Why are pediatric urologists unable to predict renal deterioration using urodynamics? A focused narrative review of the shortcomings of the literature.

In this focused narrative review we set out to review the current literature addressing the utilization of UDS in patients with spina bifida (SB). We specifically analyzed 6 urodynamic parameters and their roles as predictors of upper tract deterioration in pediatric SB patients. The material available did not allow a systematic analysis or the usage of metanalysis methodology, due to the predominance of small retrospective cohorts, and high heterogeneity. We identified 10 retrospective chart reviews that met our study criteria. The results of each of these papers, as well as other studies deemed relevant to the discussion, are included in our narrative review of the literature. We summarize the current literature, offer explanations for divergences in opinion, and identify future research directions and emerging solutions with a focus on machine learning.

Uncharted territory: navigating the pediatric urology job market.

INTRODUCTION: There is not a structured algorithm, timeline, or resource on how, when, and which programs are looking for a new staff pediatric urologist. We hypothesized that current and future pediatric urology fellows could benefit from the experiences of recently graduated fellows on navigating the urology job market. OBJECTIVE: The purpose of this study was to survey current and recent pediatric urology fellowship graduates about the process of finding their first job. STUDY DESIGN: A Research Electronic Data Capture (REDCap) online survey was designed and distributed through email to current fellows and 2012-2017 graduates. Results were evaluated using STATA. RESULTS: 153 participants were emailed, and 94 (61%) completed the survey. The plurality of applicants (44%) began the job search at the Spring American Urological Association meeting, 14 months before finishing (Figure). Of those who started 14 months before finishing, 33% wished they started earlier and 61% would have started at the same point. The median number of programs contacted for a position was 3-4 and the median number of programs visited and offers received was two. After the offer, 40% reported having >8 weeks to decide. Less than half (38.5%) hired an attorney for contract negotiation. Of those who hired an attorney, 68% felt it provided benefit. Regarding contract negotiation, 22% did not negotiate and 35% negotiated for salary. Unsurprisingly, 28% of those who took academic jobs thought negotiating for protected research/educational time was most important compared with only 4% of those who took non-academic jobs (P = 0.02). When asked how they learned about the job they accepted: 28% were contacted by the program, 25% cold called the program, 30% accepted where they did residency or fellowship, and 18% learned through society websites. The plurality (50%) thought the number of desirable positions during the process were as they expected. 41% however, thought the number of desirable positions were expectedly or surprisingly low. Regarding quality of life and satisfaction with job/career choice, 98% stated that they would still choose to subspecialize in pediatric urology. CONCLUSIONS: The results from this survey should provide guidance to fellows on how to approach the job search with respect to timing, expectations, contract negotiation, and initial job satisfaction.

Ipsilateral ureteroureterostomy: does function of the obstructed moiety matter?

PURPOSE: Upper pole nephrectomy has been the traditional surgical management of children with poorly functioning upper pole moieties in duplex renal collecting systems having ureteral ectopia and ureterocele. However, ablative surgery confers a risk of functional loss to the remnant moiety due to vasospasm or vascular injury. It was hypothesized that ipsilateral ureteroureterostomy (IUU) is a safe and feasible approach for the management of these patients and that residual function in the obstructed upper pole does not affect surgical outcomes. MATERIALS AND METHODS: All patients with duplex systems who underwent IUU between 2010 and 2016 were retrospectively reviewed. Patients were sorted into two groups based on pre-operative imaging: those having <10% upper pole moiety function (UPMF) and those having ≥ 10% UPMF. Outcomes assessed were postoperative complications (Clavien-Dindo classification), need for secondary surgery, and radiological outcomes. RESULTS: The study cohort comprised 53 children with ectopia or ureterocele affecting the upper pole in a duplex system, 21 with UPMF <10% (median function 0% and median age 1.49 years) and 32 with UPMF ≥ 10% (median function 15% and median age 0.91 years). Median follow-up was 27.4 months and 27.6 months. In both the groups, prenatal hydronephrosis was the most common presentation (57% and 56%, respectively; p = 0.18) followed by urinary tract infection. Mann-Whitney U test comparing the two groups revealed no significant differences in any of the outcomes assessed. No patient required secondary surgery. CONCLUSION: Ipsilateral ureteroureterostomy is a safe, definitive surgical intervention that preserves the renal architecture in children with duplex collecting systems regardless of upper pole function.

Pediatric robotic-assisted laparoscopic pyeloplasty: Does age matter?

BACKGROUND: Although shown to be safe in infancy, robotic-assisted laparoscopic pyeloplasty (RALP) for ureteropelvic junction obstruction (UPJO) is most commonly performed in older children and adolescents. OBJECTIVE: This study examined a contemporary RALP experience at a single tertiary pediatric center and compared outcomes in infants aged ≤1 year with an older cohort. METHODS AND DESIGN: All RALP procedures were entered into an Institutional Review Board-approved data registry from 2012 to 2016. Patients were retrospectively grouped according to age. The primary outcome was success rate. Secondary outcomes included complications and length of hospital stay. Failure was defined as the need for secondary surgical intervention for UPJO or worsening urinary tract dilation on imaging. Statistical analysis was performed using SPSS version 20. Mann-Whitney U testing was used for comparison. RESULTS: A total of 138 patients underwent RALP during 2012-2016, with a median age of 6 years (IQR 1, 13.25) and a male:female ratio of approximately 2:1. Of these, 34 (24.6%) were aged ≤1 year. Of all patients, 60 (43.5%) presented with a history of prenatal hydronephrosis, and 32% had a crossing vessel causing obstruction. An indwelling stent was placed in antegrade fashion in 71% of cases, and 18% had a percutaneously placed externalized stent. There were six (4%) failures requiring reoperation. Multivariate and comparative analysis demonstrated that the infant cohort utilized less morphine equivalents and more often had a percutaneous stent placed compared to the older cohort. Of the complications that occurred, 60% were minor (Clavien grades 1 and 2) and 40% were Clavien grade 3 in the infant cohort, and 70.1% and 29.9% in the older cohort, respectively. No studied criteria predicted failure in either cohort. CONCLUSION: This study presented one of the largest contemporary series of consecutive pediatric RALPs, and showed an overall success rate of 96%. There were no significant differences in length of hospital stay, and complications or failure rates in infants compared to older children. This study substantiated the ongoing trend towards the adaptation of robotic-assisted surgery for the entire pediatric patient population.

Outcomes of externalized pyeloureteral versus internal ureteral stent in pediatric robotic-assisted laparoscopic pyeloplasty.

INTRODUCTION: After pyeloplasty, urinary drainage options include internal double-J (DJ) ureteral stents or externalized pyeloureteral (EPU) stents, which can avoid bladder symptoms and additional anesthetic exposure from stent removal. Comparative outcome studies, however, are lacking following primary pediatric robotic-assisted laparoscopic pyeloplasty (RALP). OBJECTIVE: To compare operative success, operative time, hospitalization, and postoperative complications of EPU versus DJ stents following RALP. STUDY DESIGN: Consecutive children undergoing primary RALP from 10/2013 to 9/2015 were retrospectively identified. Data collected included patient demographics, stent type and duration, postoperative complications, and operative success. To control for confounding by indication for EPU stent, propensity score weighting was used to balance baseline covariates. Weighted regression analyses compared between-group differences in study outcomes. RESULTS: At median follow-up of 12.3 months, 44 and 17 patients underwent DJ and EPU stenting, respectively. At baseline, DJ stent patients were older than EPU stent patients (median 7.7 vs 1.2 years, P = 0.01) and were less likely to be on postoperative antibiotic prophylaxis (25 vs 76%, P < 0.001). After weighting, these differences disappeared. All EPU stents were removed in the outpatient clinic; all DJ stents were removed under anesthesia. On weighted regression analyses (Summary Fig.), EPU stents had no different associations than DJ stents with operative success (95 vs 94%, between-group difference 1%, 95% CI -11, 13; P = 0.86), complications, or operative time, but did have 0.6 of a day more hospitalization (95% CI 0.04, 1.2; P = 0.04). DISCUSSION: Patients receiving EPU stents were different at baseline from those receiving DJ stents. After propensity score weighting balanced these covariates, EPU stents were associated with similar operative success, complications, and operative time to DJ stents. Further study is warranted in larger prospective cohorts. CONCLUSION: Use of EPU stents provided a viable alternative, particularly in younger patients, to DJ stenting with comparable success and complications, while avoiding the need for an additional anesthetic.

Tunica vaginalis flap for salvaging testicular torsion: A matched cohort analysis.

INTRODUCTION: In testicular torsion, ischemia time from pain onset impacts testicular salvage. A tunica albuginea fasciotomy to relieve compartment pressure followed by a tunica vaginalis flap (TVF) may enhance salvage. OBJECTIVE: To define the optimal window of ischemia time during which TVF may be most beneficial to avoid orchiectomy. STUDY DESIGN: A retrospective cohort study of males presenting with testicular torsion at a single tertiary-care institution from January, 2003 to March, 2017. Ischemia time was defined as duration of pain from onset to surgery. Because TVF would be an option to orchiectomy, and it was found that ischemia time was longer in testicles that underwent orchiectomy, matching was performed. Cases of torsion treated with TVF were matched 1:1 with cases treated with orchiectomy on age at surgery, and ischemia time. Outcomes included postoperative viability, defined as palpable testicular tissue with normal consistency, and atrophy, defined as palpable decrease in size relative to contralateral testicle. Sensitivity analyses were performed restricting to the subgroups with postoperative ultrasound, >6 months' follow-up, and additionally matching for degrees of twist. RESULTS: A total of 182 patients met eligibility criteria, of whom 49, 36, and 97 underwent orchiectomy, TVF, and septopexy alone, respectively. Median follow-up was 2.7 months; 26% of patients had postoperative ultrasound (61% of TVF group). In the orchiectomy, TVF, and septopexy groups, respectively, median ischemia times were 51, 11, and 8 h, postoperative viability rates were 0, 86, and 95%, and postoperative atrophy rates were 0, 68, and 24%. After matching, 32 patients with TVF were matched to 32 patients who underwent orchiectomy. In the TVF group, postoperative viability occurred in 95% (19/20) vs 67% (8/12) of patients with ischemia times ≤24 and >24 h, respectively. Atrophy occurred in 67% (12/18) vs 83% (10/12) of these same respective patients. Sensitivity analysis by ultrasound and longer follow-up found similar viability results, although atrophy rates were higher. Additional matching for degrees of twist showed lower viability and higher atrophy rates for increasing ischemia times. DISCUSSION: Patients who presented with testicular torsion with ischemia times ≤24 h and who were being considered for orchiectomy may have benefitted most from TVF, albeit at high risk of atrophy. However, for ischemia times >24 h, TVF may still have preserved testicular viability in two-thirds of cases. A limitation was short follow-up. CONCLUSION: A TVF was a valid alternative to orchiectomy for torsed testicles, albeit with high testicular atrophy rates.

Genitourinary involvement and management in children with Stevens-Johnson syndrome and toxic epidermal necrolysis.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are devastating hypersensitivity disorders that cause epidermal cell death and can affect all epidermal surfaces, including the urethra, vagina, labial and scrotal skin. Despite the well-described ocular and orofacial manifestations of SJS/TEN, there is a paucity of reports on the genitourinary (GU) symptoms and their management. Specifically, consulting services often ask the pediatric urology team if it is safe to place a urethral catheter, but there is no data in the literature to help guide management. The present study sought to review all pediatric cases of SJS/TEN in a tertiary care hospital to determine the incidence and optimal management of GU manifestations, including the use of urethral catheters. METHODS: With IRB approval, cases of SJS and TEN that were managed as an inpatient between January 2008 and June 2015 were retrospectively reviewed in order to identify the extent of GU involvement/manifestations, the treatment provided, use of urethral catheterization and long-term follow-up or complications. RESULTS: Thirty-one patients (15 female, 16 male; age range 2-18 years) presented with SJS or TEN over the study period. Etiologies for SJS/TEN included mycoplasma infection (48%) and medications (45%). Incidences of GU manifestations at presentation and their management are shown in Summary Table. Overall, 74% of patients had genital involvement of skin lesions. In 12 cases (39%), urology consultation was obtained. Twenty patients (61%) complained of dysuria and one child had gross hematuria in the setting of meatal lesion. Petroleum jelly was used in the majority of patients. A urethral catheter was placed in eight patients (25.8%, four female, four male) with a range of duration of 7-23 days. No patient developed hematuria or any other complications (i.e. strictures or urinary symptoms) after catheter removal. One boy required lysis of penile adhesions in the short-term. One of each gender developed penile and labial adhesions on long-term follow-up that self-resolved. CONCLUSIONS: GU involvement in SJS/TEN occurred in almost three-quarters of patients and was managed conservatively like other skin/mucosal manifestations. Long-term sequelae were rare and urethral catheterization appeared to be safe, without any short-term or long-term complications.

Fertility in disorders of sex development: A review.

INTRODUCTION: Disorders of sex development (DSD) are a heterogeneous group of complex conditions that can affect chromosomal, gonadal, and/or phenotypical sex. In addition to impacts on internal and external genitalia,these conditions can affect fertility potentialto various degrees. In this review we discuss fertility issues including gonadalpreservation and reproductive outcomes based on specific DSD conditions. METHODS AND MATERIALS: A systematic literature review was performed on Embase™, PubMed®, and Google Scholar™ for disordersof sex development and infertility. Original research articles and relevant reviews were examinedand a synopsis of these data was generated for a comprehensive review of fertility potential in disorders of sex development. RESULTS: While patients with some DSDs may have functioning gonads with viable germ cells but an inability to achieve natural fertility secondary to incongruent internal or external genitalia, other patients may have phenotypically normal genitalia but infertility due to abnormal gonad development. Fertility rates in females with congenital adrenal hyperplasia (CAH) depend on phenotype and are inversely proportionalto the severity of the disease. Men with classic CAH have reduced fertility and due to the presence of testicular adrenal rest tumors and to suppression of the hypothalamic-pituitary-gonadal axis by high systemic levels of androgens. Infertility is seen in complete androgen insensitivity and subfertility is common in partial cases. Fertility is rare in pure or mixed gonadal dysgenesis, ovotesticular disorder, Klinefelter syndrome, and XX males. CONCLUSION: Fertility potential appears to be the highest in patientswith XX or XY CAH, especially non-classic forms. Advancements in assisted reproduction techniques has in rare cases produced offspring in some diagnoses thought to be universally infertile. Discussion of fertility issues with the patient and family is essential to the optimal treatment of each patient and an important part of the multi-disciplinary approach to evaluating and counseling these families.

Use of non-invasive uroflowmetry with simultaneous electromyography to monitor patient response to treatment for lower urinary tract conditions.

OBJECTIVE: Non-invasive uroflowmetry with simultaneous electromyography (uroflow/EMG) has previously been reported as effective in triaging patients into four specific non-neurogenic lower urinary tract (LUT) conditions for targeted treatment. In this study we sought to determine if the same parameters would be useful for measuring response to treatment. MATERIAL AND METHODS: We reviewed our database of normal children with LUT dysfunction, screened with uroflow/EMG, and diagnosed with a LUT condition: (1) dysfunctional voiding (DV); (2) idiopathic detrusor overactivity disorder (IDOD); (3) detrusor underutilization disorder (DUD); (4) primary bladder neck dysfunction (PBND). Pre- and on-treatment (minimum 3 months) uroflow/EMG parameters and subjective improvements were compared. RESULTS: Of 159 children (71 boys, 88 girls; median age 7.0 years, range 3.5-18.0 years), median follow up was 13.1 months (range 3-43 months). On targeted treatment, DV patients showed relaxation of pelvic floor during voiding and significant decrease in PVR on biofeedback; IDOD patients had normalization of short lag time and increased capacity on antimuscarinics; DUD patients had a decrease in capacity on timed voiding; PBND patients on alpha-blocker therapy showed improved uroflow rates and a decrease in mean EMG lag time (all p < 0.05). CONCLUSION: Non-invasive uroflow/EMG is useful not only for diagnosing specific LUT conditions, but also in objectively monitoring treatment efficacy. Subjective improvement on targeted therapy correlates well with objective improvements in uroflow/EMG parameters lending validation to this simplified approach to diagnosis.

Adolescent varicocelectomy: does artery sparing influence recurrence rate and/or catch-up growth?

The prevalence of varicocoeles is 15% in the general adolescent and adult male population and in 35-40% of men evaluated for infertility. While varicocelectomy can be performed using various methods and techniques, the laparoscopic approach allows for clear visualization of the testicular artery and lymphatics. Amongst urologists, particularly paediatric urologists, and andrologists there is much debate regarding the significance of testicular artery sparing when performing a varicocelectomy, with some believing that ligating the testicular artery impairs catch-up growth and future fertility. On the other hand, several studies have reported higher failure rates with artery preservation. To help resolve the debate regarding the significance of artery sparing, we sought to compare varicocoele recurrence rate and catch-up growth in patients who underwent artery sparing laparoscopic varicocelectomy compared with those who had the artery sacrificed. We identified 524 laparoscopic varicocelectomies in 425 patients from our adolescent varicocoele database. Only patients who had ultrasound determined testicular volume measurements pre-operatively and at least 6 months post-operatively were included. Post-operative persistence/recurrence of varicocoele, testicular atrophy and repeat varicocelectomy were noted. Catch-up growth was compared between procedures in those with significant pre-operative asymmetry. Four hundred and forty primary laparoscopic varicocelectomies were performed in 355 patients (mean age: 15.5 years, range 9.3-20.6; mean follow-up: 32.9 months, range 6.0-128.9) who had both pre- and post-varicocelectomy scrotal Duplex Doppler ultrasound performed. The testicular artery was preserved in 54 varicocoeles (41 patients) and ligated in 384 varicocoeles (312 patients). We observed an increased rate of persistent/recurrent varicocoele in the artery-sparing vs. artery ligating patients (12.2% vs. 5.4%, p = 0.09). In addition, there was no difference in catch-up growth and no instance of testicular atrophy. As artery sparing varicocelectomy offered no advantage in regards to catch-up growth and was associated with a higher incidence of recurrent varicocoele, preservation of the artery does not appear to be routinely necessary in adolescent varicocelectomy.

Physical exam and ultrasound characteristics of right varicocoeles in adolescents with left varicocoeles.

The published incidence of bilateral varicocoeles in adults is much higher than among adolescents. We investigated our adolescent varicocoele database to determine (i) the presence of subclinical and palpable right varicocoele s in boys with a left varicocoele, (ii) the effect, if any, of right varicocoele on asymmetry measurements and (iii) the difference in duplex doppler ultrasound (DDUS) findings between left varicocoele s and contralateral right varicocoele s. Among 506 boys with a known left varicocoele who underwent DDUS, the incidence of right clinical and subclinical varicocoele s as well as the influence of Tanner stage, varicocoele grade, laterality, peak retrograde flow (PRF) and maximum vein diameter (MVD) on testicular volume and asymmetry was examined using univariate and multivariate linear regression. Right retrograde flow was detected in 204 of 506 (40.3%) boys. Varicocoele grades were subclinical in 89 (18%), grade I in 51 (10%), grade II in 63 (12%) and grade III in 1 (0.2%). In patients with bilaterally palpable varicocoele s, increasing left grade was associated with greater testicular asymmetry (p = 0.02) and increasing right grade was associated with less testicular asymmetry (p = 0.01). Mean PRF and MVD values were lower in right as compared to left varicocoele s. Contrary to common perception, both subclinical and palpable right varicocoele s are frequently associated with left varicocoele s in adolescents. Although the contribution of right varicocoele s to fertility is controversial, these right varicocoele s can influence clinically relevant parameters, particularly testicular asymmetry, and thus influence which left varicocoele s undergo surgery.