Dysmenorrhea among 12-year-old teenagers from different socioeconomic backgrounds.

BACKGROUND: The age of menarche has been gradually declining since the end of the 20th century. Few studies have been carried out about dysmenorrhea in Europe and they mainly included girls over the age of 15 years. METHODS: We conducted an observational study among sixth-grade schoolgirls (usually 11-12 years) during the 2020 academic year in 72 randomly selected public schools in a French region (approval number #20.94). RESULTS: Among 1712 girls interviewed, 257 (23 %) had reached menarche. Overall, 83 % of these teenagers had dysmenorrhea. Symptoms were reported to occur since the first periods in 59 % of the girls. School absenteeism was positively correlated with severe abdominal pain (p = 0.001). Median age at menarche was 11 years (10.7-11.5). Periods lasted less than 3 days, 3-8 days, and more than 8 days in, respectively, 18 %, 70 %, and 7 % of the girls. Symptoms were significantly more frequent and more severe in the urban group than the rural group (p = 0.005). Symptoms were abdominal pain (70 %), fatigue (48 %), difficulty in concentrating (26 %), headache (25 %), digestive disorders (16 %), and breast discomfort (11 %). Of the girls with dysmenorrhea, 61 % took painkillers; 10 % were uncomfortable despite taking painkillers. Sport exemption was more frequent in the urban group (p = 0.003) and among girls with severe abdominal pain (p = 0.001). CONCLUSIONS: The duration of the menstrual cycle was similar between the various socioeconomic groups, but symptoms and ways of coping were significantly different. Dysmenorrhea is definitely an issue that has to be raised with teenagers as soon as menarche occurs or even before that. Easy access to skilled health practitioners should be widespread.

Meconium Concentrations of Pesticides and Risk of Hypospadias: A Case-Control Study in Brittany, France.

BACKGROUND: Hypospadias is a male genital tract defect for which an increase in prevalence has been documented over the last few decades. A role for environmental risk factors is suspected, including prenatal exposure to pesticides. OBJECTIVES: To study the risk of hypospadias in association with multiple pesticide measurements in meconium samples. METHODS: The Brittany Registry of Congenital Anomalies (France) conducted a case-control study between 2012 and 2018. Cases were hypospadias, ascertained by a pediatrician and a pediatric surgeon, excluding genetic conditions, following European Surveillance of Congenital Anomalies guidelines (N = 69). Controls (N = 135) were two male infants without congenital anomaly born after each case in the same maternity unit. Mothers in the maternity units completed a self-administered questionnaire, we collected medical data from hospital records, and medical staff collected meconium samples. We performed chemical analysis of 38 pesticides (parent compound and/or metabolite) by UHPLC/MS/MS following strict quality assurance/quality control criteria and blind to case-control status. We carried out logistic regression accounting for frequency-matching variables and major risk factors. RESULTS: Among the 38 pesticides measured, 16 (42%) were never detected in the meconium samples, 18 (47%) were in <5% of samples, and 4 (11%) in ≥5% of the samples. We observed an association between the detection of fenitrothion in meconium and the risk of hypospadias (OR = 2.6 [1.0-6.3] with n cases = 13, n controls = 21), but not the other pesticides. CONCLUSIONS: Our small study provides a robust assessment of fetal exposure. Fenitrothion's established antiandrogenic activities provide biologic plausibility for our observations. Further studies are needed to confirm this hypothesis.

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

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

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

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

Different Fecal Microbiota in Hirschsprung's Patients With and Without Associated Enterocolitis.

Background and Objectives: Patients with Hirschsprung's disease are at risk of developing Hirschsprung-associated enterocolitis, especially in the first 2 years of life. The pathophysiology of this inflammatory disease remains unclear, and intestinal dysbiosis has been proposed in the last decade. The primary objective of this study was to evaluate in a large cohort if Hirschsprung-associated enterocolitis was associated with alterations of fecal bacterial composition compared with HD without enterocolitis in different age groups. Methods: We analyzed the fecal microbiota structure of 103 Hirschsprung patients from 3 months to 16 years of age, all of whom had completed definitive surgery for rectosigmoid Hirschsprung. 16S rRNA gene sequencing allowed us to compare the microbiota composition between Hirschsprung's disease patients with (HAEC group) or without enterocolitis (HD group) in different age groups (0-2, 2-6, 6-12, and 12-16 years). Results: Richness and diversity increased with age group but did not differ between HD and HAEC patients, irrespective of the age group. Relative abundance of Actinobacteria was lower in HAEC than in HD patients under 2 years of age (-66%, P = 0.045). Multivariate analysis by linear models (MaAsLin) considering sex, medications, birth mode, breast-feeding, and the Bristol stool scale, as well as surgery parameters, highlighted Flavonifractor plautii and Eggerthella lenta, as well as Ruminococcus gnavus group, as positively associated with Hirschsprung-associated enterocolitis in the 0-2 years age group. Conclusion: Hirschsprung-associated enterocolitis was associated with features of intestinal dysbiosis in infants (0-2 years) but not in older patients. This could explain the highest rate of enterocolitis in this age group. Clinical Trial Registration: https://clinicaltrials.gov/ct2/show/NCT02857205, MICROPRUNG, NCT02857205, 02/08/2016.

Long-Term Functional Outcomes of an Anorectal Malformation French National Cohort.

OBJECTIVES: The present study aimed to assess long-term functional outcomes of children with anorectal malformations (ARMs) across a network of expert centers in France. METHODS: Retrospective cross-sectional study of patients ages 6-30 years that had been surgically treated for ARM. Patient and ARM characteristics (eg, level, surgical approach) and functional outcomes were assessed in the different age groups. RESULTS: Among 367 patients, there were 155 females (42.2%) and 212 males (57.8%), 188 (51.2%) cases with, and 179 (48.8%) higher forms without, perineal fistula. Univariate and multivariate statistical analyses with logistic regression showed correlation between the level of the rectal blind pouch and voluntary bowel movements (odds ratio [OR] = 1.84 [1.31-2.57], P < 0.001), or soiling (OR = 1.72 [1.31-2.25], P < 0.001), which was also associated with the inability to discriminate between stool and gas (OR = 2.45 [1.28-4.67], P = 0.007) and the presence of constipation (OR = 2.97 [1.74-5.08], P < 0.001). Risk factors for constipation were sacral abnormalities [OR = 2.26 [1.23-4.25], P = 0.01) and surgical procedures without an abdominal approach (OR = 2.98 [1.29-6.87], P = 0.01). Only the holding of voluntary bowel movements and soiling rates improved with age. CONCLUSION: This cross-sectional study confirms a strong association between anatomical status and functional outcomes in patients surgically treated for ARM. It specifically highlights the need for long-term follow-up of all patients to help them with supportive care.

Transperitoneal Versus Retroperitoneal Robotic-assisted Laparoscopic Pyeloplasty for Ureteropelvic Junction Obstruction in Children. A Multicentre, Prospective Study.

Background: Robotic-assisted laparoscopic pyeloplasty (RALP) has been gaining acceptance among paediatric urologists. Objective: To compare surgical variables and clinical outcomes, including complications and success rate, with RALP using the transperitoneal (T-RALP) and retroperitoneal (R-RALP) approaches. Design setting and participants: We performed a multicentre, prospective, cohort study (NCT03274050) between November 2016 and October 2021 in three paediatric urology teaching centres (transperitoneal approach, n = 2; retroperitoneal approach, n = 1). The diagnosis of ureteropelvic junction obstruction (UPJO) was confirmed by renal ultrasound and mercaptoacetyltriglycine-3 renal scan or uro-magnetic resonance imaging with functional evaluation. The exclusion criteria were children <2 yr old, persistent UPJO after failed pyeloplasty, and horseshoe and ectopic kidney. Intervention: We performed dismembered pyeloplasty using running monofilament 6-0 absorbable suture. Outcome measurements and statistical analysis: We assessed intra- and postoperative morbidity (primary outcome) and success (secondary outcome). Data were expressed as medians and interquartile range (25th and 75th percentiles) for quantitative variables, and analysed comparatively. Results and limitations: We operated on 106 children (T-RALP, n = 53; R-RALP, n = 53). Preoperative data were comparable between groups (median age 9.1 [6.2-11.2] yr; median weight 26.8 [21-40] kg). Set-up time (10 vs 31 min), anastomotic time (49 vs 73 min), and console time (97 vs 153 min) were significantly shorter with T-RALP than with R-RALP (p < 0.001). No intraoperative complications occurred. No conversion to open surgery was necessary. The median hospital stay was longer after T-RALP (2 d) than after R-RALP (1 d; p < 0.001). Overall, postoperative complication rates were similar. No failure had occurred at the mean follow-up of 25.4 (15.1-34.7) mo. Conclusions: In selected children, RALP is safe and effective using either the transperitoneal or the retroperitoneal approach, with a shorter hospital stay after R-RALP. Patient summary: In our multicentre, prospective study, we compared the results and complications of robotic-assisted laparoscopic pyeloplasty (RALP) using the transperitoneal and retroperitoneal approaches. We found that RALP is safe and effective using either approach, with a shorter hospital stay after R-RALP.

Anti-reflux surgery in children with congenital diaphragmatic hernia: A prospective cohort study on a controversial practice.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity of congenital diaphragmatic hernia (CDH) survivors. Performing a preventive fundoplication during CDH repair remains controversial. This study aimed to: (1) Analyze the variability in practices regarding preventive fundoplication; (2) Identify predictive factors for fundoplication. (3) Evaluate the impact of preventive fundoplication on gastro-intestinal outcomes in children with a CDH patch repair; METHODS: This prospective multi-institutional cohort study (French CDH Registry) included CDH neonates born in France between January 1st, 2010-December 31st, 2018. Patch CDH was defined as need for synthetic patch or muscle flap repair. Main outcome measures included need for curative fundoplication, tube feed supplementation, failure to thrive, and oral aversion. RESULTS: Of 762 CDH neonates included, 81 underwent fundoplication (10.6%), either preventive or curative. Median follow-up was 3.0 years (IQR: 1.0-5.0). (1) Preventive fundoplication is considered in only 31% of centers. The rates of both curative fundoplication (9% vs 3%, p = 0.01) and overall fundoplication (20% vs 3%, p < 0.0001) are higher in centers that perform preventive fundoplication compared to those that do not. (2) Predictive factors for preventive fundoplication were: prenatal diagnosis (p = 0.006), intra-thoracic liver (p = 0.005), fetal tracheal occlusion (p = 0.002), CDH-grade C-D (p < 0.0001), patch repair (p < 0.0001). After CDH repair, 8% (n = 51) required curative fundoplication (median age: 101 days), for which a patch repair was the only independent predictive factors identified upon multivariate analysis. (3) In neonates with patch CDH, preventive fundoplication did not decrease the need for curative fundoplication (15% vs 11%, p = 0.53), and was associated with higher rates of failure to thrive (discharge: 81% vs 51%, p = 0.03; 6-months: 81% vs 45%, p = 0.008), tube feeds (6-months: 50% vs 21%, p = 0.02; 2-years: 65% vs 26%, p = 0.004), and oral aversion (6-months: 67% vs 37%, p = 0.02; 1-year: 71% vs 40%, p = 0.03). CONCLUSIONS: Children undergoing a CDH patch repair are at high risk of requiring a curative fundoplication. However, preventive fundoplication during a patch repair does not decrease the need for curative fundoplication and is associated with worse gastro-intestinal outcomes in children. LEVEL OF EVIDENCE: II - Prospective Study.

The Potential and the Limitations of Esophageal Robotic Surgery in Children.

INTRODUCTION: There have been numerous reports of robotic pediatric surgery in the literature, particularly regarding urological procedures for school-aged children. Thoracic procedures appear to be less common, despite the fact that encouraging results were reported more than 10 years. Our aim was to report a national experience of esophageal robotic-assisted thoracoscopic surgery (ERATS) and to discuss the most appropriate indications. MATERIALS AND METHODS: A retrospective multicenter study was conducted to compile the ERATS performed at five French surgical centers that have been involved in spearheading robotic pediatric surgery over the past 15 years. The data were supplemented by a review of the literature. RESULTS: Over the study period, 68 cases of robotic thoracic surgery were performed at the five pediatric centers in question. ERATS was performed for 18 patients (mean age 7.1 years [ ± 5.6]) in four of the centers. These comprised seven esophageal duplications, four esophageal atresias, five Heller's myotomies, and two cases of esophagoplasty. A conversion was needed for two neonates (11%) due to exposure difficulties. Four other procedures for patients who weighed less than 15 kg were successfully completed without causing postoperative complications. In the past 12 years, 22 other cases of ERATS were published worldwide. The indications were the same, except for esophagoplasty, which was not found. CONCLUSION: Aside from accessibility issues with the robotic platform, the main limitation is still very much that the low body weight of children results in incompatibility between the size of the trocars and the size of the intercostal space. ERATS is clearly a feasible procedure with technical advantages for most pediatric cases with body weights more than 15 kg. A transdiaphragmatic abdominal approach should be considered for lower esophagus surgery.

DECONbench: a benchmarking platform dedicated to deconvolution methods for tumor heterogeneity quantification.

BACKGROUND: Quantification of tumor heterogeneity is essential to better understand cancer progression and to adapt therapeutic treatments to patient specificities. Bioinformatic tools to assess the different cell populations from single-omic datasets as bulk transcriptome or methylome samples have been recently developed, including reference-based and reference-free methods. Improved methods using multi-omic datasets are yet to be developed in the future and the community would need systematic tools to perform a comparative evaluation of these algorithms on controlled data. RESULTS: We present DECONbench, a standardized unbiased benchmarking resource, applied to the evaluation of computational methods quantifying cell-type heterogeneity in cancer. DECONbench includes gold standard simulated benchmark datasets, consisting of transcriptome and methylome profiles mimicking pancreatic adenocarcinoma molecular heterogeneity, and a set of baseline deconvolution methods (reference-free algorithms inferring cell-type proportions). DECONbench performs a systematic performance evaluation of each new methodological contribution and provides the possibility to publicly share source code and scoring. CONCLUSION: DECONbench allows continuous submission of new methods in a user-friendly fashion, each novel contribution being automatically compared to the reference baseline methods, which enables crowdsourced benchmarking. DECONbench is designed to serve as a reference platform for the benchmarking of deconvolution methods in the evaluation of cancer heterogeneity. We believe it will contribute to leverage the benchmarking practices in the biomedical and life science communities. DECONbench is hosted on the open source Codalab competition platform. It is freely available at: https://competitions.codalab.org/competitions/27453 .

Unresectable thoracic neuroblastic tumors: Changes in image-defined risk factors after chemotherapy and impact on surgical management.

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

Single bilateral ovarian venous return in uterine transplant: Validation in an orthotopic auto-transplant model in the Yucatan minipig.

BACKGROUND: Uterine transplant (UT) represents an opportunity to treat absolute uterine infertility. However, the use of uterine veins for venous return, in addition to ovarian veins, significantly increases the risk of ureteral wounds in the living donor and UT time for the recipient. Our aim was to demonstrate that dual ovarian venous return is sufficient for graft viability and survival. METHODS: Uterine orthotopic auto-transplant was performed under general anaesthesia in six Yucatan minipig sows. The uterus graft was implanted with termino-lateral anastomoses between the ovarian and external iliac veins, and between the uterine and external iliac arteries, respectively. RESULTS: The macroscopic physical aspect of the graft was adequate in 83 % of the sows (5/6) 30 min after reperfusion with a surgical time of 439±54 min (mean anastomosis time: 153±49 min). Two sows died the day after surgery. In the four remaining sows, two uteri were necrotic and two were adequately vascularized on Day 7. CONCLUSIONS: the learning curve was relatively fast, the sole use of bilateral ovarian venous return is possible and might reduce post-surgery morbidity in human living donors as well as UT time for the recipient.

A multicenter review of undescended testis torsion: A plea for early management.

INTRODUCTION: Torsion of an undescended testis (UT) is a surgical emergency, difficult to diagnose, whose prognosis depends on a quick management. AIM OF THE STUDY: To evaluate the management and outcome of these patients. STUDY DESIGN: We retrospectively analyzed all cases of UT torsion operated in nine French hospitals between 1997 and 2017. We divided patients in two groups: patients referred less than 6 h after the onset of symptoms (group A) or more than 6 h (group B). MAIN RESULTS: We collected 60 cases (17 in group A and 43 in group B). Median age was 2.2 years [IQR = 0.7-7.8] (2.3 y in group A and 2 y in group B, p = 0.76). Eleven patients (10 in group B) had neurological disorders (p = 0.15). The main reason for absence of UT treatment was the absence of surgical consultation in a normal delay (n = 44, 73%). Symptoms were pain (n = 58, 97%), inguinal mass (n = 55, 92%) and vomiting (n = 16, 27%). An inguinal mass with no palpable testis in the ipsilateral hemiscrotum was seen in 55 patients (92%). An ultrasound scan performed in 27 patients led to the diagnosis in 16 patients (59%). At surgery, an orchiectomy was performed in 4 patients (23%) of group A and 24 patients (56%) of group B (p = 0.04). After a median follow-up of 11 months [IQR = 4-23], 11 patients of group A (65%) and 7 patients of group B (16%) had a clinically normal testis (p = 0.03). The salvage rate among patients with conservative treatment was 85% for group A and 37% for group B (p = 0.01). DISCUSSION: Our study reveals that although UT torsion is an emergency, 72% of patients are referred more than 6 h after the onset of symptoms. We mostly found classic clinical presentation of UT torsion: a painful inguinal mass with an empty ipsilateral scrotum. Ultrasound was performed in half cases, and even if the result was not significant, it still seemed to be associated with a higher rate of orchiectomy especially in group B because of the delay in care. However, when ultrasound was realized early, it led to diagnosis in all cases. This dilemma poses the problem of the role of imaging in diagnostic management. CONCLUSIONS: Early clinical diagnosis in front of a painful inguinal mass with an empty scrotum is essential to improve the salvage rate of testis in UT torsion. Early management of UT should have avoided 68% of testis loss.

Performance of diagnostic ultrasound to identify causes of hydramnios.

INTRODUCTION: We aimed to assess the diagnostic yield of ultrasonography in the identification of the etiology of hydramnios, and the added value of MRI or amniocentesis. METHODS: We conducted a single-center retrospective study including pregnancies with confirmed hydramnios (defined as deepest pocket ≥8 cm) between January 2013 and May 2017. Twin pregnancies, secondary hydramnios discovered after the diagnosis of a causal pathology, and pregnancies of unknown outcome were excluded. All pregnancies underwent a targeted scan, and selected cases underwent MRI or amniocentesis. RESULTS: A total of 158 patients with confirmed hydramnios were included. Hydramnios was associated with a fetal pathology in 37 cases (23.4%), with diabetes in 39 (24.6%), isolated macrosomia in 16 (10.1%), and considered idiopathic in 66 (41.7%). Ultrasonography established a diagnosis of the underlying pathology in 73% of cases. Amniocentesis was done in 31 cases (20%) and it allowed diagnosis of chromosome anomalies, esophageal atresia, myotonic dystrophy congenital type, Prader-Willi syndrome, and Bartter syndrome. MRI was done in 15 cases (10%) and it allowed one additional diagnosis of esophageal atresia. The diagnostic yields of MRI and amniocentesis were 91.7% and 95.2%, respectively. There were five false positive diagnoses at ultrasonography, and one false positive diagnosis at MRI. CONCLUSION: Hydramnios can be associated with a wide variety of underlying pathologies. Diagnostic ultrasound can attain a diagnosis in the majority of cases. Amniocentesis offers a valuable complementary assessment.

Management of lower urinary tract fibroepithelial polyps in children.

INTRODUCTION: Fibroepithelial polyps (FEP) of the lower urinary tract are relatively common in adults but rare in children, with fewer than 250 cases reported in the literature to date. OBJECTIVE: The aim of this study was to address the experience of FEP management in children. STUDY DESIGN: A retrospective multicenter review was undertaken in children with defined FEP of the lower urinary tract managed between 2008 and 2018. The data at 18 pediatric surgery centers were collected. Their demographic, radiological, surgical, and pathological information were reviewed. RESULTS: A total of 33 children (26 boys; 7 girls) were treated for FEP of the lower urinary tract at 13 centers. The most common presentation was urinary outflow as hematuria (41%), acute urinary retention (25%), dysuria (19%), or urinary infections (28%). A prenatal diagnosis was made for three patients with hydronephrosis. Almost all of the children (94%) underwent ultrasound imaging of the urinary tract as the first diagnostic examination, 23 (70%) of them also either had an MRI (15%), cystourethrography (25%), computerized tomography (6%), or cystoscopy (45%). Two of these children (6%) had a biopsy prior to the surgery. The median preoperative delay was 7.52 (range: 1-48) months. Most of the patients were treated endoscopically, although four (12.1%) had open surgery and two (6.1%) had an additional incision for specimen extraction. The median hospital stay was 1.5 (range: 1-10) days. There were no recurrences and no complications after a median follow-up of 13 (range: 1-34) months. DISCUSSION: The main limitation of our study is the retrospective design, although it is the largest one for this pathology. CONCLUSION: This series supports sonography as the most suitable diagnosis tool before endoscopy to confirm the diagnosis and to perform the resection for most FEP in children. This report confirms the recognized benign nature in the absence of recurrences. LEVEL OF EVIDENCE: Level V.

A piglet model of iatrogenic rectosigmoid hypoganglionosis reveals the impact of the enteric nervous system on gut barrier function and microbiota postnatal development.

BACKGROUND: Hirschsprung-associated enterocolitis physiopathology likely involves disturbed interactions between gut microbes and the host during the early neonatal period. Our objective was to create a neonatal porcine model of iatrogenic aganglionosis to evaluate the impact of the enteric nervous system (ENS) on microbiota and intestinal barrier postnatal development. METHODS: Under general anesthesia, the rectosigmoid serosa of 5-day-old suckling piglets was exposed to 0.5% benzalkonium chloride solution (BAC, n = 7) or saline (SHAM, n = 5) for 1 h. After surgery, animals returned to their home-cage with the sow and littermates and were studied 21 days later. RESULTS: BAC treatment induced partial aganglionosis with absence of myenteric plexus and reduced surface area of submucosal plexus ganglia (-58%, P < 0.05) in one third of the rectosigmoid circumference. Epithelial permeability of this zone was increased (conductance +63%, FITC-dextran flux +386%, horseradish-peroxidase flux +563%, P < 0.05). Tight junction protein remodeling was observed with decreased ZO-1 (-95%, P < 0.05) and increased claudin-3 and e-cadherin expressions (+197% and 61%, P < 0.05 and P = 0.06, respectively). BAC piglets harbored greater abundance of proinflammatory bacteria (Bilophila, Fusobacterium) compared to SHAM in the rectosigmoid lumen. CONCLUSIONS: This large animal model demonstrates that hypoganglionosis is associated with dramatic defects of gut barrier function and establishment of proinflammatory bacteria.

Outcomes of laparoscopic and open surgical treatment of intestinal malrotation in children.

BACKGROUND: The safety of the laparoscopic treatment of intestinal malrotation remains controversial. This study compared the outcomes of laparoscopic and open surgical treatment of intestinal malrotation. METHODS: A multicentric retrospective study included pediatric cases of intestinal malrotation operated on between 2005 and 2016. RESULTS: This study included 227 children with a median age of 17 days (0-17.2 years), including 161 with a midgut volvulus. Forty-six(20.3%) procedures were started by laparoscopy and 181(79.7%) by laparotomy. Laparoscopy was more frequent for elective surgery (45.9%) than for emergency procedures (10.8%, p < 0.001). Conversions were significantly more frequent during emergency procedures (66.7% vs 17.9%)(p = 0.001). Considering only 61 elective surgeries, the mean hospital stay was significantly shorter after laparoscopy (5.3 days +/-5.2 vs 10.1 days +/-13, p = 0.01), the overall complication rate was comparable (15.8% vs 21.7%, p = 0.7) but post-operative volvulus was significantly more frequent after laparoscopy (13% vs 0%, p = 0.04). Outcomes of the two approaches were not significantly different after 166 emergency procedures. CONCLUSION: Laparoscopy can be performed by experienced team for the treatment of selected cases of intestinal malrotation. Conversion to open surgery should be done with a low threshold, as the rate of volvulus recurrence is concerning. LEVEL OF EVIDENCE: Level III.

Multicystic and diffuse malignant peritoneal mesothelioma in children.

BACKGROUND: Malignant and multicystic peritoneal mesotheliomas are extremely rare tumors in children, developing from mesothelial cells. No specific guidelines are available at this age. METHODS: We performed a retrospective analysis of all identified children (< 18-year-old) treated in France from 1987 to 2017 for a diffuse malignant peritoneal mesothelioma (DMPM) or a multicystic peritoneal mesothelioma (MCPM). RESULTS: Fourteen patients (5 males and nine females), aged 2.2 to 17.5 years, were included. The most frequent presenting symptoms were abdominal pain, ascitis, and alteration in the general condition. Eight patients had epithelioid mesothelioma, three had biphasic mesothelioma, and three had MCPM. Eight patients with DMPM diagnosis received cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). Among them, six patients had neoadjuvant systemic chemotherapy, one patient, post-operative chemotherapy, and one patient CRS and HIPEC only. Three patients received only systemic chemotherapy. All patients with MCPM had only surgery. After a median follow-up of seven years (2-15), six patients (6/11; one death) with DMPM and two patients (two/three) with MCPM had a local and distant recurrences. CONCLUSION: Peritoneal mesothelioma in children is a rare condition with difficult diagnosis and high risk of recurrence. Worldwide interdisciplinary collaboration and networking are mandatory to help diagnosis and provide harmonious treatment guidelines.