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To assess the contribution and impact of fetal magnetic resonance imaging (MRI) in managing fetal gastroschisis.We conducted an observational retrospective study of gastroschisis patients at three fetal medicine centers from 2008 to 2019. The primary endpoint was the number of cases in which the MRI provided relevant information related to gastroschisis.A total of 189 patients were included, and our study group included 38 patients who underwent MRI. For the eight patients with suspected gastroschisis, MRI confirmed the diagnosis. In six cases, it provided additional relevant information (spiral turn, intestine ischemia, and bowel size discrepancy). For the 17 patients with ultrasound signs of additional gastrointestinal anomalies, MRI detected one case of unidentified complex gastroschisis on sonography. For the 13 patients undergoing routine MRI, no significant information was obtained. One termination of pregnancy and one fetoscopy were performed a few days after the MRI results. There was no subsequent follow-up or additional bowel complications to support management.Although MRI did not change the management of pregnancies complicated by fetal gastroschisis, patients presenting with fetal gastroschisis with intraabdominal bowel dilatation could benefit from MRI to allow for more precise prenatal counseling to predict postnatal intestinal complications before birth.

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.

Management of teenagers with primary spontaneous pneumothorax (PSP) is not consensual. We report our experience over an 11-year period.For each patient under 20 years hospitalised with PSP from 2008 to 2018, demographic data, smoking habits, clinical presentation, hospitalisation unit, radiological management and its results, therapeutic management (observation, needle aspiration, chest tube drainage and surgery), complications, length of stay, given advice at discharge and recurrence were collected.Seventy patients were included in different paediatric or adult surgery or pulmonology wards (82.9% boys; 16.8 ± 1.7 years; one severe presentation; 18/58 smokers). Chest CT-scan (n = 42/70, 60%) revealed blebs/bullae in 18/39 examinations (46.2%). Treatment consisted of observation (14/70, 20%), needle aspiration (2/70, 2.9%), chest tube (53/70, 75.7%) and video-assisted thoracoscopy surgery (27/70, 38.6%). Half patients with interventional procedure presented complications. A median of 10 chest X-rays was noted during a median stay of 8 days. Advice concerning sport practice, flying, smoking, etc., was variably delivered. PSP recurrence concerned 35/70 patients (50%) without identified predictive factors.Compared to recent recommendations of a more conservative approach, chest CT-scan and interventional strategy are overused in our teenagers with PSP. Observation, more or less needle aspiration, should be clearly the first-line treatments.

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

Purpose To determine the quality of life and neuropsychological development of school-aged children with Hirschsprung's disease. Methods In this observational monocentric study, a multidisciplinary team prospectively assessed quality of life, neuropsychometric development and bowel functional outcomes. This study was registered on ClinicalTrial.gov ( NCT03406741 ). Kidscreen and VSP-A questionnaires assessed the quality of life and were compared to the reference population (Eurostat database). Intelligence, attention and executive functions, perceptual organization and memory were evaluated using the Wechsler Children's Intelligence Scale, the NEuroPSYchological assessment, and the Rey figure test. Bowel functional outcomes were obtained using the Krickenbeck score. Results Fifteen patients were included, with a mean age of 10.25 years. The children's Kidscreen-assessed quality of life index was higher than the reference population (p = 0.01). The Full-Scale Intelligent Quotient was dissociated in 64% of children. The Perceptional Reasoning Index and the Processing Speed Index were observed at lower levels. There were no disturbances in executive functions. A satisfactory bowel functional outcome was noted in 46.7% of children. Conclusion Children with Hirschsprung's disease have been shown to have subtle decreased performances in some areas of intelligence. Performing a neuropsychological assessment upon entering elementary school could help to detect these specific learning disabilities. Levels of evidence Level II, prognosis study.

Summary Introduction Paediatric recurrent epididymitis is frequently observed in several urogenital conditions, and may result in deterioration of long-term fertility. The management of recurrent epididymitis is still a therapeutic challenge for paediatric urologists, and as yet there is no consensus for treatment. Objective To present a minimally invasive endoscopic approach for the treatment of recurrent epididymitis (more than three episodes). Patients and methods Eleven boys were referred with a history of recurrent epididymitis in a context of urogenital malformations. All children underwent endoscopic transurethral injection. Dextranomer/hyaluronic acid was injected around the ejaculatory ducts at the verumontanum (Summary Fig.). The medical records and outcomes of the patients were retrospectively reviewed. Results Of the 11 boys, two (18%) had a history of bladder exstrophy, three (27%) anorectal malformation, two (18%) peno-scrotal hypospadias, two (18%) posterior urethral valves, one (9%) seminal vesicle cyst, and one (9%) urethral stricture. The median age at injection was 3.75 years (range 8 months–14.7 years). Endoscopic injection effectively prevented recurrence in eight patients (73%) with a mean follow-up of 3 years (range 6 months–8.8 years). The mean injected volume was 0.7 ml/session. No perioperative complications were recorded. Vas clipping was performed in three patients after unsuccessful injections. Discussion The current discussion for management of recurrent epididymitis is mainly based on vas clipping. Endoscopic injection in the verumontanum could offer several potential advantages over vas clipping; moreover, it is easy to perform for an urologist who usually uses endourological approaches. It is believed that only Kajbafzadeh et al. have reported their experience with endoscopic injection in the verumontanum in seven patients with structural anomalies, and they had a 42% success rate. Similarly, the current study did not observe perioperative or postoperative complications. Conclusion In this series, endoscopic injection of the verumontanum was considered to be a safe and effective treatment in almost 73% of children with recurrent epididymitis. It did not result in perioperative complications and not contraindicate a subsequent surgical procedure such as vas clipping.

Objective To compare the long-term efficacy of Young-Dees bladder neck reconstruction (YDBNR) alone versus YDBNR plus bladder neck injection (BNI) in patients with urinary incontinence caused by urethral sphincter insufficiency. Patients and Methods Between 1987 and 2006, we assessed the continence rates obtained with YDBNR and BNI as a supplementary treatment for persistent outlet insufficiency in patients with neurogenic bladder (group 1, n = 35) and bladder exstrophy (group 2, n = 20). Median postoperative follow-up was 16 years (range: 5-29). Results A total of 55 children (23 males and 32 females) underwent YDBNR at the median age of 7.6 years (range: 1.9-17.25). Only 10 patients (18%) were considered continent after the isolated YDBNR; 17% (n = 6/35) from group 1 and 20% (n = 4/20) from group 2 (p = 1). Because of unsatisfactory results after YDBNR, 81.8% (n = 45/55) received BNI. Fifteen patients (33%) became continent with an average of 2.29 injections (±1.1); 44.8% (n = 13/29) from group 1 and 12.5% (n = 2/16) from group 2. A significant difference was found on comparing the social continence rate attained with YDBNR plus BNI between patients from group 1 and group 2 (54% vs 30%, P = .04). The difference between males and females in terms of continence rates was not statistically significant. Conclusion Long-term results of YDBNR are modest. BNI does increase the results of YDBNR, especially in patients with neurogenic bladder.

Objective The objective of this study was to evaluate the accuracy of fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient (ADC) determination to predict postnatal renal function (nadir creatinine at 1 year and eGFR) of men with posterior urethral valves (PUV). Methods Between 2003 and 2014, 11 MRI were performed on fetuses (between 28 and 32 weeks) in whom second trimester sonography suggested severe bilateral urinary tract anomalies, suspected of PUV. Results The ADC of the 11 fetuses ranged from 1.3 to 2.86 mm2 s-1 (median = 1.79 mm2 s-1 , normal range for fetal kidney: 1.1-1.8). Two pregnancies with ADC > 2.6 mm2 s-1 were interrupted; the autopsy confirmed PUV and Potter syndrome. For the remaining nine babies, the follow-up was 5.4 years (0.8-10). Four children with abnormal ADC (1.8-2.3) had chronic kidney disease. The remaining five cases with normal nadir creatinine and eGFR had normal ADC. One case with unilateral elevated ADC had a poor ipsilateral renal function on dimercaptosuccinic acid scan. Conclusion Here, it seems that diffusion-weighted magnetic resonance imaging with ADC determination could be useful in accurately evaluating fetal kidneys in PUV and predicting renal function. It may be an additional, non-invasive method when biologic and sonographic findings are inconclusive, especially in the case of oligohydramnios. Further studies are needed to confirm our data. © 2017 John Wiley & Sons, Ltd.

Ectopic pancreas also known as heterotopic pancreas (HP) is a rare congenital anomaly, mainly found as incidental finding during autopsy or abdominal exploration for an other condition. Incidence rate is probably underestimated as patients are mostly asymptomatic; otherwise, it is capable of producing symptoms depending on its location, size, often appearing in the 4th-to-6th decades. Complications such as inflammation, obstruction, bleeding, and malignancy degeneration must be considered. Pediatrics cases are very rare, generally concerning HP within Meckel's diverticulum, manifesting by gastrointestinal bleeding and intussusception. We report a rare case of jejunum bleeding, due to an isolated HP in a 15-year-old adolescent. Endoscopic and computed tomographic scan were normal, in particular did not found Meckel's diverticulum. Diagnosis and treatment have been apprehended performing a laparoscopic exploration. It is a singular location for HP, predominantly found in upper gastrointestinal tractus. So far, there have been no case reports of jejunal bleeding from ectopic pancreas without Meckel's diverticulum in children.

The natural evolution of bronchogenic cysts (BCs) is unpredictable. Although most surgeons agree that symptomatic BCs should be resected, questions remain regarding the optimal management of asymptomatic mediastinal cysts. We present a case series of BCs to compare patients who underwent preventive operation with those who underwent surgical procedure after symptom onset.This 15-year multicenter retrospective study included 114 patients (32 children and 82 adults). Data on clinical history, pathology, mean hospital stay, intraoperative and postoperative complications, and associated intraoperative procedures were analyzed separately for symptomatic and asymptomatic patients.A total of 53 asymptomatic patients (46.5%) were compared with 61 symptomatic patients (53.5%). There were significantly more adults in the symptomatic group than in the asymptomatic group (48 vs 34 patients, P.05). A thoracoscopic approach was used in 88 patients (77%), with 7 conversions to thoracotomy (9%), all in symptomatic patients. There were significantly more additional procedures (20% vs 4%, P = .01) and more intraoperative complications (20% vs 4%, P = .01) in symptomatic patients, but postoperative complications between symptomatic and asymptomatic patients were similar. The postoperative length of stay was significantly longer in symptomatic patients (5.71 days vs 4 days, P.001). Pathologic examination found significantly more inflammatory reactions in symptomatic patients.Early surgical management of BCs may be recommended to prevent symptomatic complications, which are unpredictable and whose management is more complicated in advanced BCs. Surgery can be performed with a thoracoscopic approach, which is easier and safer when the cyst is small and uncomplicated.

Las urgencias quirurgicas pediatricas del recien nacido y del lactante son una situacion clinica angustiosa para el medico. La urgencia perinatal puede ser previsible en la sala de partos o surgir de imprevisto. El tratamiento de las urgencias quirurgicas en la sala de partos se ha modificado por el seguimiento obstetrico y el diagnostico prenatal. El descubrimiento de una malformacion durante la evaluacion sistematica prenatal permite orientar a las familias hacia una maternidad de nivel 3. El objetivo es que el nacimiento tenga lugar en un medio especializado con el fin de no perder un tiempo precioso para el recien nacido. Algunas de estas patologias pueden comprometer el pronostico vital inmediato. Dependiendo de la edad, existen varias situaciones: las urgencias toracicas por la dificultad respiratoria (malformacion congenita pulmonar, hernia diafragmatica), los obstaculos digestivos responsables de oclusiones (volvulo del intestino delgado debido a malrotacion, atresias digestivas, malformaciones anorrectales, ileo meconial, enterocolitis ulceronecrosante, enfermedad de Hirschsprung, apendicitis, estenosis del piloro, invaginacion intestinal aguda), las urgencias parietales (laparosquisis, onfalocele, hernia inguinal) o las urgencias urologicas (valvulas de la uretra posterior, torsion del pediculo espermatico). El objetivo principal en las urgencias quirurgicas del lactante es establecer el diagnostico y realizar el tratamiento lo antes posible. La anamnesis y la exploracion fisica son esenciales para un diagnostico adecuado. Las pruebas de imagen necesarias no deben retrasar el tratamiento quirurgico, por lo que es importante una buena colaboracion entre las distintas especialidades medicas que se encargan del nino (medico de urgencias, pediatra, reanimadores, radiologos). En todas estas situaciones de urgencia, se debe informar a los progenitores. Estos se encuentran en una situacion muy vulnerable y requieren una atencion particular en servicios que puedan acoger a los ninos y los progenitores.

OBJECTIVES In Hirschsprung disease (HD), despite successful surgical treatment, 50% of children experience long-term functional gastrointestinal problems, particularly chronic functional obstructive symptoms. We report our experience regarding clinical effects of neurostimulation-guided anal intrasphincteric botulinum toxin (BT) injections on postoperative obstructive symptoms attributed to a nonrelaxing anal sphincter complex in HD patients. METHODS In this monocenter cohort study, 15 HD patients with postoperative functional intestinal obstructive symptoms received neurostimulation-guided anal intrasphincteric BT injections. Short-, medium-, and long-term effects were evaluated. The Bristol stool form scale was used to assess stool consistency, and the Jorge-Wexner (JW) score to assess fecal continence. RESULTS The median age at first injection was 4 years. In the short-term, a significant improvement in stool consistency was noted in 12 of 14 patients (P = 0.0001) and JW score decreased for 14 of 15 patients (P = 0.001). In the medium-term, JW score significantly decreased for all patients (P = 0.0001), with an improvement of 50% or more for 10 patients (66.7%). In the long term, 83.3% of patients had normal stool consistency and JW score was

International audience; PURPOSE:While familial forms of complex disorders/differences of sex development have been widely reported, data regarding isolated hypospadias are sparse and a family history is thought to be less frequent. We aimed to determine the frequency of hypospadias in families of boys with hypospadias, to establish whether these familial forms exhibit a particular phenotype and to evaluate the prevalence of genetic defects of the main candidate genes.MATERIALS AND METHODS:A total of 395 boys with hypospadias were prospectively screened for a family history with a standardized questionnaire, extensive clinical description, family tree and sequencing of AR, SF1, SRD5A2 and MAMLD1.RESULTS:Family history of hypospadias was more frequent than expected (88 patients, 22.3%). In 17 instances (19.3%) familial hypospadias cases were multiple. Familial hypospadias was related to the paternal side in 59.1% of cases, consisting of the father himself (30.7%) as well as paternal uncles and cousins. Premature birth, assisted reproductive techniques, other congenital abnormalities and growth retardation were not more frequent in familial hypospadias than in sporadic cases. The severity of phenotype was similar in both groups. The results of genetic analysis combined with previous data on androgen receptor sequencing revealed that familial cases more frequently tend to demonstrate genetic defects than sporadic cases (5.68% vs 1.63%, p = 0.048).CONCLUSIONS:Familial forms of hypospadias are far more frequent than previously reported. Even minor and isolated forms justify a full clinical investigation of the family history. Detecting these hereditary forms may help to determine the underlying genetic defects, and may improve followup and counseling of these patients.

International audience; In the skin, Merkel cells connect with keratinocytes and Ab nerve fibers to form a touch receptor that functions as a slow adapting mechanoreceptor (slow adapting type 1). In human and mouse Merkel cells, we observed an increased concentration of intracellular Ca 2þ ions in response to cold temperature and transient receptor potential melastatine 8 (TRPM8) ion channel agonists. A reduction in the response to cooling and TRPM8 ag-onists occurred after the addition of TRPM8 antagonists, as well as in TRPM8 knockout mice. Cold temperature and TRPM8 agonists also induced a current that was inhibited by a TRPM8 antagonist. Our results indicate that Merkel cells sense cooling through TRPM8 channels. We hypothesized that cooling modulates the slow adapting type 1 receptor response. Cooling mouse skin to 22 C reduced the slow adapting type 1 receptor discharge frequency. Interestingly, we observed no such reduction in TRPM8 knockout mice. Similarly, in human skin, a temperature of 22 C applied to the slow adapting type 1 receptive field reduced the spiking discharge. Altogether, our results indicate that Merkel cells are polymodal sensory cells that respond to mild cold stimuli through the activation of TRPM8 channels. Thermal activation of Merkel cells, and possibly other TRPM8-expressing non-neuronal cells, such as keratinocytes, potentially adapts the discharge of slow adapting type 1 receptors during cooling.

Mild head trauma can be associated with concussion, defined as transient brain function impairment without radiological findings. Sports-related concussion is also reported in pediatrics (rugby, ice hockey, football, boxing, etc.). Misdiagnosis can lead to persistent neurocognitive signs with athletic and academic problems. Consensual tools are available, but they are not well-known by first-line doctors, coaches, and patients or parents. Concussed players should not be allowed to return to the field on the same day. Return to play should be gradual over 3weeks or more. School activities may need to be modified to favor cognitive rest. Prevention is based on risk information and knowledge transfer, rule changes, and protective helmets, whose effectiveness is not always proven.

Introduction Quantify quality of life (QoL) outcomes in gastroschisis children is little assessed. The primary objective was to describe the long-term outcome of newborns with gastroschisis treated in three tertiary care hospitals of France in terms of neurodevelopment and QoL. Materials and Methods The study reported was a cross-sectional, descriptive multicentric retrospective study assessing the outcome of newborns with gastroschisis, born between January 1, 2009, and December 31, 2014, treated at two large and French level III neonatal intensive care units. Long-term outcome data were assessed by questionnaires sent to the infants' parents. Questionnaires explored global health, neurological development, and quality of life (overall assessment including socio-economic and medical), Age & Stages Questionnaire, infants' quality of life (KIDSCREEN), and quality of parents' life (General Questionnaire Short Form-36). Results In this study, 50% of the survivor's families answered the assessment form (n = 33). The average follow-up age was 40 months, ranging from 8 months to 6 years. Cases of simple gastroschisis more often had a normal score for “communication” (p = 0.033), while patients who received morphine for a longer duration had significantly lower scores for the items “communication” and “problem resolving” (p = 0.024 and p = 0.011, respectively). Children's QoL was significantly lower for patients with gestational age younger than 36 weeks (p = 0.023) and for patients born following “fetal cause delivery” (p = 0.022). Parents had a significantly higher physical composite score if their child underwent primary closure (p = 0.012). Conclusion Our analyses confirm the idea that cases of complex gastroschisis and preterm delivery may lead to poorer outcome. Such hindsight (40 months in mean) allowed for an interesting assessment of development long after the patient's initial hospitalization and to confirm these results, a standardized neuropsychological evaluation of patients should be done when at least 6 years old. An accurate assessment of the social environment and its impact on the development and QoL of children will be fundamental to avoid selection bias.

International audience; Objective: The aim of this study was to identify antenatal prognostic factors of neonatal outcomes in cases of fetal echogenic bowel (FEB). Study design: A retrospective study in three tertiary referral centers including fetal echogenic bowel over a 10-year period (from January 2003 to December 2013). The echogenicity of the fetal bowel was graded from 1 to 3, according to Slotnick's definition. Associated echographic findings such as bowel dilations, gallbladder abnormalities, calcifications, extra-abdominal abnormalities, intrauterine growth restriction (IUGR) and a decrease in amniotic fluid volume, if present were also recorded. This was followed by the FEB's sonographic evolution. The sonographic evolution was considered favorable if it was stable or decreasing and unfavorable if the echogenicity of the bowel increased or if additional sonographic findings appeared. Neonates had a pediatric examination in the delivery room and upon discharge from the maternity hospital. An outcome was considered good in the case of on-term delivery of a newborn with normal clinical examination and meconium elimination. Results: Complete pregnancy outcome data were available for 409 pregnancies. 338 newborns had uneventful outcomes (82.6%). Antenatal exploration diagnosed 4 cases of aneuploidy (1 case of trisomy 13, 1 case of trisomy 18 and 2 cases of triploidies), 16 cases of congenital infections, 9 cases of cystic fibrosis and 11 cases of bowel abnormalities. After a multivariate analysis, we discovered the sonographic grade of the echogenic bowel was not a prognostic factor of neonatal outcome. The isolated fetal echogenic bowel had a 6.6-fold increase chance of uneventful outcomes (adjusted odd ratio (aOR) 6.6, 95% CI 3-14.4). Notably, favorable sonographic evolution (aOR 8.1, 95% CI 4.1-16) and late gestational age at the time of the diagnosis (aOR 1.17, 95% CI 1.07-1.27) are independent, good prognostic factors of good neonatal outcomes. None of the 180 fetuses with isolated fetal echogenic bowel and favorable sonographic evolution had adverse outcomes. Among these, 4 cases (0.98%) of aneuploidy, 17 cases (4.2%) of congenital infections and 9 cases (2.2%) of cystic fibroses were also diagnosed. No cases of Down syndrome (DS) were reported. Conclusion: Our study shows that the grade should not be considered a prognostic factor of neonatal outcomes. Our data suggests the need to reevaluate the concept of systematic amniocentesis. Sonographic evolution of fetal bowel is an independent, strong prognostic factor for good neonatal outcomes. It also better defines the FEB prognostic. (C) 2017 Elsevier B.V. All rights reserved.

Resume Objectif Le choix de l’antibiotique et la duree d’administration au cours de l’appendicite aigue de l’enfant restent debattus. L’objectif de ce travail etait de comparer l’efficacite de deux protocoles d’antibioprophylaxie associee a l’appendicectomie chez l’enfant. Materiel et methodes Le taux de complications infectieuses apres appendicectomie a ete compare sur deux periodes distinctes (etude avant/apres). Pendant la premiere periode, l’antibioprophylaxie associant ticarcilline et acide clavulanique etait poursuivie en cas d’appendicite perforee. Pendant la seconde periode, elle associait amoxicilline et acide clavulanique pour les formes non perforees ou les formes localisees non perforees et ticarcilline et acide clavulanique pour peritonites generalisees ou stercorales. Tous les enfants de moins de 16 ans ayant subi une appendicectomie pendant les periodes etudiees ont ete inclus. Les donnees ont ete recueillies d’apres les comptes rendus operatoires et les dossiers medicaux. Resultats Quatre-vingt-quinze et 238 enfants ont ete respectivement inclus au cours de la premiere et de la seconde periode. En cas d’appendicite non perforee, le taux de complication infectieuse postoperatoire avait ete de 0 % (0/74) au cours de la premiere periode et 1 % (2/153) pendant la seconde. En cas d’appendicite perforee, ce taux avait ete respectivement de 10 % (2/21) et 11 % (9/85). Conclusion Le protocole associant amoxicilline et acide clavulanique a permis d’obtenir un taux de complications infectieuses postoperatoire faible, comparable a ceux rapportes dans la litterature et sans difference significative avec celui obtenu avec le protocole associant ticarcilline et acide clavulanique.

Objective To describe the diagnostic criteria and outcome of fetal megacystis according to the gestational age at diagnosis. Methods A 7-year retrospective study was carried out from 2004 to 2011, including cases of megacystis referred to 2 prenatal fetal medicine units. The following data were collected and analyzed: maternal age, term at diagnosis (gestational weeks), ultrasonographic and magnetic resonance imaging data, karyotype, decision of the multidisciplinary prenatal team, fetopathology in cases of termination of pregnancy or fetal death, final diagnosis at birth after ultrasonography and voiding cystourethrography, and medical and surgical follow-up. Results Of the 69 fetuses included in this study, 82.6% were males; 26 were diagnosed during the first trimester, 21 during the second, and 22 during the third. During the first trimester, the main etiologies were urethral occlusions and prune-belly syndrome with poor fetal prognosis. Nineteen pregnancies (69%) were terminated for medical reasons including the association with other malformations, poor evolution, or miscarriage. Only 4 children were born alive. The main etiologies of megacystis discovered during the second and third trimesters were vesicoureteral reflux and urethral occlusion. Twenty of 22 fetuses (91%) were born alive when the fetal megacystis was discovered after 27 weeks of gestation. Conclusion Antenatal discovery of megacystis is a complex and challenging prognostic situation. The prognosis depends on the gestational age at discovery. Megacystis is not always associated with obstruction. In a newborn with megacystis, bladder outlet obstruction has to be excluded. Optimal counseling of the involved parents requires a multidisciplinary approach.

Resume La repermeation de fistule œso-tracheale (FOT) est une complication secondaire possible de l’atresie de l’œsophage (AO) operee observee dans environ 10 % des cas. A partir d’une cohorte de 67 enfants operes d’une AO de type III entre 1998 et 2009, nous avons voulu preciser le nombre d’enfants ayant presente une repermeation de FOT, les facteurs de risque de repermeation et les traitements entrepris. Le sex-ratio etait de 1,7. La cure chirurgicale initiale avait eu lieu entre 4 et 36 heures de vie. Cinq enfants (7,5 %) avaient presente une recidive fistulaire, la plupart dans les 3 premiers mois de vie, se revelant par des symptomes respiratoires rythmes ou non par l’alimentation. Cette repermeation etait plus frequente en cas de fuite anastomotique (p = 0,09) ou de pneumothorax (p

Objective To describe surgical procedure and results of Congenital Megaprepuce (CMP) that consists in phimosis and megaprepuce; glans and corpora trapped into pre-pubic fat; incomplete micturition with typical “ballooning” aspect. Methods Genitoplasty with penoscrotal separation (GPS) was performed at Pediatric Surgery Department of Hospital Nord - Marseille, between July and December 2010, in 5 uncircumcised children with CMP, by the same surgeon. Transversal incision at the ventral penoscrotal junction was used, with shaft bipartition and Buck's ventral fascia exposition. The inner layer of prepuce was resected while the outer layer was preserved for coverture of the penile shaft. The dorsal foreskin was medially and longitudinally incised to obtain adequate length of the dorsal shaft. The first middle dorsal suture was made using PDS 6.0 and completed in ventral direction with interrupted stitches. The urinary catheter was placed and hydrocolloid duoderm dressing used. Perioperative prophylactic antibiotic was given and pain control achieved with paracetamol and codeine. Results All patients presented urinary troubles before surgery. Skin penile coverage was complete in all patients with cosmetically and functionally satisfying results; no complication was observed at 12 months mean follow-up. Conclusion CMP is a specific entity of “inconsistent penis.” Surgical treatment should be discussed at diagnosis, resolving micturition troubles. GPS is a simple and reproducible procedure by experienced surgeons. Excellent cosmetic and functional results are obtained at mean follow-up, even if long-term results are required. GPS can be useful in other malformations of the penile shaft.

Intracellular Ca(2+) increase is a common feature of multiple cellular pathways associated with receptor and channel activation, mediator secretion and gene regulation. We investigated the possibility of using this Ca(2+) signal as a biomarker for a reaction to chemical irritants of normal human keratinocytes (NHK) in submerged primary cell culture. We tested 14 referenced chemical compounds classified as strong (seven), weak (four) or non- (three) irritants in acute irritant contact dermatitis. We found that the strong irritant compounds tested at 20-40 mM induced an intracellular Ca(2+) increase measurable by spectrofluorimetry in an automated test. Weak and non-irritant compounds however did not increase intracellular Ca(2+) concentration. We further investigated the mechanisms by which the amine heptylamine, classified as a R34 corrosive compound, increases intracellular Ca(2+). Heptylamine (20mM) induced an ATP release that persisted in the absence of intra- and extra-cellular Ca(2+). In addition, we found that this ATP activates NHK purinergic receptors that subsequently cause the increase in intracellular Ca(2+) from sarcoplasmic reticular stores. We conclude that measuring the intracellular Ca(2+) concentration in NHK is a suitable and easy way of determining any potential reaction to soluble chemical compounds.

Place: England; International audience; OBJECTIVE: To identify the gestational age (GA) at which risk of mortality and severe outcome was minimized comparing preterm delivery and expectant management. METHODS: Retrospective study performed between 2009 and 2014 of newborns with gastroschisis in three large French level III neonatal intensive care units. Each department followed two distinct strategies: elective delivery at 35 weeks' GA and a delayed approach. RESULTS: We included 69 gastroschisis cases. The lengths of stay lasting more than 60 days were significantly greater in the planned delivery group than in the expectant approach group (18/30 (60%) vs. 8/39 (20.5%), p = 0.001). Gastroschisis cases receiving antenatal corticoids during the last two weeks of gestation required significantly less surgeries during their initial stay (p = 0.003) as well as shorter parenteral feedings (p = 0.002). A multivariate logistic regression showed that a GA of less than 36 weeks' GA was is a pejorative factor for a stay above 60 days, regardless of whether it was a simple or complex gastroschisis, (OR= 3.8; p = 0.021). A complex gastroschisis was a risk factor for significantly longer parenteral feedings, regardless of the center where patient is treated (Beta = -0.3, p = 0.035). CONCLUSIONS: Future research should focus on decisions about delivery timing by incorporating risk of neonatal morbidity.

The objective of this study was to evaluate the accuracy of fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient (ADC) determination to predict postnatal renal function (nadir creatinine at 1 year and eGFR) of men with posterior urethral valves (PUV).Between 2003 and 2014, 11 MRI were performed on fetuses (between 28 and 32 weeks) in whom second trimester sonography suggested severe bilateral urinary tract anomalies, suspected of PUV.The ADC of the 11 fetuses ranged from 1.3 to 2.86 mmHere, it seems that diffusion-weighted magnetic resonance imaging with ADC determination could be useful in accurately evaluating fetal kidneys in PUV and predicting renal function. It may be an additional, non-invasive method when biologic and sonographic findings are inconclusive, especially in the case of oligohydramnios. Further studies are needed to confirm our data. © 2017 John WileySons, Ltd.

Resume Le rhabdomyosarcome paratesticulaire est une tumeur embryonnaire rare et agressive de l’enfant et du jeune l’adolescent. Il se developpe a partir des tissus mesenchymateux du cordon spermatique, de l’epididyme ou de la vaginale testiculaire. Il represente 10 % des tumeurs testiculaires de l’enfant. Cliniquement, il se revele souvent par la decouverte fortuite d’une volumineuse bourse indolore. La strategie therapeutique (associant chimiotherapie et curage ganglionnaire ou radiotherapie) depend du stade de la tumeur et du groupe pronostique selon la classification Intergroup Rhabdomyosarcoma Study (IRS). La precocite du diagnostic conditionne son pronostic. Le taux de survie est nettement superieur en cas de tumeur localisee, embryonnaire et survenant avant 10 ans. Nous rapportons le cas d’un rhabdomyosarcome paratesticulaire diagnostique devant une volumineuse bourse d’apparition recente chez un garcon de 6 ans. A la lumiere de ce cas clinique, les orientations diagnostiques devant une bourse aigue indolore et les modalites therapeutiques des rhabdomyosarcomes paratesticulaires de l’enfant sont discutees.

Post-traumatic small-bowel obstructions are rare and late complications following blunt abdominal trauma. Timely diagnosis is frequently impeded because of nonspecific associated symptoms, and furthermore, the degree of urgency is frequently underestimated due to the accidental cause. During the last 6 years (2005-2011), we have observed only 2 cases (4 and 10 years old) with post-traumatic small-bowel obstruction. On admission the patients had a contusion on the abdominal wall, duodenal hematoma and a Chance fracture of the lumbar spine on the computed tomography (CT) scan. Although early clinical improvement was noted, progression of the disorder was observed for the following 15 days like an occlusive syndrome (abdominal distension, nausea, emesis). Abdominal ultrasound and computed tomography scan revealed small-bowel obstruction. At laparotomy, jejunal stenosis was found associated with mesenteric tears, which was resected with end-to-end anastomosis. Six to 8 months of follow-up ascertained the therapeutic efficacy of the procedure. Post-traumatic small-bowel obstructions are rare; the clinical presentation is unspecific and appears with a time lag following the trauma. Echography and computed tomography scan can establish both the diagnosis and therapeutic choice.

Resume Buts Analyser les modalites de la prise en charge conservatrice des fractures severes du rein de l’enfant ainsi que leur pronostic fonctionnel a distance. Patients et methodes Il s’agissait d’une etude retrospective de 22 enfants admis pour une fracture du rein severe (grade V) dans deux services de chirurgie pediatrique sur une periode de 16 ans. Un traitement conservateur initial a ete realise chez 19 patients (86,5 %). Trois enfants presentant une lesion vasculaire ont eu un traitement par radiologie interventionnelle. L’aspect morphologique et fonctionnel du rein lese ont ete determines par une scintigraphie au DMSA et/ou une Uro-IRM. Resultats Vingt et un patients (95,5 %) ont eu une scintigraphie au DMSA dont deux ont ete associees a une Uro-IRM, un patient a eu uniquement une Uro-IRM. Dix enfants (45,5 %) ont eu une restitution ad integrum du parenchyme renal ; on notait sept atrophies polaires superieures (30 %), une atrophie polaire inferieure (4,5 %), deux atrophies renales completes (9 %) et deux patients (9 %) pour lesquels on n’avait aucune precision. Six patients (27 %) ont presente un urinome qui a necessite un drainage dans cinq cas et une simple surveillance chez un enfant. On ne notait aucun pseudokyste renal secondaire ni d’hypertension arterielle. La moitie des lesions renales grades V (50 %) (11 sur 22 patients) avait une fonction normale apres un recul moyen de 19 mois. Conclusion La prise en charge non operatoire des traumatismes severes du rein a ete efficace avec parfois la necessite d’un drainage interne ou percutane afin de reduire l’urinome. Le pronostic fonctionnel renal etabli par imagerie etait satisfaisant avec une restitution ad integrum chez un patient sur deux.

Resume La pyelonephrite xanthogranulomateuse diffuse (PXG) est une pathologie infectieuse tres rare chez l’enfant de moins d’un an. Nous rapportons l’observation d’un nourrisson âge de quatre mois ayant une masse isolee du flanc gauche. Cette lesion a ete decouverte au decours de la surveillance d’une dilatation pyelique gauche de depistage prenatal. Cliniquement, l’enfant n’avait pas de fievre ou d’alteration de l’etat general. Les urines etaient steriles et il existait un syndrome inflammatoire biologique. Le bilan radiologique (echographie renale, uro-scanner et scintigraphie renale) mettait en evidence un aspect d’« hydropyonephrose » sur un rein gauche non fonctionnel, evoquant le diagnostic de PXG. L’indication d’une nephrectomie totale par lombotomie a ete posee. L’exploration peroperatoire a mis en evidence un rein augmente de volume, avec infiltration de l’espace peri renal et avec une anomalie de la jonction pyelo-ureterale. L’examen anatomopathologique definitif a confirme le diagnostic de PXG diffuse. A 12 mois de suivi, il n’y a pas eu de complication. La PXG est une infection grave pouvant entrainer la destruction du parenchyme renal et dont la pathogenese est mal elucidee. La symptomatologie n’est pas specifique. Le traitement curatif est chirurgical et le diagnostic est confirme par l’examen de la piece de nephrectomie. Il est important d’evoquer ce diagnostic devant la survenue d’une masse renale dans un contexte d’uropathie malformative et la PXG doit faire partie des diagnostics differentiels de tumeur renale chez le nourrisson et l’enfant.

Objectives To assess the morbidity and success rate of percutaneous treatment of the postoperative ureteric strictures in children. Materials and Methods Between January 1994 and December 2003, 12 children were treated by antegrade percutaneous balloon dilation for postoperative ureteric strictures. Stenosis occurred at the pelviureteric junction in 5 cases and ureterovesical junction in 7 cases. The 10 boys and 2 girls were between 3 months and 14 years old (mean, 5 years ± 4.7 years). General anesthesia was used in 10 cases for nephrostomy catheter placement. Five ureteral stents were used additionally for nephrostomy drainage with a 6-F catheter. Both nephrostomy and ureteric stents were in place for 28.5 ± 12 days, then removed after control antegrade pyelography. Results Dilation was technically successful in 9 of our patients. Two peroperative complications occurred. Postoperative results were evaluated by ultrasonography, intravenous urography, antegrade pyelography, and diethylene triamine pentaacetic acid renography that confirmed no obstacle in all 5 cases of pelviureteric stricture with a follow-up of 4 ± 2.9 years and in 4 cases of ureterovesical junction with a follow-up of 4.7 ± 2.8 years. Three unsuccessful results were reported: in 2 cases, the guide wire could not be advanced over the stenotic ureterovesical junction and in 1 case an early restenosis occurred that eventually required surgery. Conclusions Although the main treatment of the postoperative ureteral strictures is surgical, the percutaneous antegrade balloon dilation seems to be an alternative to surgery with a low morbidity rate and short hospitalization period.

Touch is detected through receptors located in the skin and the activation of channels in sensory nerve fibres. Epidermal keratinocytes themselves, however, may sense mechanical stimulus and contribute to skin sensation. Here, we showed that the mechanical stimulation of human keratinocytes by hypo-osmotic shock releases adenosine triphosphate (ATP) and increases intracellular calcium. We demonstrated that the release of ATP was found to be calcium independent because emptying the intracellular calcium stores did not cause ATP release; ATP release was still observed in the absence of external calcium and it persisted on chelating cytosolic calcium. On the other hand, the released ATP activated purinergic receptors and mobilized intracellular calcium stores. The resulting depletion of stored calcium led to the activation of capacitative calcium entry. Increase in cytosolic calcium concentration was blocked by the purinergic receptor blocker suramin, phospholipase C inhibitor and apyrase, which hydrolyses ATP. Collectively, our data demonstrate that human keratinocytes are mechanically activated by hypo-osmotic shock, leading first to the release of ATP, which in turn stimulates purinergic receptors, resulting in the mobilization of intracellular calcium and capacitative calcium entry. These results emphasize the crucial role of ATP signalling in the transduction of mechanical stimuli in human keratinocytes.

Resume Nous rapportons l’observation d’un nouveau-ne, âge de 19 jours, ayant une valve de l’uretre posterieur depistee en antenatal qui a developpe sept jours apres la section complete de la valve, un urothorax compressif en relation avec un urinome perirenal. Un drainage pleural et vesical a permis une retrocession des epanchements ainsi qu’une cicatrisation du parenchyme renal. L’epanchement thoracique urineux est une complication tres rare d’une valve de l’uretre posterieur avec urinome perirenal. Cette derniere resulte d’une rupture probable d’un fornix dysplasique par hyperpression de la voie excretrice. Les auteurs soulignent le mode de decouverte atypique de cette affection par des troubles respiratoires et son caractere retarde apres resection de l’obstacle en periode neonatale.

Vascular ischemic lesions of the kidney in multiple trauma patients: incidence and management, a report of 7 cases Aims. Study the incidence, type and outcome of traumatic ischemic arterial lesions of the kidney in multiple trauma patients. Materials and methods. Retrospective study of 500 multiple trauma patients who underwent whole body 64 slice CT Scan (mean age 34 years old, sex ratio 4M/1W, mean ISS 29). Results. There were seven cases of vascular lesions of the left kidney 1.4% of patients (men, median age 25 years old, mean ISS43.9). These were closed traumas with sudden deceleration and dissection of the renal artery trunk in three cases and injury to its branches in four cases with preserved excretion. Three patients died of associated injuries, including one patient who received a stent on D0. One patient underwent a nephrectomy and the three other patients were not treated which did not affect renal function or pressure (follow-up 19 months). Conclusion. We found a high incidence of traumatic renal dissection in multiple trauma patients. Multidetector scan appears to provide the best results for the diagnosis of these lesions as well as the many associated lesions resulting in a very poor prognosis for these patients. Treatment is not well defined and management of these lesions is often of secondary importance because of the severity of associated injuries.

Objectives We evaluated functional outcome of kidneys with fetal urinoma associated to ureteropelvic junction obstruction. Methods We retrospectively reviewed 5 cases of antenatally diagnosed urinoma associated to hydronephrosis. Prenatal imaging work-up consisted of ultrasound (US) and magnetic resonance (MR) examination. Differential renal function was assessed postnatally with renal scintigraphy. Results Four male fetuses and 1 female fetuse presented with urinoma with hydronephrosis diagnosed by US at 24-25 weeks gestational age and confirmed by MRI examination at 28-29 weeks gestational age. On postnatal US, urinoma had disappeared in 4 cases. Parenchyma of the affected kidney was poorly differentiated in all cases. Ureteropelvic junction obstruction was confirmed in the 5 cases. Renal scintigraphy revealed poor functioning kidney (1%) in 2 cases, no function in 1 case, and impaired function in 2 cases (17%). Conclusions Association of upper urinary tract dilatation caused by ureteropelvic junction obstruction and prenatally diagnosed urinoma may suggest a poor ipsilateral functioning kidney.

We present a case of a 4-month-old boy with a right abdominoscrotal hydrocele associated to a compression of the femoral triangle, causing an unilateral leg edema. Abdominoscrotal ultrasound revealed a fluid collection with abdominal and scrotal components, communicating through the deep inguinal ring. Sagittal views of magnetic resonance imaging (MRI) showed a dumbbell-shaped hydrocele and the angio-MRI venous sequences confirmed the compression of the right iliac vessels. Curative treatment was surgical through an inguinal approach and consisted in high ligation of the processus vaginalis and hydrocelectomy. Abdominoscrotal hydrocele is an uncommon pathology, which rarely occurs in pediatric population. This diagnosis should be discussed when a cystic abdominal mass is associated to an ipsilateral scrotal hydrocele. The abdominal component of the hydrocele can result in compression of adjacent structures (iliac vessels, ureter). Surgical treatment is recommended. Epididymal and testicular abnormalities are frequently described, as in our observation, and the effects on the future fertility are unknown.

Paediatric surgeons and urologists are often asked to evaluate boys with acute scrotal pain and inflammation. Although, there is much aetiology for this syndrome: testicular torsion, appendicular testicular torsion, epididymo-orchitis, hernia, hydrocele, trauma, Henoch-Schonlein purpura, idiopathic scrotal edema. However, testicular torsion should be at the top of the list because of the medico legal aspects. It is the one diagnosis that must be made accurately and rapidly, if there is any hope for testicular salvage. Color Doppler ultrasound scan can reliably identify those children, who required exploration and spare medical causes. The purpose of this article is to update/review the appropriate evaluation and management of the acute scrotum and to guide the clinician in distinguishing testicular torsion from the other conditions that commonly mimic this surgical emergency.

Knowledge of the characteristics of thoraco-abdominal trauma in children is important to optimize the imaging work up while keeping radiation exposure to a minimum. Because of the plasticity of the pediatric rib cage, rib fractures are infrequent, and severe parenchymal injuries may be present in the absence of rib fracture. Mediastinal injuries are unusual. The increased mobility of solid intraabdominal organs combined with a weaker abdominal wall are specific to pediatric patients. First-line imaging typically includes chest radiograph and abdominal US with Doppler imaging. Contrast-material enhanced CT is used as a second-line technique, with delayed imaging in patients with urinary tract lesions. Dedicated pediatric acquisition protocols are mandatory. Follow-up is obtained mainly with US.

Las malformaciones anorrectales (MAR) agrupan un conjunto de anomalias raras, a veces complejas, de la porcion terminal del tubo digestivo y el aparato genitourinario. La frecuencia de las MAR es de 1 cada 5.000 nacidos vivos. El espectro de estas anomalias varia desde una simple malposicion anal a una agenesia anorrectal completa o hasta una cloaca. La deteccion prenatal raramente es posible. El diagnostico de una MAR se realiza durante la primera exploracion fisica del neonato o tras la aparicion de una obstruccion intestinal baja. La definicion exacta de estas malformaciones (formas altas, intermedias, bajas) y la valoracion de las malformaciones asociadas (malformaciones urogenitales, digestivas, cardiacas y oseas) se realizan en las primeras 24-48 horas de vida. Las exploraciones radiologicas, ecograficas y endoscopicas orientan la eleccion terapeutica. El objetivo del tratamiento es restablecer la anatomia y conseguir una continencia fecal y urinaria lo mas cercana posible a la normalidad. Es necesario distinguir las MAR bajas, cuyo tratamiento consiste en simples dilataciones anales en las estenosis anales bajas y en anoplastia perineal para el resto de las formas, de las MAR altas o intermedias, mas dificiles de corregir. En las formas altas, intermedias o complejas, que requeriran diversos procedimientos quirurgicos en el futuro, hay que proponer una colostomia transitoria. En cualquier tipo de MAR es frecuente observar dos complicaciones postoperatorias que llegan a ser invalidantes: el estrenimiento cronico y la incontinencia, causados por problemas de la motilidad intestinal. La insuficiencia verdadera del esfinter es mas rara, y se presenta principalmente en las formas altas o intermedias. El prolapso rectal se define como la salida de una parte de la pared rectal a traves del ano. En el nino, suele tratarse de un prolapso mucoso benigno que responde a tratamiento medico consistente en medidas higienicodieteticas.

Resume Introduction L’objectif de cette etude etait d’analyser les resultats postoperatoires du traitement conservateur des ureteres ectopiques fonctionnels sur systeme urinaire double par une uretero-ureterostomie basse chez l’enfant. Materiels et Methode Nous avons analyse retrospectivement les dossiers d’enfants ayant un systeme urinaire double avec un uretere ectopique fonctionnel admis dans notre service de 2001 a 2005. Un bilan radiologique post natal comprenant une echographie de l’appareil urinaire, une cystographie retrograde, une urographie intraveineuse et une scintigraphie renale au DMSA a ete realise. La technique operatoire utilisee chez tous les patients etait l’uretero-ureterostomie basse avec anastomose uretero-ureterale termino-laterale. Resultats II s’agissait de 3 filles et de 2 garcons. L’uretere ectopique etait localise du cote gauche chez tous les patients. L’echographie postnatale confirmait la presence d’une dilatation du pyelon superieur ainsi que de l’uretere retro vesical estime a 10 ± 2 mm dans 3 observations. Dans un cas la dilatation du pyelon superieur etait estimee a 25 mm et l’uretere retro vesical a 16 ± 2 mm. Chez le dernier patient la dilatation du pyelon superieur a l’uretere retro vesical etait estimee a 6,5 mm. La fonction renale moyenne du pole superieur (DMSA) etait 23 ± 7%. L’âge moyen au moment de l’intervention etait de 8 mois. Les suites operatoires immediates ont ete simples chez tous les patients. Le recul moyen etait de 38 mois (de 18 mois a 4,5 ans). Ces enfants etaient asympto-matiques, il y a eu une diminution de la dilatation pyelique et ureterale superieure chez tous les patients au controle echographique du 10 eme mois. Conclusion L’anastomose uretero-ureterale basse a ete une alternative therapeutique aux anastomoses pyelo-pyeliques hautes ou a la reimplantation des ureteres en presence d’un uretere ectopique fonctionnel sur un systeme double chez l’enfant.

International audience; BACKGROUND:Numerous studies have focused on the association between endocrine-disrupting chemicals (EDCs) and hypospadias. Phenotype variability, the absence of representative comparison groups and concomitant genetic testing prevent any definitive conclusions.OBJECTIVE:To identify the role of occupational and environmental exposures to EDCs in nongenetic isolated hypospadias.DESIGN, SETTING, AND PARTICIPANTS:A total of 408 consecutive children with isolated hypospadias and 302 normal boys were prospectively included (2009-2014) in a multi-institutional study in the south of France, the area of the country with the highest prevalence of hypospadias surgery.OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS:In patients without AR, SRD5A2, and MAMLD1 mutations, parental occupational and professional exposures to EDCs were evaluated based on European questionnaire QLK4-1999-01422 and a validated job-exposure matrix for EDCs. Environmental exposure was estimated using the zip code, the type of surrounding hazards, and distance from these hazards. Multivariate analysis was performed.RESULTS:Fetal exposure to EDCs around the window of genital differentiation was more frequent in the case of hypospadias (40.00% vs 17.55%, odds ratio 3.13, 95% confidence interval 2.11-4.65). The substances were paints/solvents/adhesives (16.0%), detergents (11.0%), pesticides (9.0%), cosmetics (5.6%), and industrial chemicals (4.0%). Jobs with exposure were more frequent in mothers of hypospadiac boys (19.73% vs 10.26%, p=0.0019), especially cleaners, hairdressers, beauticians, and laboratory workers. Paternal job exposure was more frequent in the cases of hypospadias (40.13% vs 27.48%, p=0.02). Industrial areas, incinerators, and waste areas were more frequent within a 3-km radius for mothers of hypospadiac boys (13.29% vs. 6.64%, p

El diagnostico de vejiga neurogena puede ser evidente en caso de enfermedad congenita (por ejemplo, meningocele), pero mucho mas dificil en caso de disrafia oculta o de trastorno medico. Una exploracion fisica minuciosa y la practica de pruebas urodinamicas suelen ser indispensables para el diagnostico y el tratamiento de estos pacientes, ya que las correlaciones anatomoclinicas son pobres. Si bien la perdida de orina es el sintoma mas aparente y el que a menudo conduce al tratamiento, el objetivo principal del cirujano pediatra consiste en preservar las vias urinarias superiores. La evolucion natural de la vejiga neurogena tiende hacia la destruccion del detrusor, la paralisis del trigono y la disinergia vesicoesfinteriana, y en realidad constituye una verdadera uropatia obstructiva. Por tanto, para caracterizar el tipo de incontinencia urinaria, la perdida de orina se debe integrar en el contexto global de la funcion vesical. La miccion ideal es voluntaria y completa, y solo se puede concebir en el marco de una sinergia de accion entre un reservorio vesical de buena capacidad, una distensibilidad normal y resistencias esfinterianas suficientes. La continencia se logra entonces gracias al equilibrio entre esas distintas funciones, de tal forma que en la mayoria de los casos se precisa combinar tratamiento medico y quirurgico. El cateterismo intermitente suele ser la clave para obtener una evacuacion completa de la vejiga y, por consiguiente, la proteccion de las vias urinarias superiores. El incremento de la capacidad vesical, que al principio se perseguia con la administracion de anticolinergicos, hoy se consigue, en general, por medio de la cistoplastia de aumento. Para incrementar las resistencias esfinterianas se debe realizar una reconstruccion quirurgica, en la mayoria de los casos asociada a la suspension del cuello vesical, a un esfinter artificial o a las inyecciones por via endoscopica. Para aplicar todas esas tecnicas se tienen en cuenta el sexo, la edad y el ambito social, pero tambien han de integrarse a una atencion mas completa, ya que los pacientes presentan muy a menudo trastornos ortopedicos, digestivos y sexuales. El proposito final es lograr que los enfermos tengan una vida social lo mas aceptable posible.

International audience; OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III.STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1year.RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, PCONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer.; ObjectifÉvaluer l’impact du diagnostic anténatal sur la prise en charge néonatale et le devenir à 1an des nouveaux-nés suivis pour atrésie de l’œsophage (AO) de type III.Matériel et méthodesÉtude de population issue du registre national des atrésies de l’œsophage avec inclusion de tous les cas d’AO de type III entre 2008et 2010. Deux groupes ont été constitués: les cas ayant bénéficié d’un dépistage anténatal et ceux dont l’atrésie de l’œsophage a été découverte à la naissance. Une variable composite de morbidité (lâchage de suture, fuite de l’anastomose, sténose œsophagienne) et de mortalité à 1an a été définie.RésultatsQuatre cent huit cas d’atrésie de l’œsophage de type 3ont été inclus avec un taux de dépistage anténatal de 18,1%. Le taux de transfert après la naissance était plus faible dans le groupe DAN (32,4% versus 81,5%, pConclusionLes cas diagnostiqués en anténatal ont une morbidité plus élevée liée au type d’atrésie de l’œsophage (défects plus longs). Même si le diagnostic anténatal ne modifie pas la prise en charge néonatale et la survie à 1an, il permet une information des parents et une préparation de la naissance dans un centre de néonatologie adapté.

International audience; OBJECTIVE: Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA.STUDY DESIGN: We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year.RESULTS: Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3%; 82.2% of EA type I were diagnosed prenatally compared with 17.9% of EA type III (P < .001). Transfer after birth was lower in case of prenatal diagnosis (25.6% vs 82.5%; P < .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P < .001). The composite variables were higher in prenatal diagnosis subset (44% vs 27.6%; P = .003) and in EA type I than in type III (58.1% vs 28.3%; P < .001).CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers.

Background: The prognosis of major renal injuries appears to have improved since a conservative approach has been promoted. The overall prognosis might be less optimistic if all cases are pooled, whatever the type of initial injury (parenchymal or vascular) or the type of treatment (operative or conservative). Methods: The records of 32 children were reviewed. Six patients had a vascular injury, and 26 had a parenchymal injury. Ten were operated on within one month after trauma, and 3 (including one previously operated) underwent delayed surgery for sequelae. Parenchymal loss from atrophy was estimated on echography, computerized tomography, or radionuclide scintigraphy. Results: Fourteen patients (44%) recovered a functioning kidney. Nine (28%: all 6 patients with vascular injury and 3 with parenchymal trauma) lost their kidney from nephrectomy (n = 3) or atrophy (n = 6). Seven patients had severe or moderate sequelae from either partial nephrectomy or partial atrophy. Two had a minimal loss from polar hypotrophy. The incidence of kidney loss or sequelae was 56% (18 of 32 patients). Conclusion: In keeping with the literature data provided all cases are analyzed, at least one out of 4 kidneys will be lost or severely damaged after major kidney trauma in children. J Pediatr Surg 37:1446-1450. Copyright 2002, Elsevier Science (USA). All rights reserved.

We report the case of a 38-week gestational age neonate, with isolated congenital diaphragmatic hernia presenting with refractory persistent pulmonary hypertension, systemic hypotension, and hypoxemia, resistant to usual therapeutics. Arginine vasopressin is responsible for systemic vasoconstriction and decreases pulmonary hypertension. We theorized that terlipressin, its long-acting analogue, could have the same properties. We used terlipressin as rescue therapy after parental and local ethics committee acceptance. After a bolus of terlipressin 20 μg/kg and continuous infusion at a rate of 5 μg/kg per hour, blood oxygen saturation improved from 75% to 98%, oxygen requirements fell from fraction of inspired oxygen 100% to 40%, and mean arterial pressure rose from 28 to 46 mm Hg, allowing a decrease of vasopressor infusion. Terlipressin may be useful in the management of neonates with congenital diaphragmatic hernia and refractory pulmonary hypertension.