 17.4% of patients undergoing 2 or more and 9.9% undergoing 3 or more additional surgeries. Outcomes included conversion to a diversion (2.7% at 10-year followup) and bladder stones (28.2% with recurrence in 52.4%) irrespective of detubularized reconfigured status (p ≥0.20). Bladder perforation risk was 9.6% for patients undergoing vs 23.7% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.01). Similarly reaugmentation rate was 5.3% for patients undergoing vs 15.2% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.001). Finally, 10-year reperforation risk was 32.1% for patients undergoing vs 73.8% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.053). Other risks included bowel obstruction (4.5% with recurrence in 15.8%), nephrogenic adenoma (2.2% with regrowth in 48.2%) and malignancy (0.0% at 20 years). For 222 patients in the modern cohort (median followup 9.1 years) 10-year risk of any reoperation was 46.0%, which consisted of diversion in 4.0%, stones in 32.9% (recurrence in 44.5%), perforation in 8.8% (recurrence in 42.2%), reaugmentation in 4.3%, obstruction in 4.9% (recurrence in 10.0%), adenoma in 4.7% (regrowth in 40.0%) and cancer in 0.0%. CONCLUSIONS: Bladder augmentation is long-lasting. While benefiting continence and renal outcomes, this operation frequently requires additional surgeries, necessitating close followup. Since survival analysis based risks of alternative management options such as incontinent diversion are unavailable, comparisons with augmentation are unfeasible.

Risk of Reaugmentation after Enterocystoplasty Using a Reconfigured Bowel Segment in Patients with Spina Bifida: A Bi-Institutional Cohort Study.

PURPOSE: Reaugmentation cystoplasty rates vary in the literature but have been reported as high as 15%. It is likely that bladders augmented with detubularized and reconfigured bowel are less likely to require reaugmentation. We assessed the incidence of reaugmentation among patients with spina bifida at 2 high volume reconstruction centers. MATERIALS AND METHODS: We retrospectively reviewed medical records of patients with spina bifida who underwent enterocystoplasty before age 21 years (1987 to 2017). Those who did not undergo augmentation with a detubularized and reconfigured bowel segment were excluded from analysis. Data on demographic and surgical variables were collected. Reaugmentation was the main outcome. One analysis was performed using the entire cohort and another analysis was restricted to patients with ileocystoplasty performed in the last 15 years (2002 to 2017). Survival analysis was used. RESULTS: A total of 289 patients were identified. Enterocystoplasty was performed in patients at a median age of 8.1 years (median followup 11.3, IQR 5.2-14.9). Most initial augmentations were performed using ileum (93.4%), followed by sigmoid (6.2%). Seven patients underwent reaugmentation, including 6 with initial augmentation using ileum and 1 with initial augmentation using sigmoid. On survival analysis risk of reaugmentation was 1.1% at 5 years and 3.3% at 10 years after the original surgery. All reaugmentations occurred within the first 9 years of followup. In the more contemporary cohort (162, median followup 7.0 years) only 1 patient underwent reaugmentation at 2.0 years. CONCLUSIONS: The risk of reaugmentation after enterocystoplasty with a detubularized and reconfigured bowel in the spina bifida population is lower than that reported in initial series.

Validation and Preliminary Results of the Parental Assessment of Children's External Genitalia Scale for Females (PACE-F) for Girls With Congenital Adrenal Hyperplasia.

OBJECTIVE: To validate a parental assessment of children's external genitalia scale for females (PACE-F) for girls with congenital adrenal hyperplasia (CAH) by adapting the validated adult female genital self-image scale. METHODS: PACE-F was administered to parents of girls (Tanner 1, 2 months-12 years) with and without CAH. Final questions were determined by clinical relevance and psychometric properties (scores: 0-100). A reference range was established using 95% confidence interval among controls. Age-matched controls were compared to girls with CAH (1) <4 years old before and after female genital reconstruction surgery (FGRS), and (2) 4-12-year olds after FGRS. Nonparametric statistics were used. RESULTS: Participants included 56 parents of 41 girls with CAH (median 3.9 years old, 97.6% FGRS) and 139 parents of 130 girls without CAH. Face and content validity was established by families, experts, and factor analysis. Internal consistency was high (Cronbach's alpha: 0.83). Population reference score range was 66.7-100. Ten consecutive girls had pre- and post-FGRS PACE-F scores. All scores improved at 4 months after surgery and all preoperative scores were below reference range and lower than controls (P = .0001). All postoperative scores were within reference range, no different from controls (P = .18). Scores for girls with CAH after FGRS aged 4-12 years were no different from controls (100.0 vs 88.9, P = .77) and 90.0% were in reference range, as expected (P = .99). CONCLUSION: We present a validated instrument for parental assessment of genital appearance in girls with CAH. We demonstrate improved parent-reported appearance after FGRS, with scores similar to age-matched controls.

Radiographic abnormalities, bladder interventions, and bladder surgery in the first decade of life in children with spina bifida.

BACKGROUND: Spina bifida (SB) patients are at increased risk for hydronephrosis, bladder storage and emptying problems, and renal failure that may require multiple bladder surgeries. METHODS: We retrospectively reviewed patients born with SB 2005-2009, presenting to our institution within 1 year of birth. Outcomes at 8-11 years old included final renal/bladder ultrasound (RBUS) results, clean intermittent catheterization (CIC) use, anticholinergic use, surgical interventions, and final renal function. We excluded those without follow-up past age 8 and/or no RBUS or fluoroscopic urodynamic images (FUI) within the first year of life. Imaging was independently reviewed by four pediatric urologists blinded to radiologists' interpretation and initial findings compared with final outcomes. RESULTS: Of 98 children, 62 met inclusion criteria (48% male, 76% shunted). Median age at last follow-up was 9.6 years. Upon initial imaging, 74% had hydronephrosis (≥ SFU grade 1), decreasing to 5% at 10 years (p < 0.0001). Initially, 9% had ≥ SFU grade 3 hydronephrosis, decreasing to 2% (p = 0.13). CIC and anticholinergic use increased from 61% and 37% to 87% and 86%, respectively (p = 0.001 and p < 0.0001, respectively). With follow-up, 55% had surgical intervention and 23% had an augmentation. Of children with a serum creatinine/cystatin-C at 8-11 years old, one had confirmed chronic kidney disease (stage 2). CONCLUSIONS: Despite initial high incidence of hydronephrosis, this was low grade and resolved in the first decade of life. Additionally, the 8-11-year incidence of kidney disease and upper tract changes was low due to aggressive medical management.

How the ESPU grades clinical abstracts.

The ability to consistently review abstracts in an unbiased and objective fashion is a skill that most academics hope to master. However, robust standardized rating systems are sparse, with most scientific boards leaving the task of rating abstracts poorly defined and at the whim of the reviewer. In an effort to bring consistency to this process, in 2013, the ESPU board adopted an abstract rating system that has been previously used in the field of plastic surgery and orthopedics. (van der Steen et al., 2004; Poolman et al., 2007). The aim of this manuscript is to outline this practice.

Characteristics of testicular tumors in prepubertal children (age 5-12 years).

INTRODUCTION: Testicular tumors in children have two peaks with different types of tumors; in the first 4 years of life a third to half are benign with increased risk of malignancy during puberty. The pathology of testicular tumors between these peaks, at the age of 5-12 years, is not known. We hypothesized that because of the low level of testosterone at this time, the incidence of malignant tumors is very low. OBJECTIVE: To compare malignancy risk of primary testicular tumors in children in the prepubertal period (5-12 years) compared with younger (0-4 years) and pubertal (13-18 years) children. STUDY DESIGN: We retrospectively (2002-2016) identified patients <18 years with surgery for primary testicular tumor. Patients with testicular tumor risk were excluded. Ultrasound studies were reviewed for contralateral testis volume, tumor morphology, and tumor maximal diameter, for three age groups: 0-4, 5-12, and 13-18 years. The Freeman-Halton extension of the Fisher exact probability test was adopted for categorical outcomes, and one-way ANOVA for continuous outcomes. RESULTS: Fifty-two patients (mean age 11.0 years, range 6 days-18 years) were identified. Malignant tumor prevalence significantly differed (p < 0.01) among age groups (Fig).: 0-4 (72.7%, 8/11), 5-12 (0%, 0/16), and 13-18 years (44.0%, 11/25). The most common tumor types in 5-12 years were epidermoid cyst (31.3%, 5/16) and tumor mimics (37.5%, 6/16). Prevalence of cystic tumors in 5-12 year olds was not significantly different compared with other age groups. Contralateral testicular volume >4 mL (pubertal surge) significantly (p < 0.01) differed among groups: 0-4 years (0/11), 5-12 years (3/16), and 13-18 years (19/20). In children aged 13-18 years the mean tumor maximal diameter (29.8 ± 4.4 mm) was significantly larger (p < 0.01) compared with children 5-12 years (9.3 ± 5.5 mm) and all malignant tumors had contralateral testicular volume >4 mL. DISCUSSION: We found that preadolescent children between the ages of 5 and 12 years have distinctive characteristics compared with the other age groups. Most importantly, no malignant testicular tumors were found in this age group. About a third of the children presented with an incidental testicular mass. The testicular tumors were significantly smaller (9.3 ± 6.7 mm) compared with those in children aged 13-18 years (29.8 ± 4.4 mm). There were limitations because of the retrospective nature of the study. CONCLUSION: We found no malignant testicular tumors in children aged 5-12 years with no risk factors and prior to pubertal surge. Our study suggests use of more conservative treatment in this group of patients.

Parental decisional regret and views about optimal timing of female genital restoration surgery in congenital adrenal hyperplasia.

PURPOSE: The role of female genital restoration surgery (FGRS) in girls with congenital adrenal hyperplasia (CAH) is controversial, with no long-term parent-reported outcomes available. Decisional regret (DR) affects most parents after their children's treatment of pediatric conditions, including hypospadias. We aimed to assess parental DR after FGRS in infancy or toddlerhood and explore optimal timing for surgery. MATERIALS AND METHODS: One-hundred and six parents of females with CAH undergoing FGRS before 3 years old and followed at our institution (1999-2017) were invited to enroll online. Higher Decision Regret Scale (DRS) scores indicated greater DR (range 0-100). Participants also reported preferred FGRS timing relative to their surgery (earlier, same, later/delayed). Non-parametric statistical tests were used. RESULTS: Thirty-nine parents (median 4.4 years after FGRS) participated (36.8% response rate). Median age at FGRS was 9 months. Median DRS score was 0 (mean: 5.0). Overall, 20.5% of parents reported some regret (all mild-moderate) (Figure). Fewer parents reported DR after FGRS compared with published DR after hypospadias repair (50-92%, p ≤ 0.001) or adenotonsillectomy (41-45%, p ≤ 0.03). No parent preferred delayed FGRS. Seven parents (18.1%) preferred earlier surgery, especially when performed after birthday (80.0% vs. 8.8%, p = 0.004). DISCUSSION: We present the first report of validated long-term parent-reported outcomes after FGRS in infant and toddler girls with CAH. One limitation is that this is largely a single surgeon series. Reasons for the observed low levels of DR are likely multifactorial. Far from a definitive study, we aimed to provide parents willing to share about their experience an opportunity to do so. For that reason, selection bias may exist in our study. While parents with higher DR were potentially less likely to participate because of mistrust of the medical establishment, those with a negative experience may in fact be more likely to voice their opinions. A low participation rate was likely a result of the sensitive nature of FGRS, a desire for privacy, and inability to locate parents. A larger study will be required to assess how DR is affected by sexual function, genital appearance and complications, and DR among women with CAH. CONCLUSIONS: Parents of females with CAH report low levels of DR after FGRS in infancy and toddlerhood. This appears to be lower than after other genital and non-genital pediatric procedures. When present, parental DR is usually mild. No parents preferred delayed surgery, even among those with DR. Some preferred earlier surgery.

Complications and Outcomes of Pregnancy and Cesarean Delivery in Women With Neuropathic Bladder and Lower Urinary Tract Reconstruction.

OBJECTIVE: To determine the outcomes of pregnancy and cesarean delivery (CD) in women with neuropathic bladder (NB) and pediatric lower urinary tract reconstruction (LUTR) as these women often have normal fertility and may become pregnant. METHODS: We reviewed consecutive patients with NB due to spinal dysraphism who underwent LUTR, became pregnant, and had a CD at our institution from July 2001 to June 2016. We collected data on demographics, hydronephrosis, symptomatic urinary tract infection, continence, and catheterization during pregnancy. CD data included gestational age, abdominal or uterine incisions, and complications. RESULTS: We identified 18 pregnancies in 11 women. Fifteen live newborns were delivered via CD (53.3% term births). Thirteen of 15 patients (86.7%) developed new (10) or worsening (3) hydronephrosis. Six of 13 patients (46.2%) underwent nephrostomy tube placement. Eight of 15 patients (53.3%) developed difficulty catheterizing (66.7% via native urethra, 44.4% via catheterizable channel); 50.0% of patients required an indwelling catheter. Five of 15 patients (33.3%) developed urinary incontinence during pregnancy. Ten of 15 patients (66.7%) had a urinary tract infection (30.0% febrile). A urologist was present for all CDs: 5 were scheduled, 10 occurred emergently. Complications occurred in 40.0% (5 cystotomies, 1 bowel deserosalization, 1 vaginal laceration). All cystotomies occurred during emergent CD. Three patients (20.0%) developed urinary fistulae after emergent CD. CONCLUSIONS: Women with NB and LUTR have high rates of complications during pregnancy and CD, despite routine involvement of urologists. Women with prolonged labor, previous CD, or those with a history of noncompliance developed the worst complications. Based on our experience, a urologist should always be present and participate in the CD.