Results of uretero-ureteral anastomosis in pathological duplex kidney.

INTRODUCTION: Duplex renal collecting system or duplex kidney (DK) commonly is associated with uropathy, with upper pole obstruction or lower pole vesicoureteral reflux (VUR). Its management is variable, ranging from therapeutic abstention to total nephrectomy. In case of damage to a single renal pole, uretero-ureteral anastomosis (UUA) is one of the surgical techniques for preserving the pathological pole. It can be performed by laparotomy, laparoscopy, or both. The aims of this study are to report the results of UUA in pathological DK, and compare outcomes depending on the surgical approach. METHODS: This is a retrospective analysis study over 20 years, from April 2002 to July 2022, including all children from 0 to 15 years old who underwent UUA for a DK. Outcome measure included per- and post-operative complications, the occurrence of urinary tract infections and ultrasound measurements during follow-up. RESULTS: Thirty-three children underwent an UUA over 20 years. The median age at diagnosis was 25 days (range: 1 day-12 years). The median age at surgery was 13 months (range: 2 months - 13 years). The sex ratio was 0.22. Prenatal diagnosis was made in 87.9 % of cases (n = 29). The left kidney was affected in 72.7 % of cases. Twenty-seven children (81.8 %) had an ectopic ureteral opening of the upper pole ureter; four children (12.1 %) had ureterocele of the upper pole and 2 children (6.1 %) had vesicoureteral reflux of the inferior pole. UUA was performed by inguinal approach (laparotomy) in 17 children (51.5 %), by laparoscopy in 9 cases (27.3 %), and by laparoscopy combined with laparotomy in 7 cases (21.2 %). The complication rate was 12.1 % (n = 4), including recurrent pyelonephritis with superior polar hydronephrosis (n = 2); iatrogenic injury of the lower pole ureter (n = 1) and a urinoma (n = 1), that all required surgical management with a polar nephrectomy. Between each surgical approach, there were no significant differences in operative time, hospital stay, complications. CONCLUSION: UUA is an effective therapeutic option in duplex kidneys as it allows the preservation of the pathological pole with low complication rates, regardless of the surgical approach.

Initial experience with ACT™ periurethral adjustable balloons to treat urinary incontinence due to intrinsic sphincter deficiency in the pediatric population.

PURPOSE: To present our initial experience with periurethral adjustable continence therapy (ACT™) for urinary incontinence due to intrinsic sphincter deficiency (ISD) in children. METHODS: This is an approved prospective non-randomized pilot study (NCT03351634) aiming to treat children born with spinal dysraphism (SD) or exstrophy epispadias complex (EEC) with ACT™. Endpoints were patient-reported changes in daily pad count, 24-h Pad test and complications. RESULTS: Since April 2018, 13 children (six girls, seven boys) were implanted at the median age of 12 years (5-16). The etiology of incontinence was neurogenic ISD (7/13, 54%) and EEC (6/13, 46%). After ACT™ implantation, continence (no pad or 1 security pad/day) was achieved in 9(69%) patients (5/7 SD, 4/6 EEC). Additionally, two (15%) patients had a significant improvement (decreasing Pad test from 1049 to 310 g at 3 months). One patient (7%) had no improvement. Results were stable at 21 months (6-43) of follow-up. Mean final balloon volume was 2.89 ml (± 0.85) with a median of 3 fillings to obtain continence. We had four revisions due to cutaneous port erosion (n = 3) and balloon migration (n = 1) and two definitive explantations. PinQ score was significantly improved (47 vs 40.5 with balloon, p = ns). Neither degradation of the upper urinary tract nor cystomanometric changes have been observed at 6 and 12 months postoperatively. CONCLUSION: Urinary incontinence due to ISD owing to EEC or SD can be successfully treated with ACT™ periurethral balloons. Given the minimal invasiveness of this therapy, it might be a first-line option treatment in children with complex stress urinary incontinence.

Financial and relational impact of having a boy with posterior urethral valves.

Introduction: Childhood chronic diseases affect family functioning and well-being. The aim of this study was to measure the impact of caring for a child with PUV, and the factors that most impact the burden of care. Patients and method: We gave a questionnaire on the familial impact of having a child with posterior urethral valves to all parents of a child included in the CIRCUP trial from 2015 onwards. The questionnaire included questions about the parents' demographics, health, professional, financial and marital status and how these evolved since the child's birth as well as the "impact on family scale" (IOFS), which gives a total score ranging from 15 (no impact) to 60 (maximum impact). We then analyzed both the results of the specific demographic questions as well as the factors which influenced the IOFS score. Results: We retrieved answers for 38/51 families (74.5% response rate). The average IOFS score was 23.7 (15-51). We observed that the child's creatinine level had an effect on the IOFS score (p = 0.02), as did the parent's gender (p = 0.008), health status (p = 0.015), being limited in activity since the birth of the child (p = 0.020), being penalized in one's job (p = 0.009), being supported in one's job (p = 0.002), and decreased income (p = 0.004). Out of 38 mother/father binomials, 8/33 (24.2%) declared that they were no longer in the same relationship afterwards. Conclusion: In conclusion, having a boy with PUV significantly impacts families. The risk of parental separation and decrease in revenue is significant. Strategies aiming to decrease these factors should be put in place as soon as possible.

Comparison of single-use flexible ureteroscopes with a reusable ureteroscope for the management of paediatric urolithiasis.

INTRODUCTION: To compare the efficacy, side effects, and cost-effectiveness between a single-use digital flexible ureteroscope and a reusable flexible ureteroscope in the treatment of paediatric renal stones. METHODS: This analytic, case-control, monocentric study included all patients undergoing flexible ureterosopies for stone treatment. Between April 2016 and February 2019, a reusable (Flex-XC®, Karl Storz) flexible ureteroscope was used (control group), whereas a single-use (Uscope®, PUSEN Medical©) flexible ureteroscope was used in all procedures from March 2019 to April 2021. Clinical and procedural outcomes, operative times, complication rates, hospital stay, and costs per procedure were evaluated. RESULTS: Forty-three cases using a reusable flexible ureteroscope and thirty-nine using a single-use flexible ureteroscope were included in the study. Demographic patient characteristics, stone burden, location and composition, preoperative presence of a double-J stent, procedural outcomes, mean length of postoperative hospital stay, and complications (4.6% versus 5%, p = 0.81) were comparable between the two groups. Median operative duration for stone removal was 93 min (20-170) with reusable versus 81 min (55-107) with the single-use scope (p = 0.18). Scope failure occurred four times with the reusable scope and in no case with the single-use. The total cost per procedure associated with the use of single-use scopes (798 Euros) was lower than a reusable scope (1483.23 Euros). DISCUSSION: Single-use flexible ureteroscopes were created to bypass the problems incurred when reusable scopes were damaged and therefore not available for use in surgical procedures. Single-use flexible ureteroscopes are always immediately available and ready to be used, even in urgent cases, as they typically do not require maintenance or sterilization. Compared with their reusable counterparts, single-use flexible ureteroscopes have similar digital performance (270°), image quality and we found no difference in the success and complication rates. Cost analysis of a reusable flexible ureteroscope must consider the purchase price, maintenance and repair costs, and decontamination costs (including handling, detergent, bacterial culture, transportation, and storage costs). In contrast, only purchase price is included in cost analysis for single-use flexible ureteroscopes. Our study suggests that single-use flexible ureteroscopes may be associated with lower costs per procedure than their reusable counterparts. CONCLUSION: Single-use flexible ureteroscopes are an interesting alternative to their reusable counterparts, particularly in terms of material resource management. Cost analyses conducted using a low volume of cases representative of a paediatric urology division favour the use of single-use ureteroscopes.

The coping strategies of individuals in multiple jeopardy settings: the case of unemployed older women.

This research investigates the harmful consequences of discrimination on self-esteem and examines the coping options of individuals belonging to several stigmatized groups (i.e., unemployed older women) within the multiple jeopardy perspective. Our sample comprised 420 individuals selected by age, gender and professional status. We tested whether the positive and negative links between discrimination and psychological distress induced by discrimination, would vary according to the number of disadvantaged categories individuals belong to. An analysis of the mediating role of some coping options was also conducted. Overall, the results support most of our hypotheses and suggest that the assumed impact of perceived discrimination on psychological outcome increase with the cumulation of discriminations. We also found that, among the various coping options used by individuals in our sample, commitment, but not age-group identification, mediated the links between the cumulated discrimination and self-esteem. The discussion addresses issues related to workplace discrimination in light of the multiple jeopardy perspective.

Risk factors for febrile urinary tract infection in boys with posterior urethral valves.

Objective: Boys with posterior urethral valves (PUV) present an increased risk of febrile urinary tract infection (fUTI). Identifying specific risk factors could allow for tailoring UTI prevention. The aim of this study was to use the data from the CIRCUP randomized controlled trial data to identify patient characteristics associated with a higher risk of fUTI. Patients and methods: We performed a secondary analysis of the data from the CIRCUP randomized trial which included boys with PUV, randomized to circumcision and antibiotic prophylaxis vs. antibiotic prophylaxis alone and followed for 2 years. There was only 1 episode of fUTI in the circumcision group vs. 17 in the uncircumcised group. We therefore only studied the antibiotic prophylaxis alone group and compared age at prenatal diagnosis, size and weight at birth, presence of dilating VUR at diagnosis, abnormal DMSA scan at 2 months, and nadir creatinine between children who presented a fUTI and those who did not, as well as age at first episode of fUTI. Results: The study group consisted of 42 patients of which 17 presented at least on fUTI. Presence of dilating VUR was significantly associated with risk of fUTI (p = 0.03), OR: 6 [CI 95% = (1.13-27.52)]. None of the other parameters were associated with increased risk of fUTI. We observed three distinct time periods for presenting a fUTI with a decrease in infection rate after the first 40 days of life, then at 240 days of life. Conclusion: In boys with PUV, presence of high-grade VUR is associated with a higher risk of presenting a fUTI. The rate of febrile UTIs seems to decrease after 9 months.

European Pediatric Surgeons' Association Survey on the Use of Splenic Embolization in Blunt Splenic Trauma in Children.

INTRODUCTION: This article assesses (1) access to splenic embolization (SE), (2) indications for SE, and (3) post-embolization management in high-grade splenic trauma in children. MATERIALS AND METHODS: An online questionnaire was sent in 2021 to all members of European Pediatric Surgeons' Association. RESULTS: There were a total of 157 responses (50 countries, 83% academic hospitals). Among them, 68% have access to SE (SE) and 32% do not (nSE). For a hemodynamic stable patient with high-grade isolated splenic trauma without contrast extravasation (CE) on computed tomography (CT) scan, 99% SE and 95% nSE respondents use nonoperative management (NOM). In cases with CE, NOM decreases to 50% (p = 0.01) and 51% (p = 0.007) in SE and nSE centers, respectively. SE respondents report a significant reduction of NOM in stable patients with an associated spine injury requiring urgent surgery in prone position, both without and with CE (90 and 28%, respectively). For these respondents, in stable patients the association of a femur fracture only tends to decrease the NOM, both without and with CE (93 and 39%, respectively). There was no significant difference in NOM in group nSE with associated injuries with or without CE. After proximal SE with preserved spleen vascularization on ultrasound Doppler, 44% respondents prescribe antibiotics and/or immunizations. CONCLUSION: Two-thirds of respondents have access to SE. For SE respondents, SE is used even in stable patients when CE showed on initial CT scan and its use increased with the concomitant need for spinal surgery. There is currently a variation in the use of SE and antibiotics/immunizations following SE.

Bladder Wall Segmentation and Characterization on MR Images: Computer-Aided Spina Bifida Diagnosis.

(1) Background: Segmentation of the bladder inner's wall and outer boundaries on Magnetic Resonance Images (MRI) is a crucial step for the diagnosis and the characterization of the bladder state and function. This paper proposes an optimized system for the segmentation and the classification of the bladder wall. (2) Methods: For each image of our data set, the region of interest corresponding to the bladder wall was extracted using LevelSet contour-based segmentation. Several features were computed from the extracted wall on T2 MRI images. After an automatic selection of the sub-vector containing most discriminant features, two supervised learning algorithms were tested using a bio-inspired optimization algorithm. (3) Results: The proposed system based on the improved LevelSet algorithm proved its efficiency in bladder wall segmentation. Experiments also showed that Support Vector Machine (SVM) classifier, optimized by Gray Wolf Optimizer (GWO) and using Radial Basis Function (RBF) kernel outperforms the Random Forest classification algorithm with a set of selected features. (4) Conclusions: A computer-aided optimized system based on segmentation and characterization, of bladder wall on MRI images for classification purposes is proposed. It can significantly be helpful for radiologists as a part of spina bifida study.

Preliminary experience in endoscopic section of posterior urethral valves using the Holmium: YAG laser.

INTRODUCTION: Refinements in endoscopic instrumentation, the widespread popularization of endourology and the minimal invasiveness of endoscopic approaches have led to evolving interest in expanding applications for their use and now include incision of posterior urethral valves (PUV). We aimed to report our paediatric experience of PUV incision with Holmium:YAG laser updating of the endoscopic technique, how we set parameters for the laser energy and provide some tips and tricks to increase the likelihood of completing treatment. METHODS: A monocentric, prospective, continuous series of boys with PUV were treated endoscopically using a Holmium: YAG laser (1.2 J, 20 Hz, 800 µs). Feasibility was evaluated using operative time in minutes, spontaneous normal micturition after bladder catheter removal, and the duration of bladder catheterization in days in the absence of satisfactory micturition. Peri-operative complications were recorded. A VCUG was performed at 6 weeks postoperatively to exclude residual valves. RESULTS: Since September 2018, 18 children with PUV were included. The median age at the time of endoscopic laser incision was 12 days (1 day-5 years). The median operative duration was 28 min (17-35). The urinary catheter was systematically removed on the first postoperative day. There were no intraoperative or anaesthesia-related complications. More specifically, no urethral injuries and no bleeding were recorded. No incomplete VUP incision was found on follow-up VCUG, and no endoscopic revision was necessary thus far, with a median follow-up of 44 months (6 months-60 months). DISCUSSION: The use of the Holmium: YAG laser introduces new perspectives in the treatment of PUV. Its mechanism of action is considered a photothermic effect with a vapourization effect. The laser energy released by the Holmium: YAG source has a short tissue penetration distance and is strongly absorbed in an aqueous environment and therefore limits thermal tissue damage and favours early tissue re-epithelialization, reducing the risk of urethral stricture and decreasing postoperative oedema. The use of the laser in "incision" mode is the setting that most solicits the capacities of the laser (high energy, high frequency, and long pulse). The use of laser energy has the advantage of allowing tissue vapourization while ensuring maximal haemostasis and the possibility of introducing the laser fibre through the working channels of small, 6-Fr paediatric endoscopes. CONCLUSION: In our experience, endoscopic PUV incision using the Holmium: YAG laser appears to be a safe and efficient technique.

Hypoxic preconditioning in renal ischaemia-reperfusion injury: a review in pre-clinical models.

Ischaemia-reperfusion injury (IRI) is a major cause of acute kidney injury (AKI) and chronic kidney disease, which consists of cellular damage and renal dysfunction. AKI is a major complication that is of particular concern after cardiac surgery and to a lesser degree following organ transplantation in the immediate post-transplantation period, leading to delayed graft function. Because effective therapies are still unavailable, several recent studies have explored the potential benefit of hypoxic preconditioning (HPC) on IRI. HPC refers to the acquisition of increased organ tolerance to subsequent ischaemic or severe hypoxic injury, and experimental evidences suggest a potential benefit of HPC. There are three experimental forms of HPC, and, for better clarity, we named them as follows: physical HPC, HPC via treated-cell administration and stabilised hypoxia-inducible factor (HIF)-1α HPC, or mimicked HPC. The purpose of this review is to present the latest developments in the literature on HPC in the context of renal IRI in pre-clinical models. The data we compiled suggest that preconditional activation of hypoxia pathways protects against renal IRI, suggesting that HPC could be used in the treatment of renal IRI in transplantation.

How Far Should We Explore Hypospadias? Next-generation Sequencing Applied to a Large Cohort of Hypospadiac Patients.

BACKGROUND: Next-generation sequencing (NGS) is generally used for patients with severe disorders of sex development (DSD). However, NGS has not been applied extensively for patients with hypospadias only, and most affected children do not benefit from an etiological diagnosis. OBJECTIVE: To evaluate the clinical usefulness of NGS for patients with hypospadias, regardless of severity. DESIGN, SETTING, AND PARTICIPANTS: Prospective multicenter research included 293 children with glandular to penoscrotal hypospadias (no undescended testis and no micropenis). After excluding likely pathogenic androgen receptor (AR) variants by Sanger sequencing, an NGS panel tested 336 genes including unexplored candidates in 284 patients. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The rate of pathogenic and likely pathogenic variants was assessed using REVEL, ClinVar, and in-house tools (Captain-ACHAB, MobiCNV, and MobiDetails). RESULTS AND LIMITATIONS: Likely pathogenic variants were identified in 16 (5.5%) patients with both Sanger sequencing and NGS taken into account. Some genes were related to DSD (AR, NR5A1, HSD17B3, and MAMLD1), but reverse phenotyping revealed two syndromic disorders with midline defects (MID1) and alteration in the retinoic acid signaling pathway (RARA). Coverage analysis revealed an 18q deletion. Identification of likely pathogenic variants increased with hypospadias severity. Other variants of unknown significance (VUSs) in genes implicated in hypogonadotropic hypogonadism, Noonan syndrome, and genital tubercle development were also identified. Genetic study mainly focused on exonic variants, and most cases remain unexplained. CONCLUSIONS: NGS reveals minor forms of DSD, undiagnosed syndromes, or candidate rare variants in new genes, indicating that even patients with mild hypospadias benefit from advanced sequencing techniques. Early molecular diagnosis would help improve follow-up at puberty and medical counseling for initially undiagnosed syndromes. Future studies will improve the diagnosis by investigating the contribution of VUSs. PATIENT SUMMARY: Next-generation sequencing enables simultaneous testing of numerous genes and should not be limited to disorders of sex development cases. Even patients with mild hypospadias would benefit from early diagnosis of a genetic defect implicated in sex development or other syndromes.

Description of a giant hypothalamic hamartoma associated with an immature ruptured giant sacrococcygeal teratoma: a case report.

Giant hypothalamic hamartomas (GHH) are rare neonatal intracerebral congenital malformations responsible for gelastic epilepsy and/or endocrine disturbances. Sacrococcygeal teratomas (SCT) are fetal neoplasms associated with perinatal morbidity and mortality, especially hemorrhagic complications in giant examples (GSCT). Here, we describe an immature ruptured GSCT complicated by hemorrhagic shock at 32-week gestation boy requiring an emergency delivery, followed immediately by urgent surgical removal. A brain lesion resembling a GHH was also present on the antenatal MRI. In order to exclude metastatic immature teratoma or glioma, a biopsy was performed by a retro-sigmoidal approach, which confirmed the nature of the hamartoma. Here, we describe for the first time the association of a ruptured immature GSCT associated with a GHH.

Micro-percutaneous nephrolithotomy (Microperc) for renal stones, outcomes and learning curve.

OBJECTIVE: To report the effectiveness, reliability and learning curve of Microperc, a minimal invasive percutaneous technique using a 4.85-Ch (16-gauge) sheath, in the treatment of nephrolithiasis. MATERIAL AND METHODS: 31 consecutive Micropercs for nephrolithiasis<2.5cm were performed by 2 operators in 2 different institutions from the 1st of May 2015 to 31st of December 2017. RESULTS: The mean size of stones was 19mm±11mm, and mean density was 1048±249UH. Stones were located in lower calyx in 21/31(68%), medium calyx in 3/31(10%), pelvis in 4/31(12%) and were multi-caliceal in 3/31(10%). Five patients (16%) had urinary diversion (4 ileal conduits, 1 enterocystoplasty with Mitrofanoff+bladder neck closure) all of those having neurological disease (2 multiple sclerosis, 3 spinal cord injury). Mean operating time was 83±35min and decreased after short period for both operators. 9/31(29%) patients had complication: 8 (26%) had fever (Clavien II) and 1 (3%) had renal colic pain (Clavien III) (required JJ stent). Stone-free was obtained in 13/31(42%) and 11/31(36%) had residual microfragments<3mm which did not require further treatment, corresponding to a technical success of 78% (24/31). Success rate was similar in patients with urinary diversion and patients with normal anatomy. CONCLUSIONS: This study showed that Microperc was an effective technic for kidney stone treatment with low complication rate, acceptable operating time and short learning curve. Microperc was useful for stones in the lower calyx and/or urinary diversion where retrograde ureteroscopy could reach its limits. LEVEL OF EVIDENCE: 3.

European Pediatric Surgeon' Association Survey on the Management of Short-Bowel Syndrome.

INTRODUCTION: The aim of this study was to assess the management of short-bowel syndrome (SBS) at the time of primary surgery, and the strategies used to facilitate enteral autonomy depending on the institutional expertise. MATERIALS AND METHODS: An online questionnaire was sent in 2019 to members of The European Pediatric Surgeons' Association. RESULTS: Among the 65 responding members (26 countries, 85% from university hospitals), 57% manage less than three new patients with SBS per year (group A), and 43% at least three patients (group B). The cut-off of three patients treated yearly used in our study was defined after statistical analysis of different cut-offs. A multidisciplinary intestinal rehabilitation program is significantly more frequent in group B than in group A (85 and 53%, respectively; p = 0.009). Considering the primary surgical management of multiple intestinal atresia and congenital ultra-short bowel with jejunal atresia, primary surgical strategies to optimize bowel length are more often used in group B than group A (p = 0.09 and p = 0.04, respectively). A minimum of one intestinal lengthening procedure every 2 to 3 years is significantly more frequent in group B than group A (95 and 45%, respectively; p = 0.0013). Among the strategies used to promote intestinal adaptation, group B (35%) uses significantly more often glucagon-like peptide 2 analogs than group A (10%) (p = 0.02). CONCLUSION: Based on our survey, a minimum number of SBS patients treated yearly is required to manage this challenging disease according to up-to-date medical and surgical strategies. However, whatever their level of expertise is in managing SBS, most of pediatric surgeons are involved in the primary surgery. Medical education programs about SBS should be more largely available to pediatric surgeons.

Optimal settings for the Holmium: YAG laser in pediatric endourology: Tips and tricks.

INTRODUCTION: To the best of our knowledge, no pediatric paper has been published regarding specifically how to set the Holmium:YAG laser for multiple urologic applications. OBJECTIVE: To provide insight into the laser parameters for pediatric applications. STUDY DESIGN: We describe the principle and the settings of the laser. RESULTS: The Holmium:YAG laser can produce four different biological effects: (1) fragmentation of stones in small fragments that can be retrieved with grasping instruments, thereby increasing the immediate stone-free outcome. For fragmentation lithotripsy, the laser has to be set with a high energy, low frequency and short pulse duration; (2) dusting which produces fine dust that can spontaneously evacuate, avoiding the use of basket retrieval. The dusting setting requires low energy, high frequency and long pulse duration; (3) incision of posterior urethral valves or ureterocele when all settings are maximized: high energy, high frequency and long pulse duration; (4) coagulation of urothelial tumors using high frequency, long pulse duration and slightly lower energy than required for incision. DISCUSSION: Both dusting by painting and fragmentation with retrieval for ureteroscopic laser lithotripsy are effective. Although dusting tends to be associated with shorter operative times and a lower risk of ureteral trauma, this approach has a potential risk of recurrent stone formation from dust failing to pass. In contrast, fragmentation with extraction may provide for a more immediate postoperative stone-free result. Altering the pulse energy, frequency, width and modulation can help to optimize lithotripsy efficiency. Lower pulse energy settings result in smaller fragments, less retropulsion and reduce fiber tip degradation. A shallow depth of penetration in water and tissue allows precise energy application and provides a margin of safety. CONCLUSION: An understanding of Ho-YAG laser settings will permit the pediatric surgeon to make a better use of the device for different urological applications.

Minimally invasive surgery for unilateral Wilms tumors: Multicenter retrospective analysis of 50 transperitoneal laparoscopic total nephrectomies.

PURPOSE: To evaluate feasibility and outcomes of minimally invasive surgery (MIS) in Wilms tumor (WT). METHODS: International multicenter review of MIS total nephrectomies for WT between 2006 and 2018. Medical records of confirmed WT were retrospectively assessed for demographic, imaging, treatment, pathology, and oncological outcome data. RESULTS: Fifty patients, with a median age of 38 months (6-181), were included in 10 centers. All patients received neoadjuvant chemotherapy, as per SIOP protocol. Median tumor volume post-chemotherapy was 673 mL (18-3331), 16 tumors crossed the lateral border of the spine, and three crossed the midline. Six patients with tumors that crossed the lateral border of the spine (tumor volumes 1560 mL [299-2480]) were converted to an open approach. There was no intraoperative tumor rupture. Overall, MIS was completed in 19% of the 195 nephrectomies for WT presenting during the study period. Tumor was stage I in 29, II in 16, and III in 5, and histology was reported as low in three, intermediate in 42, and high risk in five. Three patients had positive tumor margins. After a median follow-up of 34 months (2-138), there were two local recurrences (both stage I, intermediate risk, 7 and 9 months after surgery) and one metastatic relapse (stage III, high risk, four months after surgery). The three-year event-free survival was 94%. CONCLUSION: MIS is feasible in 20% of WT, with oncological outcomes comparable with open surgery, no intraoperative rupture, and a low rate of local relapse. Ongoing surveillance is, however, needed to evaluate this technique as it becomes widespread.

Comparative medico-economic study of reusable vs. single-use flexible ureteroscopes.

PURPOSE: Reusable flexible-ureteroscopes (fURS) require personnel and budget for processing and repairing, whereas single-use fURS were recently developed. After exclusive reusable fURS since 2011, we experienced high repair costs and single-use fURS were therefore introduced in mid-2017. We aimed to evaluate economic and practical advantages and disadvantages of reusable versus single-use fURS. MATERIALS AND METHODS: First, we evaluated the incidence of breakage and repairs of reusable fURS in 2017. We assessed the overall operational costs of reusable fURS including purchase, processing, and repairing in our institution from 2011 to 2017. Following our experience, we created a model to compare operation costs/procedure of single-use fURS with reusable fURS depending on repair costs. RESULTS: In 2017, repair costs of reusable fURS increased by 345% compared with the period 2011-2016, causing: a median unavailability per reusable fURS of 200 days/year (100-249), median number of functioning fURS 0/5-3/5 per operating day, while unavailability of reusable fURS had become the first reason for cancellation of procedure. Since it was introduced, single-use fURS accounted for 59% of the flexible ureteroscopy activity. Taking into account the costs of processing, maintenance and repair, in 2011-2016 versus 2017, the single-use fURS was cost-effective compared with the reusable fURS until the 22nd procedure versus the 73rd procedure, respectively. CONCLUSIONS: After years of exclusive reusable fURS, the rising incidence of breakage not only increased maintenance costs but also hampered daily activity owing to unavailability of the devices. The introduction of single-use with reusable fURS provided substantial help to maintain our activity.

Prenatal imaging of genital defects: clinical spectrum and predictive factors for severe forms.

OBJECTIVES: To report the clinical spectrum of genital defects diagnosed before birth, identify predictive factors for severe phenotypes at birth, and determine the rate of associated malformations. PATIENTS AND METHODS: A retrospective study (2008-2017) of 4580 fetuses, identified prenatally with abnormalities evaluated by our Reference Center for Fetal Medicine, included cases with fetal sonographic findings of abnormal genitalia or uncertainty of fetal sex determination. Familial, prenatal and postnatal data were collected via a standardised questionnaire. RESULTS: In all, 61 fetuses were included. The positive predictive value (PPV) of the prenatal diagnosis of genital defects was 90.1%. Most cases were 46,XY-undervirilized boys, 42 cases (68.8%), which included 29 with mid-penile or posterior hypospadias, nine with anterior hypospadias, and epispadias, micropenis, scrotal transposition, and buried penis (one each). In all, 46,XX-virilized girls were identified in seven cases (11.5%), which included four with congenital adrenal hyperplasia, two with isolated clitoromegaly, and one with ovotestis. Other defects included prune belly syndrome and persistent cloaca (six cases). Early detection during the second trimester (58.1% vs 18.8%, P = 0.03), intra-uterine growth restriction (IUGR) (45.2% vs 9.1%, P = 0.06), and curvature of the penis (38.7% vs 0%, P = 0.02), were more frequently related to severe defects in male newborns. Associated malformations (14 cases, 22.9%) and genetic defects (six) were frequent in undervirilized boys. CONCLUSION: Prenatal imaging of genital defects leads to a wide range of phenotypes at birth. Its PPV is high and extra-urinary malformations are frequent. Early diagnosis during the second trimester, associated IUGR, and curvature of the genital tubercle, should raise suspicion of a severe phenotype and may justify delivery near a multidisciplinary disorders/differences of sex development team.

Neurostimulation-guided Anal Intrasphincteric Botulinum Toxin Injection in Children With Hirschsprung Disease.

OBJECTIVES: In Hirschsprung disease (HD), despite successful surgical treatment, 50% of children experience long-term functional gastrointestinal problems, particularly chronic functional obstructive symptoms. We report our experience regarding clinical effects of neurostimulation-guided anal intrasphincteric botulinum toxin (BT) injections on postoperative obstructive symptoms attributed to a nonrelaxing anal sphincter complex in HD patients. METHODS: In this monocenter cohort study, 15 HD patients with postoperative functional intestinal obstructive symptoms received neurostimulation-guided anal intrasphincteric BT injections. Short-, medium-, and long-term effects were evaluated. The Bristol stool form scale was used to assess stool consistency, and the Jorge-Wexner (JW) score to assess fecal continence. RESULTS: The median age at first injection was 4 years. In the short-term, a significant improvement in stool consistency was noted in 12 of 14 patients (P = 0.0001) and JW score decreased for 14 of 15 patients (P = 0.001). In the medium-term, JW score significantly decreased for all patients (P = 0.0001), with an improvement of 50% or more for 10 patients (66.7%). In the long term, 83.3% of patients had normal stool consistency and JW score was <3 for all. Recurrent enterocolitis decreased from 86.7% to 8.3%. A complete resolution of all symptoms without further medication was observed in 66.7% of patients in the long term. CONCLUSIONS: Intrasphincteric BT injection was a safe, effective, and durable option for the management of postoperative functional intestinal obstructive symptoms in HD. The use of neurostimulator guidance for specific delivery of BT to muscular fibers of nonrelaxing anal sphincter complex takes into consideration the variability of patient's anatomy secondary to curative surgery.