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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.

Introduction Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. Materials & Methods: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. Results A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. Conclusions MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.

To report long-term results of artificial urinary sphincter implantation for urinary incontinence due to intrinsic sphincter deficiency in children.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.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.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.

International audience; 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.Keywords: Complications; Dysphagia; Esophageal atresia; Esophageal replacement; Gastro-esophageal reflux disease; Long gap esophageal atresia; Midterm outcomes.

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. 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. 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%). Kidney transplantation in children weighing 15 kg) pediatric recipients with equally low complication rates.

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.

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.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.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%).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.

Introduction: Fungus ball (FB) is a rare condition that often requires surgical management.1 Percutaneous nephroscopy is rarely described in children to remove FB.2 We report the case of a...

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.

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 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.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.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%).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.

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.

Extended Summary 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.

Summary 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.

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

International audience; Sialic acid O-acetylation is a developmentally regulated modification of gangliosides implicated in ontogeny and tumor progression. Their existence has been underestimated in the past because of their alkali-labile nature and their transient expression. New data indicates, however, that O-acetylated gangliosides perform important function in tumor malignancy. Best studied O-acetyl-GD3 blocks the pro-apoptotic activity of GD3 and promotes survival of cancer cells. In acute lymphoblastic leukaemia cells, O-acetyl-GD3 expression level also correlates with survival and drug resistance. The recent identification of the enigmatic O-acetyltransferase opens new experimental approaches for designing novel effective therapeutics targeting drug-resistant cancer cells in acute lymphoblastic leukaemia. In addition, O-acetylated gangliosides expressed at the tumor cell surface are accessible for specific monoclonal antibodies to inhibit cell growth, to induce apoptosis, and to inhibit tumor metastasis formation. Thus, passive immunotherapy using murine or murine/human chimeric monoclonal anti-O-acetylated ganglioside antibodies are currently being investigated. Particularly, targeting of O-acetyl-GD2 could reduce the acute toxicities currently associated with anti-GD2 therapeutic antibodies. This review summarizes the molecular mechanisms involved in the biosynthesis and the expression of O-acetylated gangliosides and presents the new experimental approaches that allow the characterization of their importance in tumor progression. The different strategies used by different teams to develop specific monoclonal antibodies against these poorly immunogenic glycolipids for therapeutic application are also discussed.

Resume Le sarcome a cellules claires (CCSK) du rein est une entite tumorale rare diagnostiquee chez des enfants le plus souvent entre 2 et 4 ans. Les patients avec CCSK sont traites dans les protocoles internationaux de prise en charge des tumeurs renales, dans les groupes d’histopronostic defavorable. L’objectif de cet article est de presenter les points essentiels en 2015 concernant les donnees epidemiologiques, radiologiques, anatomopathologiques et genetiques de ce type tumoral, ainsi qu’une synthese de l’evolution de la prise en charge therapeutique des enfants porteurs d’un CCSK avec les resultats des etudes successives. Seconde tumeur du rein de l’enfant de moins de 5 ans, son pronostic a ete drastiquement ameliore ces dernieres annees grâce a l’introduction des anthracyclines.

Objectifs La chirurgie percutanee est le traitement recommande des calculs > 2 cm car elle permet d’obtenir un taux de « stone free » eleve. En France, la chirurgie percutanee pediatrique reste peu developpee notamment du fait d’un taux de complications eleve. La miniNLPC semble reduire le taux de complications. Nous avons voulu rapporter notre experience concernant la miniNLPC dans la prise en charge des calculs > 2 cm. Methodes Nous avons analyse de maniere prospective toutes les miniNLPC realise de mars 2018 a decembre 2019. Tous les patients âges de moins de 18 ans avec un calcul de plus de 2 cm ont ete operes d’une miniNLPC. Le diametre de travail utilise etait entre 8,5 et 14,5 French. La chirurgie etait realisee en position de Valdivia modifie. Le stone free etait considere comme l’absence de residu > 4 mm sur l’imagerie de controle a 4 mois de la chirurgie. Resultats Huit procedures ont ete realise chez 6 patients dont 1 patient ayant 2 procedures pour le meme rein et 1 patient ayant eu un traitement bilateral. L’âge moyen etait de 8 ans (2 ;18) et le poids moyen de 21,5 kg (12 ;36). La taille cumulee moyenne des calculs etait de 40,4 mm dont 5 calculs coralliformes. Le taux de stone free apres une procedure etait de 75 % (6/8) apres une procedure et de 100 % apres 2 procedures. La duree moyenne d’intervention etait de 180 min (80 ;328). La duree d’hospitalisation moyenne etait de 5,4 jours et le suivi moyen a ete de 17,7 mois. Il y a 2 complications > Clavien 3. Une patiente de 2 ans a presente un uro-sepsis a j0 (Clavien 4a) puis un abces renal (Clavien 3) draine a j21 ( Fig. 1 , Fig. 2 , Fig. 3 ). Conclusion D’apres notre serie, la chirurgie percutanee pediatrique permet d’obtenir un taux eleve de stone free associes a un faible taux de complications malgre des tailles de calculs eleves. Nos resultats sont comparables a ceux de la litterature.

The Wilms’ tumour (synonym: nephroblastoma) represents more than 90% of the paediatric renal tumours. Other possible aetiologies comprise clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumour of the kidney (MRTK), renal cell carcinoma (RCC), and congenital mesoblastic nephroma (CMN).

Summary 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. Table . Primary delayed closure + Kelly Redo closure + Kelly n 20 7 Males/females 13/7 5/2 Age (mo.) 3.0 [0.5–37] 10 [8–33] Bladder dehiscence 0 0 Urethral stenosis 1/20 3/7 Urethral fistula 3/20 2/7

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.

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.

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.

Introduction The success of modern pediatric surgery (PS) management has led to the recommendation of regional organization and coordination of PS departments. Although small units may obtain excellent results, there is evidence that a concentration to larger units is advantageous. In France, the centralization of PS has been discussed for the past 15 years. A new organization was finally achieved in 2010. The intent was to assure children prompt access to the level of care appropriate to the degree of their pathologies to improve the outcomes and reduce morbidity and mortality. Methods One of the first pertinent governmental measures was an attempt to centralize the PS departments and encourage the development of connection between specialized PS care and routine PS unit. In this review, we report this coordination through the analysis inside two regional French areas. Results Paris is the first example that illustrates the network between four specialized PS departments and eight local hospitals. The aim of this connection was to improve the safety and quality of pediatric surgical management during the night for emergency issues and in the case of neonatology. The second example is the network that coexists in the west part of France through monthly video conferencing sessions and subspecialization (pediatric urology, pediatric oncology, and pediatric liver surgery). Moreover, a national program aimed at improving the management of patients with rare diseases since 2005. For instance, biliary atresia and anorectal malformation centers are located in two academic centers in Paris and the esophageal atresia center is in Lille. These expert centers allowed performing numerous studies along with international publications, thereby offering a better vision of the natural story of diseases to improve care. Finally, this network is emphasized during medical education from PS residency to the fellowship when trainees have to spend at least 1 year in a local hospital. Conclusion The European legislation calls for multidisciplinary centers treating children with rare diseases and proposes clear and demanding quality criteria. The French experience illustrates some interesting and encouraging aspects of the current trends in PS care. Our results strongly suggest that centralization, specialization, and connections between specialized and routine PS departments can greatly improve management of and outcomes in children treated in our units.

Resume Objectifs La pratique pediatrique est un exercice difficile ou il faut apprendre a distinguer parmi une multitude de recours pour des motifs benins des situations cliniques rares mais potentiellement graves. Notre but etait d’evaluer les connaissances des etudiants entrant en 3e cycle de medecine generale sur ces situations. Methodes Cent trois etudiants ont ete evalues sur les « feux tricolores » sous forme de 103 questions a choix multiple (QCM) a 2 modalites (vrai/faux). Ces feux tricolores avaient ete definis par des praticiens pediatriques du centre hospitalier universitaire (CHU) et des enseignants de medecine generale de Nantes comme des fondamentaux de la pediatrie : situations pieges, diagnostiques ou therapeutiques a ne pas manquer. Le degre d’urgence etait defini par la couleur, le rouge correspondant aux urgences vitales immediates. Resultats Trente-six pour cent des QCM (n = 103) ont eu un taux de bonnes reponses inferieur a 75 %. Trente-deux pour cent des QCM concernant des situations avec urgence vitale (« feux rouges ») (n = 37) ont eu un taux de bonnes reponses inferieur a 75 %. Quinze pour cent des questions (n = 103) ont eu un taux de bonnes reponses inferieur a 50 %, la moitie concernant des « feux rouges » (meningite, decouverte de diabete, choc). Les questions concernant les nourrissons (n = 24) ont eu significativement moins de bonnes reponses (taux de bonnes reponses inferieur a 50 % de 29 % versus 10 %, p = 0,047) Conclusions Certaines situations d’urgence ou concernant les nourrissons ne sont pas maitrisees par la majorite des etudiants. Il est donc indispensable de mener une reflexion sur l’optimisation de l’enseignement de la pediatrie au second cycle.

Telomeric associations (TAs) are fusions between two telomeres of two different chromosomes without visible loss of chromosomal material. Constitutional telomeric associations are rare chromosomal anomalies. We report on the cytogenetic and molecular analyses of a TA involving chromosomes Y and 7 in a child with a female phenotype. Prenatal cytogenetic analysis showed a 45,X chromosome complement in all cells. No fetal abnormality was identified at ultrasound examinations and the pregnancy went to term. During childhood, the proband had gonadal dysgenesis but no other phenotypic manifestations of Turner syndrome. Molecular genetic analyses showed the presence of genomic DNA of the SRY gene without any mutation. Karyotyping and fluorescent in situ hybridization (FISH) analyses on blood showed two cell lines: one cell line with a TA involving chromosomes Y and 7 [46,X,tas(Y;7)(p11.32;q36.3)] and a second cell line with a 45,X pattern. A human pantelomeric repeat TTAGGG probe hybridized to the junction of the TA within the derivative chromosome. FISH and array comparative genomic hybridization (aCGH) analyses demonstrated that tas(Y;7) occurred without detectable loss of any sequence at the derivative chromosome. SNP array analysis excluded an uniparental isodisomy of chromosome 7. Knowing more about TAs will help geneticists to deliver accurate genetic counseling.

To evaluate prenatal ultrasound parameters as prognostic factors for complex and vanishing gastroschisis.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'.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 of19 mm. A small abdominal wall defect diameter was also predictive for CG, with cut-off values of9.2 mm at T2 and12.5 mm at T3. Vanishing gastroschisis recorded earlier intra-abdominal bowel dilation diagnosis, associated with a small wall defect and no extra-abdominal dilation.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 WileySons, Ltd.

Purpose Minimally invasive adrenalectomy (MIA) is the criterion standard for removal of small adrenal tumors in adults. The purpose of this review was to determine the place of MIA in children. Methods The authors conducted a systematic review of the pediatric and adult literature about MIA, focusing on the technique and indications. Results Minimally invasive adrenalectomy appears superior to open adrenalectomy for small tumors. The potential advantages of MIA are appealing for postoperative pain, risk of intestinal obstruction, and quality of scars. The most common approach is the transperitoneal lateral laparoscopy, which allows for a large working space. For small tumors or for bilateral adrenalectomy, the prone retroperitoneoscopy is a promising new technique. In children, the learning curve is an issue because the indications are rare. The most common indication is neuroblastoma without image-defined surgical risk factors. The incidence of local recurrence is low, but the follow-up is short in most cases. Conclusions Minimally invasive adrenalectomy is promising for removal of small adrenal tumors. Long-term follow-up is required to evaluate the efficacy of MIA in neuroblastomas. Benign diseases are excellent candidates for this minimally invasive technique.

Resume Objectifs Comparer 2 protocoles de prise en charge d’une premiere infection urinaire (IU) febrile pour le reflux vesico-ureteral (RVU) de haut grade et pour le cout. Patients et methodes Etude menee chez des enfants de moins de 16 ans, consultant pour une premiere IU febrile. La premiere cohorte (2005) a ete prise en charge selon les anciennes recommandations (traitement intraveineux et cystographie pour tous les enfants Resultats Le nombre d’enfants inclus a ete de 225 en 2005 et 116 en 2006. Il n’y avait pas de difference significative en terme de diagnostic de RVU de haut grade (6,2 % des enfants en 2005 et 9,5 % en 2006, p = 0,274) et de recidive (5,3 % en 2005 et 8,6 % en 2006, p = 0,237). Le cout moyen de prise en charge n’etait pas different (2235 € en 2005, 2256 € en 2006, p = 0,902). Pour les enfants de plus de 6 mois, on notait un cout moyen inferieur en 2006 (1882 € en 2005 versus 1292 € en 2006, p = 0,0042). Conclusion Le protocole stratifie selon l’âge et le taux de PCT ne semble pas sous-estimer le depistage des RVU de haut grade. Le cout de prise en charge pour les enfants de plus de 6 mois a pu etre significativement diminue en 2006. La prise en charge d’une premiere IU febrile est discutee.

Patients Eleven patients with 46,XY PGD were divided into two groups. Six symptomatic girls (group 1) were referred for amenorrhea (n = 3), gonadal tumor (n = 2) or campomelic dysplasia (n = 1). Five asymptomatic screened patients (group 2) were diagnosed as 46,XY PGD after familial investigation of the two probands with gonadal tumor. Bilateral gonadectomy was performed in all patients. Results In group 1, pathologic examination revealed an association of dysgerminoma with gonadoblastoma (n = 2), bilateral gonadoblastoma (n = 2) and streak gonads (n = 2). Prophylactic gonadectomy in asymptomatic patients (group 2) also showed asymptomatic dysgerminoma with gonadoblastoma (n = 1), bilateral gonadoblastoma (n = 2) and streak gonads (n = 2). Conclusions A gonadal tumor arising in a girl with pubertal delay may be related to dysgenesis of the gonad. Primary amenorrhea or diagnosis of dysgerminoma should warrant karyotype, and familial study if 46,XY PGD is found. Considering the high incidence of gonadoblastoma and the early occurrence of dysgerminoma, early bilateral gonadectomy is recommended.

Context: The SRY gene encodes a transcription factor responsible for initiating testis differentiation. Mutations in SRY almost always result in XY sex reversal with pure gonadal dysgenesis and an increased risk of gonadal tumor. Most of these mutations are de novo, affecting only one individual in a family. Only a small subset of mutations is shared between a phenotypically normal father and one or more of his affected children. Incomplete penetrance and somatic mosaicism are two hypotheses that may explain a normal phenotype in a father carrying a SRY mutation. Patients and Results: We describe a family with two sisters with XY sex reversal and pure gonadal dysgenesis and a phenotypically normal brother. A novel constitutional frameshift SRY mutation was identified in both sisters and was absent in the brother. The single base pair deletion (c.71delA) led to a premature stop codon in position 60 of the protein, removing entirely the high-mobility group domain and the DNA-binding domain of SRY. The father of the three children presented with hypospadias; cryptorchidism; testicular seminoma and oligoasthenozoospermia, an association termed testicular dysgenesis syndrome (TDS); and the SRY mutation in a mosaic state in the peripheral blood and the tumor. Conclusions: This observation of somatic and germinal mosaicism for a SRY mutation may explain the variable penetrance in some familial gonadal dysgenesis. Importantly, the present report is the first one describing the association of SRY mutation in a male with TDS. This suggests that mutations in a sex-determining gene may contribute to the pathogenesis of TDS. A fertile man is described with hypospadias, cryptorchidism, testicular seminoma, and oligoasthenozoospermia, and a mosaic SRY mutation.

We report a case of pleuropulmonary blastoma (PPB) prenatally diagnosed at 32 weeks of gestation. The suspected diagnoses was a congenital cystic adenomatoid malformation of the lung (CCAM). The excision of the malformation was performed through a thoracotomy at 4 months of age. On pathological examination, the presence of focal stromal hypercellularity (cambium layer) in the cyst wall was consistent with both the diagnosis of type IV CCAM or grade I PPB. The presence of nodules of immature cartilage and the positivity of particular immunohistochemical staining (indicate which staining came positive) confirmed the diagnosis of grade I PPB. Pleuropulmonary blastoma is a rare malignant tumour in children, sometimes difficult to differentiate from the type IV CCAM as they share similar circumstances of diagnosis, radiological and macroscopic features. The pathological examination does not always allow to differentiate them clearly, implying the necessity of an extended medical supervision for the children carrying those border lesions. Because of the risks of malignancy and of the risks of septic complications, surgical resection during the first year of life is often advocated. Therefore, prenatal detection is essential to allow adequate early management and complete resection of these lesions.

Background Several authors have reported on laparoscopic pyloromyotomy (LP) since the technique was originally described in 1990, but its benefits remain unproven. We performed a randomized controlled trial comparing LP to open circumumbilical pyloromyotomy (OP) for hypertrophic pyloric stenosis. Methods In a prospective study, 102 infants with pyloric stenosis were randomly assigned to either LP (n = 50) or OP (n = 52). The primary outcome measure was the incidence of postoperative vomiting; the secondary parameters were the durations of surgery and anesthesia, surgical complications, and postoperative pain. All infants were managed according to standardized procedures regarding general anesthesia, surgical technique, postoperative analgesia, and feeding regimen. Parents, carers, and assessors responsible for the postoperative evaluation were blinded for the technique used. Results There was no difference in the incidence of postoperative vomiting between the 2 groups. The overall incidence of complications was similar, but the durations of surgery and general anesthesia were significantly longer in the LP group than in the OP group ( P = 10 −4 and P = .02, respectively). There were 3 cases of incomplete pyloromyotomy after laparoscopy, requiring a repeat procedure. Conclusions Laparoscopic pyloromyotomy does not decrease the incidence of postoperative vomiting, has a similar complication rate compared with the open umbilical approach, but may expose patients to a risk of inadequate pyloromyotomy.

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.

Summary 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.

El estudio urodinamico del nino es una exploracion dificil de realizar e interpretar debido a la evolucion de los valores normales segun la edad y la ausencia de referencias. Por tanto, solo se solicita cuando los estudios clinicos y radiologicos no permiten definir el trastorno de la miccion. Este estudio debe efectuarse en un laboratorio con experiencia en el tratamiento de los ninos. La medicion del flujo y la cistomanometria se llevan a cabo segun las reglas pediatricas. En cambio, la medicion del perfil de la uretra es muy dificil de conseguir, ya que el retiro de la sonda a lo largo de la uretra provoca contracciones reflejas del perine. Las indicaciones mas destacables son: las vejigas neurogenas, los trastornos de la miccion, las infecciones urinarias, las malformaciones congenitas del perine y los tumores de la pelvis. Al igual que en el adulto, la urodinamica es una ayuda inapreciable para la eleccion del tratamiento

Neonatal surgical antibioprophylaxis must take into account the specificities of this life period, as immunological immaturity, microbiotal implantation and antibiotic treatment pharmacokinetics properties. Very few data are available, but it seems that coagulase negative Staphylococcus (CNS) is the most reported bacteria, in intestinal tract at the time of surgery as well as in surgical site infections. Usual vertical transmission bacteria as S. agalactiae or E. coli K1 are rarely documented. The antibioprophylaxis treatment must consider the age and the environmental context of the newborn; first or second generation cephalosporins sometimes in association with vancomycin seem appropriate in this context. It is now urgent to conduct clinical trials in order to validate theses propositions.