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Irene Paraboschi,1 Michele Gnech,1 Dario Guido Minoli,1 Erika Adalgisa De Marco,1 Giovanni Parente,2 Guglielmo Mantica,3,4 Vincenzo Bagnara,5 Gianantonio Manzoni,1 Marc-David Leclair,6 Alfredo Berrettini1 1Department of Pediatric Urology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milano, Italy; 2Department of Pediatric Surgery, ASST Papa Giovanni XXIII Hospital, Bergamo, 24127 Italy; 3IRCCS Ospedale Policlinico San Martino, Genoa, Italy; 4Department of Surgical and Diagnostic Integrated Sciences (DISC), University of Genoa, Genoa, Italy; 5Department of Pediatric Surgery, Policlinico G.B.Morgagni, Catania, Italy; 6Chirurgie Infantile, CHU de Nantes, Nantes, FranceCorrespondence: Guglielmo Mantica, Consultant Urologist, Dirigente Medico, IRCCS Ospedale Policlinico San Martino, Genova, Italy, Email guglielmo.mantica@gmail.comAbstract: The vascular supply of the pelvic structures and the external genitalia can be easily injured during the one-stage delayed bladder closure and radical soft-tissue mobilization (Kelly procedure) for bladder exstrophy surgical repair. Aiming to help surgeons assessing and confirming tissue perfusion and viability, indocyanine green (ICG)-based laser angiography was incorporated into the operative approach to reduce the risk of ischemic injuries. The EleVision IR system (Medtronic Ltd) was adopted to confirm the identification of the vascular pedicles and assess the tissue perfusion in real-time in a 5-month-old with bladder exstrophy undergoing the one-stage delayed bladder closure and radical soft-tissue mobilization (Kelly procedure). ICG (0.15 mg/kg) was intravenously administered at 6 key steps during surgery with the ability to be re-dosed every 15 minutes. ICG-based laser angiography helped to confirm the correct identification of the vascular structures during surgery and to assess tissue perfusion in real-time. Blood flow did not change considerably after initial dissection or upon approximating the pubis symphysis. At the end of the procedure, good penile perfusion was shown, proving that no direct injury or substantial compression of the pudendal vessels had occurred following the mobilization and the reconstructive phase. ICG-based laser angiography proved to be safe, effective, and easy to employ and should be considered as a reasonable adjunct for tissue perfusion assessment and operative decision-making in patients undergoing bladder exstrophy Kelly repair.Keywords: bladder exstrophy, radical soft-tissue mobilization, fluorescence-guided surgery, indocyanine green, children

Introduction: Total nephrectomies for the treatment of Wilms' tumor (WT) are more and more performed by laparoscopy, although indications for this approach following the UMBRELLA guidelines are currently very restrictive. The purpose of this study was to assess the compliance to the criteria of the UMBRELLA protocol for minimally invasive approach of WT., Methods: This retrospective multicenter study included children operated on by laparoscopic total nephrectomy for suspected WT before 2020. Imaging was reviewed centrally., Results: Fifty-six patients (50 WT and 6 nephrogenic rests) were operated on at a median age of 3.3 ± 2.6 years. Thirteen (23%) patients had metastasis at diagnosis. The mean operative time was 213 ± 84 min. There were eight (14.3%) conversions and five peroperative complications. A local stage III was confirmed in seven (12.5%) cases, including two for tumor rupture. Only one (1.8%) of the procedures followed the SIOP-UMBRELLA indications for laparoscopy. The criterion "ring of normal parenchyma" was met only once. Conservative surgery seemed possible in ten (17.9%) cases. The extension of the tumor beyond the ipsilateral edge of the vertebra after chemotherapy and a volume over 200 mL were associated with an increased risk of conversion (p = 0.0004 and p = 0.001 respectively). After a mean follow-up of 5.2 ± 4.0 years, although there was no local recurrence, one death occurred due to metastatic progression at 15 months postoperatively., Conclusions: The laparoscopic approach of WT beyond the UMBRELLA recommendations was feasible with low risk of local recurrence. Its indications may be updated and validated., (© 2024. Society of Surgical Oncology.)

Introduction & Objectives: The use of transperitoneal robotic-assisted procedures for ureterovesical junction (UVJ) anomalies in pediatric patients is increasing. However, the extra-peritoneal approach potentially less invasive, remains underexplored with robotic approach in pediatric patients. We present our experience of robot-assisted extra-vesical laparoscopic extra-peritoneal UVJ surgery., Materials & Methods: Extraperitoneal access was made through a 1,5 cm horizontal incision below the umbilicus. Digital dissection was performed under the rectus fascia to the pubic symphysis. The space was further developed under direct vision with laparoscope. The Da Vinci Xi platform was docked after the placement of 4 robotic 8 mm-trocars and an 8 mm assistant port., Results: We performed extraperitoneal robot-assisted laparoscopic UVJ surgery in 3 patients: - Case 1: 9-year-old boy: left-sided ureterectomy, UV reimplantation (UVR) with psoas hitch for a symptomatic mega-ureter. - Case 2: 13-year-old girl: right-sided VUR on a duplex system treated with an extravesical Lich-Gregoir antireflux plasty. - Case 3: 8-year-old boy: left-sided bladder diverticulectomy with UVR. There is no complication above Clavien 2 and no recurrence of febrile UTI during the follow-up period (from 6 to 17 months)., Conclusions: Extra-peritoneal robotic approach for UVJ surgery further for UVR in pediatric urology seems feasible with good short-term result even for the complex ones., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Background: Abdominal and pelvic closure remains a challenge during bladder exstrophy initial repair. We aimed to report on the feasibility and results of a novel technique of bilateral obturator osteotomy., Methods: Retrospective study of prospective collected data of children who underwent single-stage delayed bladder exstrophy closure combined with RSTM (Radical Soft Tissue Mobilization) for BEEC (Bladder Exstrophy Epispadias Complex) by the same team at different institutions between December 2017 and May 2021. When pubic approximation was not feasible at the end of the procedure, bilateral obturator osteotomy was performed through the same approach, consisting in bilateral divisions of the ilio-pubic rami, ischio-pubic rami, obturator membrane, and detachment of the internal obturator muscle. Pubic bone fragments were approximated together on the midline. Immobilization in a thermoformed posterior splint was indicated for 3 weeks. The main outcome criterion was the bladder dehiscence rate at 6 months, assessed by physical inspection. Secondary outcome criteria included neurovascular obturator pedicle injury, analyzed during orthopedic physical examination, wound or bone infections, gait acquisition, reported by parents and evaluated during medical examination, and vascular penile impairment, judged by penile and glans coloration., Results: 17 children (11 males, 6 females) were included, at a median age of 2 months [1-33]; and representing 29% (17/58) of the children with bladder exstrophy who underwent the same surgical approach during the time of study. There was no postoperative bladder dehiscence with a median follow-up of 34 months [6-47]. No complication was observed. Pelvic X-rays showed bilateral normal ossification process. Neither gait abnormality, nor clinical indication of obturator nerve deficiency was observed during follow-up., Conclusion: When pubic bones approximation is not possible, bilateral obturator osteotomy is a useful adjunct in bladder exstrophy closure, feasible by the pediatric urologist through the same approach, and not requiring external fixator., Level of Evidence: IV., Competing Interests: Conflicts of interest All the authors declare that they have no potential conflicts of interests., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Competing Interests: Conflict of interest None.

Introduction: Ureteroscopy (URS) can be proposed as first-line therapy for the management of pelvic stones from 10 to 20 mm and for lower ureteric stones in children. However, little is known about the success and the morbidity of URS in young children. Ureteroscopic treatment may present matters in young children because of the small size of the pediatric kidney and the small size of the collecting system., Objective: To assess safety and efficacy of URS for the treatment of urinary stones in children aged of 5 years or less., Study Design: After the institutional ethical board approval was obtained, we conducted a retrospective, analytic, multicentric study that included all URS performed between January 2016 and April 2022 in children aged of 5 years or less. In this non-comparative case series, anonymized pooled data were collected from 7 tertiary care centers of pediatric patients. Endpoints were the one-session SFR at 3 months and per and postoperatives complications. Descriptive statistics were applied to describe the cohort., Results: Eighty-three patients were included. For them, 96 procedures were performed at the median age of 3.5 years (IQR: 0.8-5) and median weight of 14 Kg (6.3-23). Median stone size was 13 mm (4-45). There were 65 (67 %) renal stones treated with flexible URS, most of which were in the renal pelvis (30 %) and in the lower calix (33 %). A ureteral access sheath was used in 91 % procedures. Preoperative ureteral stent was placed in 52 (54 %) of patients. None of patients had ureteral dilatation. The single-session SFR was 67.4 % (56.3 and 89.2 % for flexible URS and semi-rigid URS respectively) and children require 1.4 procedures to achieve complete stone clearance. The overall complication rate was 18.7 %, most of them were minor (Clavien I-II). Intraoperative perirenal extravasation (Clavien IIIb) due to forniceal rupture was documented in 6.2 % of cases, related to an increased intrapelvic pressure (IPP) performed in a closed pelvicalyceal system., Discussion: Pediatric urologists should be aware of forniceal rupture based on the presence of extravasation of contrast during endourological procedures especially when they have difficulties to reach lower caliceal stone in small patient., Conclusion: URS in patients aged of 5 years or less, is a complex minimally invasive procedure with reasonable efficacy and low morbidity. Intrarenal stones treated by RIRS in young children carries the risk of additional procedures to complete stone clearance., Competing Interests: Conflict of interest The authors declare that they have no conflicts of interest., (Copyright © 2023. Published by Elsevier Ltd.)

Introduction: The Radical soft-tissue mobilisation (RSTM) described by J.H. Kelly for bladder exstrophy repair implies a detachment of levator ani muscle insertions from the pelvic wall. The aim of this controlled study was to evaluate the impact of this procedure on subsequent anorectal function., Methods: Monocentric controlled study of prospectively collected data of children who underwent RSTM for BEEC from 2010 to 2017. Patients born after 2017 were not included, as they were below the theoretical age of continence acquisition at the time of the study. Anorectal function was assessed using the Childhood Bladder and Bowel Dysfunction Questionnaire, and quality of life (QoL) related to fecal continence using the CINCY FIS questionnaire. The control group was paired on age and sex with a 1:3 patient/control ratio. Answers to questionnaires were collected from September 2021 to January 2022. Univariate statistical analysis comparing two groups and subgroup analysis following age were also performed., Results: During the period of study, 55 children with BEEC underwent Kelly RSTM. Twenty-seven (49%) were included and paired with 81 healthy children on age and sex. Median age at surgery was 15 months [0.5-93] and median follow-up was 10 years [4-13]. Patient's group median age at evaluation was 11 years [5-19]. There was no difference between patients and control group in anorectal function for both incontinence and constipation items. No significant difference was found in QoL related to fecal incontinence assessment. Subgroup analysis did not show difference., Conclusion: This study suggests that the levator ani detachment during Kelly procedure, realised in a paediatric population under the age of 8, did not impact anorectal function with a mid-term follow-up., Level of Evidence: III., Competing Interests: Declaration of competing interest None declared., (Copyright © 2023. Published by Elsevier Inc.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Harper, Botto, Peycelon, Michel, Leclair, Garnier, Clermidi, Arnaud, Dariel, Dobremez, Faure, Fourcade, Boudaoud, Chaussy, Huiart, Bocquet, Ferdynus and Sauvat.)

Background Data: EA is the most frequent congenital esophageal malformation. Long gap EA remains a therapeutic challenge for pediatric surgeons. A case case-control prospective study from a multi-institutional national French data base was performed to assess the outcome, at age of 1 and 6 years, of long gap esophageal atresia (EA) compared with non-long gap EA/tracheo-esophageal fistula (TEF). The secondary aim was to assess whether initial treatment (delayed primary anastomosis of native esophagus vs. esophageal replacement) influenced mortality and morbidity at ages 1 and 6 years., Methods: A multicentric population-based prospective study was performed and included all patients who underwent EA surgery in France from January 1, 2008 to December 31, 2010. A comparative study was performed with non-long gap EA/TEF patients. Morbidity at birth, 1 year, and 6 years was assessed., Results: Thirty-one patients with long gap EA were compared with 62 non-long gap EA/TEF patients. At age 1 year, the long gap EA group had longer parenteral nutrition support and longer hospital stay and were significantly more likely to have complications both early post-operatively and before age 1 year compared with the non-long gap EA/TEF group. At 6 years, digestive complications were more frequent in long gap compared to non-long gap EA/TEF patients. Tracheomalacia was the only respiratory complication that differed between the groups. Spine deformation was less frequent in the long gap group. There were no differences between conservative and replacement groups at ages 1 and 6 years except feeding difficulties that were more common in the native esophagus group., Conclusions: Long gap strongly influenced digestive morbidity at age 6 years., Competing Interests: Conflict of interest All the authors declare that they have no conflict of interest., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: The Anderson-Hynes technique has been the treatment of choice for primary ureteropelvic junction obstruction in children. Laparoscopic approach has shown similar outcomes to open, with advantages of shorter hospital stay and less pain. We reviewed the experience of 11 geographically diverse, tertiary pediatric urology institutions focusing on the outcomes and complications of laparoscopic pyeloplasty., Materials and Methods: A descriptive, retrospective study was conducted evaluating patients undergoing Anderson-Hynes dismembered laparoscopic pyeloplasty. Centers from four different continents participated. Demographic data, perioperative management, results, and complications are described., Results: Over a 9-year period, 744 laparoscopic pyeloplasties were performed in 743 patients. Mean follow-up was 31 months (6-120m). Mean age at surgery was 82 months (1 w-19 y). Median operative time was 177 min. An internal stent was placed in 648 patients (87%). A catheter was placed for bladder drainage in 702 patients (94%). Conversion to open pyeloplasty was necessary in seven patients. Average length of hospital stay was 2.8 days. Mean time of analgesic requirement was 3.2 days. Complications, according to Clavien-Dindo classification, were observed in 56 patients (7.5%); 10 (1%) were Clavien-Dindo IIIb. Treatment failure occurred in 35 cases with 30 requiring redo pyeloplasty (4%) and 5 cases requiring nephrectomy (0.6%)., Conclusion: We have described the laparoscopic pyeloplasty experience of institutions with diverse cultural and economic backgrounds. They had very similar outcomes, in agreement with previously published data. Based on these findings, we conclude that laparoscopic pyeloplasty is safe and successful in diverse geographics areas of the world., Competing Interests: Conflicts of interest None., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: To report long-term results of artificial urinary sphincter implantation for urinary incontinence due to intrinsic sphincter deficiency in children., Materials and Methods: This retrospective monocentric study included all patients who underwent artificial urinary sphincter (AMS 800) implantation before 18 years of age between 1986 and October 2018 for intrinsic sphincter deficiency. The primary outcome was the continence rate at the last follow-up, defined by the daily use of 0 pads. The secondary outcome was the overall survival of the device, defined as the absence of any repeated surgery (revision or explantation) during follow-up. Reoperation-free, revision-free, and explantation-free device survival rates were estimated using the Kaplan-Meier method., Results: Thirty-six patients with a median age of 12 years (interquartile range [IQR]: 10-14) were included (15 females, 21 males). The median follow-up was 18.7 years (IQR: 9-26). The main underlying condition was spinal dysraphism (n = 24; 67%). The median time to the first reoperation was 9 years (IQR: 3.75-14.7). At the last follow-up, survival rates without revision were 84%, 71%, 55%, and 33% at 5, 10, 15, and 20 years, respectively. Survival rates without explantation were 91%, 84%, 80%, and 72% at 5, 10, 15, and 20 years, respectively. At the last follow-up, 29 patients had a functional device. The overall continence rate was 88%. All patients who had their device still in place were continent at the last follow-up., Conclusion: The artificial urinary sphincter is an effective long-term treatment for urinary incontinence related to intrinsic sphincter deficiency in children, providing a high rate of continence, even if associated with a high rate of reoperation., (© 2022 Wiley Periodicals LLC.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Harper, Botto, Peycelon, Michel, Leclair, Garnier, Clermidi, Arnaud, Dariel, Dobremez, Faure, Fourcade, Boudaoud, Chaussy, Collin, Huiart, Ferdynus, Bocquet and Sauvat.)

Introduction: A wide range of surgical interventions have been described for the management of primary obstructive megaureter (POM). Endoscopic balloon dilatation has been developed through last decades as a minimally invasive alternative to classic surgery., Objective: To assess the need for placement of a double J stent after endoscopic balloon dilatation procedure, by comparing the post-operative related outcomes with and without double J placement. Secondary outcome was the success rate, considering the need for further procedure after endoscopic balloon dilatation and the improvement of the ureteral diameter in the two groups., Study Design: Historical retrospective comparison of children treated by endoscopic dilatation for POM, with post-operative JJ stent left in place (2012-2014) or without ureteral JJ stent (since 2015). Post-operative complications were reported following Clavien-Dindo grading system and compared between the two groups. Success rate was defined as absence of need for further surgical reimplantation. Ureteral diameters on preoperative and postoperative renal ultrasounds were compared., Results: Endoscopic dilatations were performed in 42 patients for 46 renal units during the study period. There was a significantly higher rate of post-operative complications in the group with JJ stenting compared to the group without double J stenting regarding all Clavien-Dindo grades (56% vs 15%, p = 0.014) and Clavien-Dindo grade III only (31% vs 0%, p = 0,0051) (Figure). The success rate was similar in the JJ group (75%, F-up: 70 months [13-101]) and the no JJ group (81%, F-up: 26 months [12-95]). There was a significant improvement of US renal pelvis and ureter dilatation in both groups, with a median follow-up of 35.5 months [12-101]., Discussion: The overall rate of complications was slightly higher than in other reports and higher in the JJ group regarding Clavien-Dindo grade III complications. The success rate was comparable to previous studies reviewing endoscopic dilatations and equivalent in the two groups., Conclusion: In our study, the omission of postoperative ureteral drainage by a JJ stent after endoscopic balloon dilatation of POM did not increase post-operative complications rate without demonstrable impact on the success rate., Competing Interests: Conflicts of interest The authors have no conflicts of interest to declare that are relevant to the content of this article., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Urethral strictures represent the most frequent long-term complication in men after history of hypospadias surgery., Objectives: To better define the spectrum of men previously treated for hypospadias during childhood, presenting at an adult urology clinic with persistent urethral complications., Study Design: Retrospective review of the records of 42 consecutive adult patients with a personal history of hypospadias repair during childhood referred at one adult tertiary urology center between 2004 and 2017. Those with persistent urethral strictures were included: 28 patients (median age 28yr [17-81]). Residual chordee, aesthetic concerns, isolated fistulas, and motives of consultation unrelated to hypospadias were excluded. Early initial success rate and location of urethral stricture were studied. Statistical Analysis was done by non-parametric tests., Results: On the 42 consecutive adults eligible, a total of 28 patients had a persistent urethral stricture. During childhood, 13/28 boys had required multiple surgical revision procedures, including six of them for postoperative urethral strictures. During adulthood, initial urethral assessment revealed 29 urethral strictures in 28 patients (penile urethra 23/29, bulbar urethra 8/29). The early initial success rate of stricture treatment was 50% (median follow-up: 4 years [1-7]). Additional surgical procedures were needed and performed in 11 patients. Eight patients were eventually diagnosed with a bulbar stricture, either isolated (n = 5) or combined with a distal stricture (n = 3), without significant relationship with initial position of meatus., Discussion: Predictive factors for bulbar strictures locations could not be identified. It was however observed that symptomatic bulbar strictures do exist in adults, in the long term after penile hypospadias repair during childhood, independently of the initial site of hypospadias, initial success of primary repair, and the type of surgical reconstructions performed during childhood., Conclusion: Bulbar strictures represent more than 25% of the overall strictures diagnosed in adult patients treated for hypospadias during childhood, independently of the original site of urethral reconstruction., Competing Interests: Conflicts of interest The authors have no conflicts of interest to declare that are relevant to the content of this article., (Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Boys with posterior urethral valves (PUVs) have an increased risk of febrile urinary tract infections (fUTIs). Circumcision is believed to reduce the risk of fUTIs in boys, although there are no randomized trials demonstrating this., Objective: To determine the effect of circumcision on the risk of fUTIs in boys with PUVs., Design, Setting, and Participants: A clinical randomized trial that ran between August 2012 and July 2017 was conducted. The trial was multicentric, including 13 referral centers for pediatric urology. Male boys, aged 1-28 d, diagnosed with posterior urethral valves, confirmed by voiding cystogram, were included. The exclusion criteria included presence of a genital malformation contraindicating performing a circumcision., Intervention: Participants were randomized to neonatal circumcision + antibiotic prophylaxis (CATB) or antibiotic prophylaxis alone (ATB), and followed for 2 yr., Outcome Measurements and Statistical Analysis: The primary outcome was a risk of presenting fUTIs in each group. An fUTI was defined as fever (>38.5 °C) with evidence of pyuria and culture-proven infection on urinalysis, obtained by urethral catheterization or suprapubic aspiration. A bivariate analysis of the primary outcome was performed using the Kaplan-Meier method., Results and Limitations: In total, 91 patients were included: 49 in group CATB and 42 in group ATB. The probability of presenting an fUTI was 20% in group ATB versus 3% in group CATB. The hazard ratio of presenting an fUTI within 2 yr in the ATB group compared with that in the CATB group was 10.3 (95% confidence interval: 1.3-82.5). Sixty-four children (70.3%) had a complete follow-up at 2 yr of age., Conclusions: Circumcision significantly decreases the risk of presenting an fUTI in boys with PUVs., Patient Summary: In this report, we compared, in a multicentric trial, the number of febrile urinary tract infections (UTIs) in boys with posterior urethral valves who had either antibiotic prophylaxis alone or antibiotic prophylaxis and circumcision. We found that those who had a circumcision had a significantly lower risk of febrile UTIs., (Copyright © 2021 European Association of Urology. Published by Elsevier B.V. All rights reserved.)

Purpose: Neuroblastoma management in children is multimodal and depends on multiple factors, including the possibility of complete surgical resection. Image-defined risk factors (IDRFs) are used to assess the feasibility of primary surgery. We studied the changes in IDRFs after neoadjuvant chemotherapy for thoracic neurogenic tumors., Methods: We performed a multicenter review of 27 patients presenting with unresectable thoracic neurogenic tumors. Patients received neoadjuvant chemotherapy, according to their risk group. IDRF at diagnosis and before surgery were retrospectively analyzed by a radiologist and a surgeon, blind to the initial assessment. Surgical and oncologic outcomes were reviewed., Results: None of the patients presented MYCN amplification, and 78 IDRFs were identified at diagnosis. Vascular IDRFs were the most frequent, with 28 vascular IDRFs detected in 18 patients, 22 of which disappeared after chemotherapy. Reductions of tumor volume were associated with a regression of IDRFs. Patients undergoing minimally invasive surgery had smaller tumor volumes than those undergoing open surgery, and no vascular IDRF. Two patients received two additional courses of chemotherapy to reduce tumor volume sufficiently for surgery. One patient with ganglioneuroblastoma underwent early surgery due to a lack of response to initial chemotherapy., Conclusion: Tumor volume reduction with neoadjuvant chemotherapy eliminates most IDRF in thoracic neurogenic tumors. Vascular IDRF are rapidly resolved at this site, making surgical resection and minimally invasive surgery possible., (© 2021 Wiley Periodicals LLC.)

Background: The renal transplantation is the best treatment for end-stage renal disease in children. We present the findings of an analysis of our institution's paediatric transplant outcomes comparing recipients under 15 kg, who represent this potentially higher risk group, to those above 15 kg., Methods: We retrospectively identified consecutive paediatric kidney transplants from a prospectively collected database for analysis. We included all recipients under the age of 18 years at the time of transplant between 2006 and 2018 without any exclusion criteria. The primary outcome was death-censored graft survival at 1 year, 5 years and 10 years., Results: 109 paediatric kidney transplants were performed in 100 children. Graft survival in the all population was 98%, 96% and 76% at 1 year, 5 years and 10 years, respectively. Recipient weight below 15 kg was not found to be a risk factor of graft loss. Overall, we found no individual factor to be statistically significantly associated with renal graft lost. The overall complication rate was 16% (18/109) with 12 early complications (11%) and 6 late ones (5%)., Conclusion: Kidney transplantation in children weighing < 15 kg seems safe and offers the same patient and graft survival outcomes as in other (> 15 kg) pediatric recipients with equally low complication rates., (© 2021. Springer-Verlag GmbH Germany, part of Springer Nature.)

In Hirschsprung's disease (HSCR), postoperative course remains unpredictable. Our aim was to define predictive factors of the main postoperative complications: obstructive symptoms (OS) and Hirschsprung-associated enterocolitis (HAEC). In this prospective multicentre cohort study, samples of resected bowel were collected at time of surgery in 18 neonates with short-segment HSCR in tertiary care hospitals. OS and HAEC were noted during postoperative follow-up. We assessed the enteric nervous system and the intestinal epithelial barrier (IEB) in ganglionic segments by combining immunohistochemical, proteomic and transcriptomic approaches, with functional ex vivo analysis of motility and para/transcellular permeability. Ten HSCR patients presented postoperative complications (median follow-up 23.5 months): 6 OS, 4 HAEC (2 with OS), 2 diarrhoea (without OS/HAEC). Immunohistochemical analysis showed a significant 41% and 60% decrease in median number of nNOS-IR myenteric neurons per ganglion in HSCR with OS as compared to HSCR with HAEC/diarrhoea (without OS) and HSCR without complications (p = 0.0095; p = 0.002, respectively). Paracellular and transcellular permeability was significantly increased in HSCR with HAEC as compared to HSCR with OS/diarrhoea without HAEC (p = 0.016; p = 0.009) and HSCR without complications (p = 0.029; p = 0.017). This pilot study supports the hypothesis that modulating neuronal phenotype and enhancing IEB permeability may treat or prevent postoperative complications in HSCR.

The Canadian Inflammatory Myopathy Study (CIMS) is a multicenter prospective cohort recruiting in 8 centers across Canada. One of the aims of CIMS is to conduct and participate in clinical trials in autoimmune inflammatory myopathies (AIM). Conducting clinical trials in rare diseases such as AIM presents challenges. During this symposium, experts in the field presented different solutions to successfully conduct clinical trials in AIM, including the importance of collaboration and careful trial design, as well as training and mentoring of young investigators.

Purpose: Urethral fistula and dehiscence are common after hypospadias surgery. Preoperative androgens have been considered to reduce these complications although this consideration is not evidence-based. Dermatologists have reported the benefits of topical estrogens on skin healing. We investigated whether the preoperative use of topical promestriene could reduce healing complications in hypospadias surgery. Our primary objective was to demonstrate a reduction of healing complications with promestriene vs placebo. Impact on reoperations and other complications, clinical tolerance, bone growth, and biological systemic effects of the treatment were also considered., Methods: We conducted a prospective, randomized, placebo-controlled, double-blind, parallel group trial between 2011 and 2015 in 4 French centers. One-stage transverse preputial island flap urethroplasty (onlay urethroplasty) was selected for severe hypospadias. Promestriene or placebo was applied on the penis for 2 months prior to surgery. The primary outcome was the presence of postoperative urethral fistula or dehiscence in the first year postsurgery. For safety reasons, hormonal and anatomical screenings were performed., Results: Out of 241 patients who received surgery, 122 patients were randomized to receive placebo, and 119 patients received promestriene. The primary outcome was unavailable for 11 patients. Healing complications were assessed at 16.4% (19/116) in the placebo vs 14.9% (17/114) in the promestriene arm, and the odds ratio adjusted on center was 0.93 (95% confidence interval 0.45-1.94), P = 0.86., Conclusions and Relevance: Although we observed an overall lower risk of complications compared to previous publications, postsurgery complications were not different between promestriene and placebo, because of a lack of power of the study or the inefficacy of promestriene., (© Endocrine Society 2020. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

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., (© 2020 Wiley Periodicals, Inc.)

Objective: Evidence suggests an increased risk of cardiovascular (CV) diseases, including acute coronary syndrome (ACS), in idiopathic inflammatory myopathies (IIM). The aim of this study was to investigate the risk of ACS in an incident IIM cohort compared to the general Swedish population., Methods: A cohort of 655 individuals with incident IIM and 6813 general population comparators were identified from national registries. IIM subjects were diagnosed from 2002 to 2011. Followup started at IIM diagnosis and corresponding date in the general population. ACS, CV comorbidities, and CV risk factors were defined using International Classification of Diseases codes. Incidence rates including 95% CI were calculated. Cox proportional hazards models were used to compare the risk of ACS in patients with IIM and the general population. The competing risk of death was accounted for using competing risk regression models., Results: The incidence rate of ACS in IIM was higher than in the general population, particularly within the first year of diagnosis and in older individuals. The overall ACS incidence rate in IIM was 15.6 (95% CI 11.7-20.4) per 1000 person-years, with an HR of 2.4 (95% CI 1.8-3.2) compared with the general population. When accounting for the competing risk of death, the risk of ACS in IIM remained increased with a cumulative incidence of 7% at 5 years compared to 3.3% in the general population., Conclusion: IIM individuals are at higher risk of ACS, particularly within the first year after diagnosis.

The investigation, management and follow-up of paediatric ureteropelvic junction obstruction is not standardized. The Young Pediatric Urology Committee of the European Society of Pediatric Urology interviewed five experts in the field on various aspects of management and compared this with published literature., (Copyright © 2019 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure., Objective: The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure., Design, Setting, and Participants: From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5-37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8-33m]., Intervention: RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction., Outcome Measurements: The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage., Results and Limitations: All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3-30] follow-up., Complications: Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1-3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery., Conclusion: The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy., (Copyright © 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: In children, intermittent catheterization by appendicovesicostomy according to Mitrofanoff is an interesting alternative to the urethral approach. Objective of the study was to evaluate the rate of appendicovesicostomy's specific complications., Method: From 1997 to 2017, data on children treated and followed for an appendicovesicostomy in an academic institution were collected retrospectively. Rates of surgical complications specifically encountered on appendicovesicostomy, time of onset, frequency, and necessity of surgical reinterventions have been reported., Results: Thirty-four patients were operated on and followed for a median of 6.2 years [0.3-24]. Fifty percent had a complication, occurring after a median of 8 months [2-90], and 38% required at least one surgical revision. If complication occurred, adjustment of medical treatment and intermittent catheterization was effective in 12% of patients, endoscopic or over-fascial surgery was necessary in 17% of cases, and under-fascial revision in 21% of cases. Median time to complication was 4 months [1-90] after creation or revision of appendicovesicostomy. Thirty-height percent of difficult channel catheterization were reported, of which 46% were over or under-fascial stenosis. Inaugural urinary incontinence was 18%, and only 9% if using the appendix. At the end, 97% of appendicovesicostomy were continent., Conclusions: Appendicovesicostomy is a high risk of complications and postoperative revisions surgery, in order to have a functional continent channel., Level of Evidence: 4., (Copyright © 2018. Published by Elsevier Masson SAS.)

Background: Efficacy and role of cytoreductive surgery (CRS) and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) remain poorly documented in pediatric tumors., Methods: This retrospective national study analyzed all pediatric patients with peritoneal tumor spread treated by CRS and HIPEC as part of a multimodal therapy in France from 2001 to 2015., Results: Twenty-two patients (nine males and 13 females) were selected. The median age at diagnosis was 14.8 years (4.2-17.6). Seven had peritoneal mesotheliomas; seven, desmoplastic small round cells tumors (DSRCT); and eight, other histologic types. A complete macroscopic resection (CC-0, where CC is completeness of cytoreduction) was achieved in 16 (73%) cases. Incomplete resections were classified as CC-1 in four (18%) cases and CC-2 in two (9%) cases. Fourteen (64%) patients had complications within 30 days from HIPEC, requiring an urgent laparotomy in eight (36%) cases. Thirteen (59%) patients received adjuvant chemotherapy and four (18%) received total abdominal radiotherapy after surgery. Sixteen (72%) patients had relapse after a median time of 9.6 months (1.4-86.4) and nine (41%) eventually died after a median time of 5.3 months (0.1-36.1) from relapse. Six (27%) patients (four mesotheliomas, one pseudopapillary pancreatic tumor, and one DSRCT) were alive and in complete remission after a median follow-up of 25.0 months (5.3-78.2). The mean overall survival (OS) and disease-free survival (DFS) were 57.5 months (95% CI [38.59-76.32]) and 30.9 months (95% CI [14.96-46.77]). Patients with a peritoneal mesothelioma had a significantly better OS (p = 0.015) and DFS (p = 0.028) than other histologic type., Conclusions: In this national series, outcomes of HIPEC are encouraging for the treatment of peritoneal mesothelioma in children., (© 2017 Wiley Periodicals, Inc.)

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Paediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication between different clinicians involved in pediatric urology and nephrology.

Objectives: Primary female epispadias encompasses a spectrum of disease, presenting with a variable degree of incontinence. We hypothesized that although perineal urethrocervicoplasty can be a successful first-line procedure in patients with normal bladder, a more radical reconstruction was necessary to achieve continence in cases lying at the most severe end of the spectrum. Our aim was to assess the results of a surgical management using perineal approach in girls with normal bladder capacity, and Kelly radical soft-tissue mobilization (RSTM) in patients with inadequate bladder, based on the assumption that bladder capacity (BC) is a reliable marker of epispadias severity., Study Design: Prospective inclusion of incontinent girls with female epispadias referred to a single institution. Patients with normal BC were treated with perineal urethrocervicoplasty (PUCP, group 1). Patients with small bladder underwent RSTM (group 2). Follow-up was at 1, 3, 6, and 12 months postoperatively, then annually, including physical examination, renal ultrasound at each visit, continence status, and estimation of functional/maximal BC. The main study outcome was continence status at the age of 5 years or later, if postoperative follow-up was >12 months., Results: From 2006 to 2017, 16 consecutive children were prospectively included in this study, at a median age of 39 months (5-102 months). Seven girls were included in group 1 and underwent PUCP; at the last follow-up, five out of seven were dry by day (4/5 day and night), although three out of five required bladder-neck injection after perineal reconstruction due to stress incontinence. Two patients with persistent incontinence and absence of BC increase after PUCP subsequently underwent RSTM. Eleven patients with low BC (56% [10-94%] of expected BC) were included in group 2 (9 without prior surgery, 2 after PUCP failure). Among the eight evaluable patients, eight out of eight achieved diurnal continence, and 3/8 were fully continent. One girl with obstructive micturition required clean intermittent catheterization., Discussion: The traditional approach of female epispadias based on staged reconstruction (urethroplasty followed by bladder-neck reconstruction) raised concerns regarding the risk of non-physiological obstructive micturition. The perineal approach was suggested as an alternative, with reported diurnal continence rates of 60-80%, but less than 50% of nocturnal continence, presumably in relation with limited bladder capacity. In cases selected within the most severe end of the epispadias spectrum, the Kelly RSTM seems to offer excellent continence rates., Conclusion: A tailored approach to female epispadias, based on perineal reconstruction in favorable cases, and radical soft-tissue mobilization in severe cases, seems to yield good continence outcomes in the long term., (Copyright © 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Competing Interests: Conflict of Interest: None.

Despite recent advances in high-risk neuroblastoma therapy, the prognosis for patients remains poor. In addition, many patients suffer from complications related to available therapies that are highly detrimental to their quality of life. New treatment modalities are, thus, urgently needed to further improve the efficacy and reduce the toxicity of existing therapies. Since antibodies specific for O-acetyl GD2 ganglioside display pro-apoptotic activity against neuroblastoma cells, we hypothesized that combination of immunotherapy could enhance tumor efficacy of neuroblastoma chemotherapy. We demonstrate here that combination of anti-O-acetyl GD2 monoclonal antibody 8B6 with topotecan synergistically inhibited neuroblastoma cell proliferation, as shown by the combination index values. Mechanistically, we evidence that mAb 8B6 induced plasma cell membrane lesions, consistent with oncosis. Neuroblastoma tumour cells treated with mAb 8B6 indeed showed an increased uptake of topotecan by the tumor cells and a more profound tumor cell death evidenced by increased caspase-3 activation. We also found that the combination with topotecan plus monoclonal antibody 8B6 showed a more potent anti-tumor efficacy in vivo than either agent alone. Importantly, we used low-doses of topotecan with no noticeable side effect. Our data suggest that chemo-immunotherapy combinations may improve the clinical efficacy and safety profile of current chemotherapeutic modalities of neuroblastoma.

Objective: To evaluate prenatal ultrasound parameters as prognostic factors for complex and vanishing gastroschisis., Methods: Retrospective multicentre study of 200 gastroschisis over 13 years (2000-2013). Collection of prenatal ultrasound evaluation on maternal and fetal growth parameters, intra- and extra-abdominal bowel and stomach dilation, abdominal wall defect diameter and changes in bowel appearance. Correlation of these factors with the presence of mechanical intestinal complications at birth, named 'complex gastroschisis'., Results: Fifty-two patients (26%) had complex gastroschisis (CG), including ten vanishing gastroschisis. The presence of intra-abdominal bowel dilation at the second (T2) or third (T3) trimester ultrasound was predictive for CG, with odds ratios at 6.69 (95%CI 2.41-18.55) and 4.72 (95%CI 2.16-10.28), respectively, with a cut-off value at the last examination of >19 mm. A small abdominal wall defect diameter was also predictive for CG, with cut-off values of <9.2 mm at T2 and <12.5 mm at T3. Vanishing gastroschisis recorded earlier intra-abdominal bowel dilation diagnosis, associated with a small wall defect and no extra-abdominal dilation., Conclusion: Intra-abdominal bowel dilation and a small abdominal wall defect diameter accurately predict CG and could be a first sign of vanishing gastroschisis when they occur early. © 2016 John Wiley & Sons, Ltd., (© 2016 John Wiley & Sons, Ltd.)

Purpose: Laparoscopic transposition of lower-pole crossing vessels (LPCV) has been described as an effective alternative to dismembered pyeloplasty in selected indications of hydronephrosis, with purely extrinsic vascular PUJ obstruction. We hypothesized that the initial good results of laparoscopic transposition of LPCV in children presenting with pure extrinsic PUJO were sustained at puberty when these children go through statural growth, without inducing significant changes in systemic arterial blood pressure. Hence, we analysed the long-term follow-up of adolescents successfully treated with this technique during childhood, reviewed after they have reached puberty, focusing on the incidence of recurrent symptoms, renal dilatation, and systemic hypertension., Patients and Methods: Early 2015, among a cohort of 70 patients prospectively followed-up since they had undergone laparoscopic transposition of LPCV during childhood (2005-2012), we performed systematic clinical assessment of the 33 adolescent patients (16 years; range 12-22) who had reached puberty age. Assessment focused on clinical examination, arterial blood pressure measurements, and renal ultrasonography (Table)., Results: The median delay since surgery was 67 months (31-113 months). Arterial blood pressure adjusted for age and height was within normal range in all patients. Three patients had occasional episodes of abdominal pain: two of them had normal US (including during pain episodes), one had persistent extra-renal dilated pelvis with no calyceal dilatation. None of them showed obvious clinical characteristics linking the pain to a renal origin. Renal US showed residual SFU grade 2 pelvicalyceal dilatation in 2/33 asymptomatic patients; SFU grade 1 extra-renal pelvis dilatation in 3, and was normal in the remaining. When Doppler analysis was performed, there was no evidence of lower-pole parenchyma perfusion defect., Discussion: In adolescents successfully treated during childhood with transposition of LPCV, there seemed to be no impact of this procedure on systemic arterial blood pressure in adolescents after puberty, nor any evidence of late recurrence of symptoms or hydronephrosis. The main limitation of the present study relies in its retrospective nature, the limited sample size, and the obvious difficulty in adequate selection of candidate patients to this technique. The present experience however reinforces the hypothesis that a vast majority of children can be definitely cured with transposition of LPCV when they represent the sole aetiology of obstruction., Conclusion: In the long-term follow-up, most adolescents successfully treated during childhood by laparoscopic transposition of LCPV for PUJ extrinsic obstruction remain asymptomatic, with normal arterial blood pressure, and normal renal ultrasound when they reach puberty., (Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Clear cell sarcoma of the kidney (CCSK) is a rare tumor that is diagnosed most often in children between 2- and 4-years-old of age. Usually, patients with CCSK are treated in international study for intrarenal tumors, preferentially Wilms tumor, according to bad histopronostic group. The purpose of this paper is to review the most important features in 2015 about epidemiology, radiology, anatomopathology and genetic of CCSK, and above all a synthesis about successive treatment strategies with their results. Second most common pediatric renal tumor in children less than 5-years-old, its prognosis has improved dramatically in recent years with the use of anthracyclines., (Copyright © 2016 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Unlabelled: The radical soft-tissue mobilization (RSTM) technique has been proposed as an alternative for staged reconstruction of bladder exstrophy. In this video, we demonstrate the successive steps of this technique., Methods: A newborn male with classic bladder exstrophy underwent bladder closure without pelvic osteotomies at birth; he then underwent RSTM at 1 year of age., Technique: Mobilization: The RSTM involved simultaneous intra-pelvic and perineal dissection, aiming at full mobilization of potential urethral sphincter striated structures, levatores ani muscles, pudendal pedicles and corpora cavernosa from their attachment on the pubic bones. From the perineal approach, a periosteum incision was performed on the antero-medial aspect of the pubic bones, and the periosteum was peeled from the ischio-pubic ramus, lifting the corpora from the bone and providing control on the pudendal pedicles. The attachments of all striated muscular structures on the lower aspects of the posterior corpora (transverse perinei, bulbo-spongiosus, ischio-cavernosus muscles) were released to allow adequate re-approximation around the reconstructed urethra. Reconstruction: After ureteric reimplantation, a funnel-shaped cervicoplasty was performed and continued by urethroplasty over an 8-Fr catheter. The muscular structures mobilized from the corpora and the bones were wrapped around the neo-urethra, which was transposed ventrally in the scrotal position, creating a temporary hypospadiac urethrostomy. After abdominal wall closure, the corpora were then de-rotated, and secured to the neo-symphisis with non-absorbable sutures, and the penile skin shaft was reconstructed., Results: At the 12-month follow-up, the boy was asymptomatic, still in nappies, with non-dilated upper-tracts on renal ultrasound, and dryness intervals of 90 min., Conclusion: Radical soft-tissue mobilization allows a tension-free bladder-neck reconstruction, and may favour development of physiological continence mechanisms independent of the inevitable pubic symphisis re-enlargement. Creation of temporary hypospadias is most often needed to maximize penile length. This technically demanding procedure requires thorough knowledge of detailed anatomy of the bladder exstrophy pelvis, as demonstrated in this video., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) are serious abdominal conditions that affect preterm infants but are poorly understood. This single-center retrospective study was performed to identify the factors associated with NEC and SIP., Methods: This study involved 3,464 preterm infants recruited over 16 years. A total of 136 NEC and 24 SIP were analyzed and adjusted odds ratios (aOR) were determined by logistic regression., Results: Compared with the controls, NEC was associated with a Z-score for birth weight lower than -1 (aOR = 2.1 [1.1-3.9], p = 0.02) and lower than -2 (aOR = 4.4 [1.8-10.4], p < 0.01). NEC was associated with gestational ages of less than 31 weeks and with breech presentations (aOR = 1.5 [1.0-2.3], p = 0.03). In contrast, compared with the controls, SIP was associated with gestational ages of less than 26 weeks (aOR = 17.4 [3.1-96.2], p < 0.001) and multiple pregnancy (aOR = 2.9 [1.2-6.9], p = 0.02). Rates of mortality and cerebral lesions were higher in patients with NEC (25.0 and 13.2%, respectively) than with the controls (10.1 and 6.9%, respectively) and similar between patients with SIP (12.5 and 8.3%, respectively) and the controls. CONCLUSIon: NEC and SIP differed in risk factors and prognosis. Early distinction of SIP from NEC could impact on surgical decision., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: For the treatment of ureterovascular pelviureteric junction obstruction (PUJO), transposition of lower pole crossing vessels (LPCV) has been described as an alternative to dismembered pyeloplasty., Purpose: To report on the long-term follow-up of children after laparoscopic transposition of LPCV., Methods: A retrospective analysis of 70 children consecutively treated by laparoscopic transposition of LPCV. Candidate patients were selected on the basis of clinical history, renal ultrasound (US), and pre-operative mercaptoacetyltriglycine (MAG-3) scan. Selection criteria included: presence of LPCV with SFU Grade 1-2 hydronephrosis, impaired drainage on MAG-3 and intraoperative normal pelviureteric junction (PUJ) and ureter peristalsis. Thinned parenchyma, impaired renal function, or history of prenatal hydronephrosis were not considered as exclusion criteria. Children were clinically followed up with US and MAG-3 scan. Success was defined by symptom resolution with improvement in hydronephrosis., Results: Seventy children, aged 8.3 years (range 2.75-16.0), were selected. Procedures were performed through transperitoneal laparoscopy (n = 42) or were robotic-assisted (n = 28). Operative time was 120 min and length of hospital stay was 2 days. The outcome was successful in 67/70 patients (96%), with a median follow-up of 52 months (range 13-114). There were three failures in children who eventually underwent dismembered pyeloplasty for a symptomatic, undiagnosed, intrinsic PUJ obstruction. Two of them had been postnatally followed for a resolving prenatally diagnosed hydronephrosis. Three children became free of symptoms, had improved hydronephrosis, but still showed impaired drainage on MAG-3 and are being closely followed up., Discussion: Although this procedure proves to have long-term efficiency in selected indications, the main challenge is to intraoperatively ascertain the absence of associated intrinsic stenosis. Objective criteria remain difficult to establish, but intraoperative findings, including dependent, funnel-shaped, normal-looking PUJ with decreasing hydronephrosis after pelvis and LPCV mobilisation, and efficient peristalsis across the PUJ under intraoperative diuretic test, represent a low likelihood of associated intrinsic stenosis. Ipsilateral impaired renal function doesn't seem to be associated with an adverse outcome. In contrast, a prenatal history of mild or self-resolving hydronephrosis in a patient later presenting with intermittent dilatation, raises the suspicion of associated intrinsic PUJ obstruction, as it is associated with a higher risk of failure., Conclusion: With a long postoperative follow-up, the robotic-assisted or laparoscopic vascular hitch procedure has been successful in treating a selected group of children with obstructive LPCV, and represents a safe and reliable alternative to standard dismembered pyeloplasty in the absence of intrinsic PUJO suspected on prenatal US., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Primary endobronchial tumors are rare in children and they include a broad spectrum of lesions. The aim of this study was to determine the characteristic features, treatments and outcomes of these tumors. We report a retrospective analysis of all patients treated for endobronchial tumor in nine French hospitals between 1990 and 2010 and a comparison of the results with those reported in the medical literature. Twelve tumors were reported: five low grade muco epidermoid carcinomas, two inflammatory myofibroblastic tumors, two hemangiomas, one anaplastic large cell lymphoma, one carcinoid tumor, and one juvenile xanthogranuloma. The mean age of the patients was 7.5 ± 3.5 years. The most common sign revealing the disease was persistent atelectasis or recurrent pneumonia (eight cases). The other revealing signs were a persistent bronchospasm (three cases) and hemoptysis (one case). The clinical presentation, biology, serum tumor markers, and chest X-ray abnormalities were not specific to a particular histological diagnosis. Chest CT scan revealed the presence of an endobronchial tumor in 11 cases. Nine tumors could be diagnosed from a biopsy obtained by video endoscopy. Complete surgical resection was performed in seven patients. Bronchoscopic removal was performed in five cases and was successful in three. There were no deaths. Endobronchial tumors are rare in childhood and their histology is diverse. Chest CT scan and per-endoscopic endobronchial biopsies are required for diagnosis, when possible. Surgical or endoscopic treatment should be discussed by a multidisciplinary team. Despite the multiple etiologies, the prognosis of these tumors is good if diagnosis is early and if resection is complete. Long-term recurrences have been described, so long-term follow-up of these children is recommended., (© 2014 Wiley Periodicals, Inc.)

Objective: The aim was to report a multicentric study with a longer follow-up to evaluate the laparoscopic radical nephrectomy in children with renal cancer., Material and Methods: This was a retrospective multicentric study, from October 2005 to January 2012, of children who underwent a laparoscopic radical nephrectomy for small renal malignant tumors., Results: Seventeen children were included in this study. Sixteen underwent chemotherapy before surgery according the SIOP (Société Internationale d'Oncologie Pédiatrique) protocol and one was treated by surgery only for a carcinoma. All except one could be treated by laparoscopy; the biggest tumoral size was 8 cm. The median hospital stay was 3 days (2-10). The pathologic examination showed 15 Wilms' tumors, one clear cell sarcoma and one TFE3 renal cell carcinoma. With a median follow-up of 42 months (range 12 and 77 months) after laparoscopic radical nephrectomy, 15 children had no oncological complications (port site or local recurrence, pulmonary metastasis) and one had a local recurrence without intraoperative tumoral rupture. The child with TFE3 renal cell carcinoma died 4 years after surgery from brain and lung metastases without local recurrence. No small bowel obstruction occurred., Conclusions: Radical nephrectomy in children for Wilms' tumor or other renal cancer can be safely performed laparoscopically and our indications can be summarized, for trained laparoscopic surgeons, by small tumors under about 8 cm diameter, especially without crossing the lateral edge of the vertebra on the CT scan at the time of surgery., (Copyright © 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)