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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.

Here we investigated the role of murine mast cell protease 4 (MCPT4), the functional counterpart of human mast cell chymase, in an experimental model of renal ischemia reperfusion injury, a major cause of acute kidney injury. MCPT4-deficient mice had worsened kidney function compared to wildtype mice. MCPT4 absence exacerbated pathologic neutrophil infiltration in the kidney and increased kidney myeloperoxidase expression, cell death and necrosis. In kidneys with ischemia reperfusion injury, when compared to wildtype mice, MCPT4-deficient mice showed increased surface expression of adhesion molecules necessary for leukocyte extravasation including neutrophil CD162 and endothelial cell CD54. In vitro, human chymase mediated the cleavage of neutrophil expressed CD162 and also CD54, P- and E-Selectin expressed on human glomerular endothelial cells. MCPT4 also dampened systemic neutrophil activation after renal ischemia reperfusion injury as neutrophils expressed more CD11b integrin and produced more reactive oxygen species in MCPT4-deficient mice. Accordingly, after renal injury, neutrophil migration to an inflammatory site distal from the kidney was increased in MCPT4-deficient versus wildtype mice. Thus, contrary to the described overall aggravating role of mast cells, one granule-released mediator, the MCPT4 chymase, exhibits a potent anti-inflammatory function in renal ischemia reperfusion injury by controlling neutrophil extravasation and activation thereby limiting associated damage.

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.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).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.II - Prospective Study.

A small proportion of patients with EB develop genitourinary disorders, which can nevertheless have a major impact on their morbidity. We present the case of a 7-year-old boy with junctional EB who presented with dysuria and lower back pain caused by bladder lithiasis complicating posterior urethral stenosis, surgical management, and postoperative outcomes.

Large and rapidly growing abdominal tumors endanger vital prognosis in neonates. Sometimes, quickly worsening patients’ clinical conditions demand surgical decision-making that cannot wait for precise histological diagnosis. There is neither consensus nor rules to manage these situations. We presented our experience with five patients and we looked in the literature for the best possible management of such rare disease. Laparostomy should be considered only as a safe-life procedure and if the liver is affected by the tumor and a coagulopathy is present, the prognosis is often compromised.

Prematurity affects 6.6 % of births in France [1,2]. It is the first cause of neonatal mortality [3]. It induces a high rate of neonatal morbidity correlated with gestational age...

Resume Le traitement endoscopique est la reference dans la prise en charge des reflux vesico-ureteraux (RVU) symptomatiques de bas grade chez l’enfant. L’injection in situ de Deflux ® (acide hyaluronique et dextranomere) est efficace et presente peu de complications. Nous presentons deux cas de mega-ureteres secondaires a l’injection de Deflux ® . Le premier patient etait un garcon presentant un RVU bilateral de grade IV decouvert a l’occasion d’une pyelonephrite. Il avait beneficie d’une injection bilaterale de Deflux ® (trois ampoules au total) a l’âge de 4 ans. L’echographie postoperatoire etait normale. Un controle a trois ans a montre une dilatation uretero-pyelocalicielle gauche importante avec stase et degradation de la fonction renale. Une intervention chirurgicale selon Cohen a ete realisee. Le second etait une fille chez qui le diagnostic de RVU bilateral avait ete fait a la naissance. Elle avait beneficie d’une injection bilaterale de Deflux ® (une ampoule par uretere) a l’âge de 12 mois. Un mois plus tard, l’echographie a montre une dilatation des ureteres distaux (diametre = 10 mm a droite et 6,7 mm a gauche). Elle a ete operee 8 mois plus tard (technique de Cohen) du fait de pyelonephrites recidivantes et de la persistance de la dilatation. Ces deux cas de mega-ureteres ont ete observes parmi un total de 452 injections (4‰). En conclusion, le traitement endoscopique par Deflux ® est un geste mini-invasif qui ameliore la situation de facon transitoire dans les RVU. Hormis l’echec, il existe un faible risque de dilatation secondaire justifiant un controle echographique a long terme.

Resume Introduction Quelle prise en charge pour les mega-ureteres primitifs chez les enfants de moins de un an ? La question est discutee dans la litterature et la controverse persiste. Objectif Evaluer les risques et les resultats a long terme des prises en charges medicale et chirurgicale chez les enfants de moins de un an. Materiel et methode Etude retrospective monocentrique de 1990 a 2010. Tous les enfants de moins de un an retrouves ont eu une evaluation incluant un examen clinique, une echographie, une scintigraphie et une cystographie. Ils ont ete repartis en deux groupes : groupe 1 : enfants operes avant l’âge de un an, groupe 2 : enfants non operes ou operes apres l’âge de un an. Nous avons analyse l’evolution a long terme de ces enfants sur les criteres suivants : reflux, pyelonephrites, evolution de la dilatation, fonction renale, necessite d’une reprise chirurgicale ou d’une chirurgie secondaire, et impact sur la fonction vesicale. Resultats Soit 54 patients dans le groupe 1 et 56 dans le groupe 2. Pour un suivi median de 12 ans. Un total de 101 garcons pour 9 filles (sex-ratio 11,22). Il y avait 57 MUP gauche (52 %), 22 droit (20 %) et 31 bilateraux (28 %). Un total de 71 % de diagnostic antenatal. Pas de difference sur l’emergence de complications : 25 (groupe 1) versus 31 (groupe 2) OR = 0,69 ; IC 95 % (0,307 ; 1,574) ; p = 0,44. Pas de difference entre chirurgie secondaire et reprise chirurgicale : groupe 1 = 12, groupe 2 = 22, OR = 0,45 ; IC 95 % (0,17 ; 1,09) ; p = 0,06. Pas de difference pour l’incontinence diurne : OR = 1,04 ; IC 95 % (0,14 ; 7,64) ; p = 0,67. Soixante-seize enfants (69 %) avaient finalement ete operes, 12 enfants operes deux fois (10,9 %) et 34 enfants (31 %) jamais operes. Conclusion Le principal enjeu de la prise en charge des MUP est la preservation de la fonction renale. Soixante-neuf pour cent de nos enfants ont beneficie d’un traitement chirurgical a cause d’une alteration de la fonction renale inferieure a 30 %, d’une dilatation ureterale superieure a 10 mm associee a un reflux ou de pyelonephrites recidivantes. Un suivi clinique, echographique et isotopique regulier de depistage est necessaire et devrait etre prolonge jusqu’a l’âge adulte. Niveau de preuve 5.

The hypospadiac genital tubercle (GT) can be defined as a development arrest of the tissues forming its ventral aspect which occurs during the “masculinization window” (6–16 weeks of gestation). The causes of this common congenital malformation are essentially unknown and probably multifactorial. Four main actors may be responsible of hypospadias: (1) The child's genes and hormonal system; (2) The placenta; (3) The mother, and (4) the environment with its potential hormonal disruptors. Assessment of the hypospadiac GT is an important step to evaluate the context and plan surgery which, in some cases, can be preceded by hormonal stimulation. Surgery entails three main steps: (1) Degloving the GT to identify the level of division of the corpus spongiosum which is the main criteria to define hypospadias severity; (2) Choosing the adequate technique of urethroplasty; (3) Covering the straightened GT with penile skin. Evaluation of outcome is another critical step and includes functional and cosmetic results until adulthood.

International audience; Ureteropelvic junction obstruction constitutes a major cause of progressive pediatric renal disease. The biological mechanisms underlying the renal response to obstruction can be investigated using a clinically relevant mouse model of partial unilateral ureteral obstruction (pUUO). Renal function and kidney morphology data can be evaluated using renal ultrasound, scintigraphy and uro-magnetic resonance imaging (uro-MRI), but these methods are poorly linked to histological change and not all are quantitative. Here, we propose to investigate pUUO for the first time using an intravoxel incoherent motion diffusion sequence. The aim of this study was to quantitatively characterize impairment of the kidney parenchyma in the pUUO model. This quantitative MRI method was able to assess the perfusion and microstructure of the kidney without requiring the injection of a contrast agent. The results suggest that a perfusion fraction (f) reduction is associated with a decrease in the volume of the renal parenchyma, which could be related to decreased renal vascularization. The latter may occur before impairment by fibrosis and the findings are in accordance with the literature using the UUO mice model and, more specifically, on pUUO. Further investigation is required before this technique can be made available for the diagnosis and management of children with antenatal hydronephrosis and to select the optimal timing of surgery if required.Copyright © 2017 John Wiley & Sons, Ltd.

Objectifs : Faire le recensement des internes et chefs de clinique des universites-assistants des hopitaux (CCA) en formation en chirurgie pediatrique et analyser les resultats en fonction des besoins de la specialite. Materiel et methodes : Les donnees concernant la demographie des chirurgiens pediatres en formation ont ete recueillies par un questionnaire adresse aux internes, CCA et coordonnateurs interregionaux de la specialite, de septembre 2009 a mars 2010. Les informations suivantes ont ete recueillies : nom et prenom, sexe, annee de validation du concours de l’internat ou des epreuves classantes nationales, orientation choisie en chirurgie pediatrique, le centre hospitalier universitaire (CHU) de formation durant l’internat, lieu d’exercice. Resultats : Cinquante-trois internes sont inscrits en diplome d’etudes specialisees complementaires (DESC) de chirurgie infantile avec une orientation urologique, viscerale, thoracique ou plastique et vingt-et-un en orthopedie pediatrique. Par ailleurs treize internes en DESC d’orthopedie adulte vont s’orienter vers l’orthopedie pediatrique. La repartition des internes sur le territoire montre des differences importantes entre les inter-regions. Il existe 55 postes de CCA en chirurgie pediatrique. Conclusion : Cette etude devra servir de socle aux reflexions engagees par la Commission nationale de l’internat et du post-internat. Le nombre important d’internes formes chaque annee montre l’attractivite de la chirurgie pediatrique pour les chirurgiens en formation. Il nous alerte sur la necessite d’adapter la formation des chirurgiens pediatres aux capacites de formation de notre systeme de sante et aux besoins de sante de la population francaise.

Immune-mediated glomerulonephritis is caused by deposition of immune complexes on the glomerular basement membrane or of autoantibodies directed against the glomerular basement membrane. Depositions lead to an inflammatory response that can ultimately destroy renal function and lead to chronic kidney disease. However, the pathological processes leading to the development of renal injury and disease progression remain poorly understood. To investigate the mechanisms of disease development in glomerulonephritis various animal models have been developed, which include as the most popular one the induction of glomerulonephritis by the injection of heterologous antibodies directed to the glomerular basement membrane. The role of mast cells and mast cell-derived mediators has been evaluated in these models. In this chapter we describe the methods that allow to set up and study the disease parameters of immune-mediated glomerulonephritis development.

Immune-mediated glomerulonephritis is caused by deposition of immune complexes on the glomerular basement membrane or of autoantibodies directed against the glomerular basement membrane. Depositions lead to an inflammatory response that can ultimately destroy renal function and lead to chronic kidney disease. However, the pathological processes leading to the development of renal injury and disease progression remain poorly understood. To investigate the mechanisms of disease development in glomerulonephritis various animal models have been developed, which include as the most popular one the induction of glomerulonephritis by the injection of heterologous antibodies directed to the glomerular basement membrane. The role of mast cells and mast cell-derived mediators has been evaluated in these models. In this chapter we describe the methods that allow to set up and study the disease parameters of immune-mediated glomerulonephritis development.

Mast cells are hematopoietic cells involved in inflammation and immunity and have been recognized also as important effector cells in kidney inflammation. In humans, only a few mast cells reside in kidneys constitutively but in progressive renal diseases their numbers increase substantially representing an essential part of the interstitial infiltrate of inflammatory cells. Recent data obtained in experimental animal models have emphasized a complex role of these cells and the mediators they release as they have been shown both to promote, but also to protect from disease and fibrosis development. Sometimes conflicting results have been reported in similar models suggesting a very narrow window between these activities depending on the pathophysiological context. Interestingly in mice, mast cell or mast cell mediator specific actions became also apparent in the absence of significant mast cell kidney infiltration supporting systemic or regional actions via draining lymph nodes or kidney capsules. Many of their activities rely on the capacity of mast cells to release, in a timely controlled manner, a wide range of inflammatory mediators, which can promote anti-inflammatory actions and repair activities that contribute to healing, but in some circumstances or in case of inappropriate regulation may also promote kidney disease.

Summary Objectives The Mitrofanoff principle is an accepted continent urinary diversion. We studied the feasibility and the possible benefits of using a laparoscopic approach in children with significant bladder dysfunction associated with difficulty doing efficient urethral catheterization. Patients and methods A fully laparoscopic Mitrofanoff continent cystostomy was attempted in 15 children with a median age of 9 years (IQR 6), between 2003 and 2013. Before the Mitrofanoff procedure was considered, urodynamic evaluation was done for each patient, to study bladder compliance, detrusor activity, and bladder capacity. The procedure was performed using a transperitoneal four-port approach. A 30-degree down camera angle was optimal for viewing the appendix and the posterior wall of the bladder. The operative steps of the open procedure were replicated laparoscopically. The proximal end of the appendix was spatulated and anastomosed to the posterior wall of the bladder, providing an antireflux mechanism by an extramucosal tunnel. The distal end of the appendix was brought out as the cutaneous umbilical stoma. Some modifications were done because of the high rate of conversion due to early opening of the mucosa (harmonic hook) or difficult anastomosis: (a) use of 5-mm trocars to change the laparoscope position from the left to right subcostal area to better visualize the anastomosis, (b) the anastomosis was suspended at its two ends during suturing; a trans-abdominal traction suture of the bladder was inserted for better exposure of the anastomosis (hitch stitch) and to stabilize the anastomotic line during suturing, (c) use of a monopolar hook to cut the detrusor muscle fibers, to avoid incidental opening of the mucosa, and (d) the window between the appendix and the peritoneum was closed to avoid internal hernia. Results The procedure was totally completed by laparoscopy in 12 cases. Three were converted to an open procedure due to tearing of bladder mucosa (n = 2) or appendix ischemia (n = 1). Median operative time for fully laparoscopic Mitrofanoff was 255 min (IQR 52). Median follow-up was 18 months (IQR 35). No patient required stomal revision. Seven patients were continent, five experienced urinary leakage from urethra n = 1 and/or stoma n = 5. Three patients with stomal urinary leakage were successfully managed by Deflux (dextranomer-based implants) injection in the catheterizable channel. Two patients required an open revision of the appendicovesical anastomosis. The patient with both stomal and urethral urinary leakage also required the implantation of an artificial urinary sphincter 1.5 years after Mitrofanoff. One patient had bladder augmentation. Conclusion Although our results of laparoscopic Mitrofanoff procedure in children are unsatisfying in cases of high-pressure bladders in terms of incontinent stoma, we still believe that it is justified to develop this challenging technique with more refinement and improvement, to provide a minimal invasive procedure that may postpone or even avoid bladder augmentation in pediatric age.