Recurrence of anterior congenital diaphragmatic hernia after laparoscopic repair in children.

PURPOSE: To report our experience with laparoscopic repair of anterior congenital diaphragmatic hernia (CDH) using extracorporeal subcutaneous knot tying and to define recurrence risk factors. METHODS: This retrospective unicentric study included children who underwent laparoscopic repair of anterior CDH without patch, using extracorporeal knot tying of sutures passed through the full thickness of the abdominal wall (2013-2020). A systematic review of the literature with meta-analysis was performed using the MEDLINE database since 2000. RESULTS: Eight children were included (12 months [1-183]; 10.6 kg [3.6-65]). Among the two patients with Down syndrome, one with previous cardiac surgery had a recurrence at 17 months postoperatively. In our systematic review (26 articles), among the 156 patients included, 10 had a recurrence (none with patch). Recurrence was statistically more frequent in patients with Down syndrome (19.4%) than without (2.5%) (p < 0.0001), and when absorbable sutures were used (50%) instead of non-absorbable sutures (5.3%) (p < 0.0001). CONCLUSION: Laparoscopic repair of anterior CDH without patch was a safe and efficient surgical approach in our patients. The use of a non-absorbable prosthetic patch should be specifically discussed in anterior CDH associated with Down syndrome and/or in case of previous cardiac surgery to perform a diaphragmatic tension-free closure.

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

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

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

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

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

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

Can fetal magnetic resonance imaging aid prognosis in gastroschisis: A multicenter study.

OBJECTIVE: To assess the contribution and impact of fetal magnetic resonance imaging (MRI) in managing fetal gastroschisis. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Management of primary spontaneous pneumothorax in teenagers: An 11-year study.

AIM: Management of teenagers with primary spontaneous pneumothorax (PSP) is not consensual. We report our experience over an 11-year period. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Resection of Bronchogenic Cysts in Symptomatic Versus Asymptomatic Patients: An Outcome Analysis.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Quality of life and neuropsychological development at school age in Hirschsprung's disease.

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.

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

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

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

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

Heterotopic pancreas without Meckel's diverticulum in children as unique cause of gastrointestinal bleeding: think about it!

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.

Family History is Underestimated in Children with Isolated Hypospadias: A French Multicenter Report of 88 Families.

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.

Merkel Cells Sense Cooling with TRPM8 Channels.

In the skin, Merkel cells connect with keratinocytes and Aß 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 Ca2+ 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 agonists 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.

Long-Term Efficacy of Young-Dees Bladder Neck Reconstruction: Role of the Associated Bladder Neck Injection for the Treatment of Children With Urinary Incontinence.

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.

Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination.

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.

Antenatal prognostic factor of fetal echogenic bowel.

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 aneuploïdy, 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.

Neurodevelopment and Health-Related Quality of Life in Infants Born with Gastroschisis: A 6-Year Retrospective French Study.

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.

Management and outcome challenges in newborns with gastroschisis: A 6-year retrospective French study.

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.

[Case study of two children with intervertebral disc calcifications]. / Calcifications discales intervertébrales chez l'enfant: à propos de deux cas.

Disc calcifications are usually revealed in children by pain crises that affect the spine. The most common location for disc calcifications is the cervical spine. However, other locations are less symptomatic and they may well go unnoticed. We here report two cases of children hospitalized with symptomatic cervical disc calcifications. The first case involves a girl aged four and a half suffering from torticollis revealing the migration of a previously asymptomatic disc calcification ; the second case, instead, involves a 5-year old boy presenting for acute neck pain and in whom calcification disc was detected. Treatment was essentially conservative. Intervertebral disc calcifications in children are a rare condition whose etiopathogenesis remains unknown. In the case of children with unexplained back pain the diagnosis should not be made without careful neurological examination and imaging tests.

Is Hypospadias Associated with Prenatal Exposure to Endocrine Disruptors? A French Collaborative Controlled Study of a Cohort of 300 Consecutive Children Without Genetic Defect.

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<0.00005). Association of occupational and environmental exposures increases this risk. CONCLUSIONS: This multicenter prospective controlled study with a homogeneous cohort of hypospadiac boys without genetic defects strongly suggests that EDCs are a risk factor for hypospadias through occupational and environmental exposure during fetal life. The association of various types of exposures may increase this risk. PATIENT SUMMARY: Our multi-institutional study showed that parental professional, occupational, and environmental exposures to chemical products increase the risk of hypospadias in children.