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To report experience and technical refinements with the Koyanagi urethroplasty for proximal hypospadias.A retrospective study was conducted between 2004 and 2017. Medical records of patients treated by the Koyanagi technique were investigated. The penile ventral skin was closed either with a Byars flap or using an "Ombredanne's chasuble" (OC). The cohort was divided chronologically into two groups of the same number of patients (early -E- and late experience group-LEG-). Demographics, anatomical findings and surgical outcomes were compared between groups using univariate analysis. A logistic regression was performed to assess factors associated with the occurrence of a postoperative urethrocutaneous fistula or urethroplasty dehiscence (UD).The Koyanagi urethroplasty was performed in 67 patients, 33 in the EEG and 34 in the LEG. The overall surgical complication rate was 64.2% (n=43), including 42 fistula or UD, higher in the EEG (81.8%) than in the LEG (44.1%, P0.01). However, in the LEG, patients underwent the surgery older and the use of OC more frequent. After multivariate analyses, factors associated with a postoperative fistula or UD were the year of surgery (OR=0.71 [0.53-0.96] P=0.02), the age at surgery (OR=1.11 [1.01-1.22], P=0.03); contrary to the skin coverage method or the stenting duration (P0.05). An urethral stenosis occurred in 1 patient (1.5%). At last follow-up, 64.2% of patients required a further procedure and 80.6% of urethral meatus were glandular.In this study the complication rate, particularly the urethrocutaneous fistula, remained high. Urethral stenosis, were rare but the follow-up was too short to clearly identified them. Considering as a 2 stage procedure koyanagi uretroplasty allows to obtain finally good results.III.

Supplementary Figure S1; Supplementary Table S1.

Purpose: Germline hSNF5/INI1 mutations are responsible for hereditary cases of rhabdoid tumors (RT) that constitute the rhabdoid predisposition syndrome (RPS). Our study provides the first precise overview of the prevalence of RPS within a large cohort of RT.Experimental Design: hSNF5/INI1 coding exons were investigated by sequencing and by multiplex ligation-dependent probe amplification.Results: Seventy-four constitutional DNAs from 115 apparently sporadic RT were analyzed from 1999 to 2009. Germline mutations were found in 26 patients (35%). Data from 9 individuals from 5 RPS families (siblings) were also studied. The median age at diagnosis was much lower (6 months) in patients with germline mutation (P < 0.01) than in patients without (18 months). Nevertheless, 7 of 35 patients with germline mutation (20%) developed the disease after 2 years of age. The mutation could be detected in only 1 parent whereas germline blood DNA was wild type in the 20 other parent pairs, therefore indicating the very high proportion of germ-cell mosaicism or of de novo mutations in RPS. The former hypothesis could be clearly documented in 1 case in which prenatal diagnosis was positive in a new pregnancy. Finally, the 2 years' overall survival was 7% in mutated and 29% in wild-type patients, mainly due to the worse outcome of RT in younger patients.Conclusions: Our results show a high proportion of germline mutations in patients with RT that can be found at any age and up to 60% in the youngest patients. Genetic counseling is recommended given the low but actual risk of familial recurrence. Clin Cancer Res; 17(1); 31–8. ©2011 AACR.

Introduction Multiple intestinal atresia (MIA) is a rare cause of neonatal intestinal obstruction. To provide an overview of the current prenatal, surgical, and nutritional management of MIA, we report our experience and a literature review of papers published after 1990. Methods All cases of isolated MIA (non-hereditary, not associated with apple-peel syndrome or gastroschisis) treated at our institution between 2005 and 2016 were reviewed and compared with cases found in the literature. Results Seven patients were prenatally suspected of having intestinal obstruction and were postnatally diagnosed with MIA, with a mean 1.7 (1–2) resections–anastomoses (RA) and 6 (1–10) strictureplasties performed, resulting in a mean resected bowel length of 15.1 cm (15–25 cm). Median time to full oral feed was 46 days (14–626 days). All patients were alive and none had orality disorder after a mean follow-up of 3.1 years (0.2–8.1 years). Three surgical strategies were found in the literature review: multiple RA (68%, 34/50) including Santulli's technique in four of 34 (12%) and anastomoses over a transanastomotic tube (32%, 16/50), with a 98% survival rate, and short-bowel syndrome for only two patients. Conclusion Bowel-sparing surgery and appropriate medical management are key to ensuring a favorable nutritional and gastrointestinal outcome and a good prognosis. Prenatal assessment and standardization of the surgical course of treatment remain challenging.

Pre- and post-pubertal testicular tumors are two distinct entities in terms of epidemiology, diagnosis and treatment. Most pre-pubertal tumors are benign; the most frequent are teratomas, and the most common malignant tumors are yolk-sac tumors. Post-pubertal tumors are similar to those found in adults and are more likely to be malignant. Imaging plays a pivotal role in the diagnosis, staging and follow-up. The appearance on ultrasonography (US) is especially helpful to differentiate benign lesions that could be candidates for testis-sparing surgery from malignant ones that require radical orchidectomy. Some specific imaging patterns are described for benign lesions: epidermoid cysts, mature cystic teratomas and Leydig-cell tumors. Benign tumors tend to be well-circumscribed, with decreased Doppler flow on US, but malignancy should be suspected when US shows an inhomogeneous, not-well-described lesion with internal blood flow. Imaging features should always be interpreted in combination with clinical and biological data including serum levels of tumor markers and even intra-operative frozen sections in case of conservative surgery to raise any concerns of malignity. This review provides an overview of imaging features of the most frequent testicular and para-testicular tumor types in children and the value of imaging in disease staging and monitoring children with testicular tumors or risk factors for testicular tumors.

Botryoid growth pattern (BGP) is a polypoid mass growing into the renal pelvis, rarely seen in bilateral Wilms tumors where it represents a surgical challenge. We report our experience of nephron sparing surgery in 3 patients with BGP in bilateral Wilms tumor. Surgical en bloc removal was performed after calyx opening with no complications. The histology of the BGP was Intralobar Nephrogenic Rest in all cases while all Wilms tumors were of intermediate risk. One patient early recurred. At a follow-up of 9 months, 22 and 23 years, all patients were alive with a moderate renal insufficiency and hypertension.

Outcome of children with bladder-prostate rhabdomyosarcoma (RMS) has improved with multimodal therapies, including surgery and/or radiotherapy for local treatment. Our aim was to report long-term urological complications after a conservative approach combining conservative surgery and brachytherapy.Eighty-six patients, free of disease, were retrospectively reviewed. Symptoms related to urinary tract obstruction, incontinence, infection, and lithiasis were reported and graded according to the Common Terminology Criteria for Adverse Events (CTCAE) classification. Only symptomatic patients underwent urodynamic studies. Risk factors for complications were analyzed.There were 76 males and 10 females. The median follow-up was 6.3 years (18 months to 24 years). Complications occurred after a median follow-up of 5 years (0-21). Twenty-two patients (26%) had long-term urological complications. Urinary tract obstruction was found in 15 patients (17%) and urinary incontinence in 14 patients (16%). Recurrent urinary tract infection occurred in four patients and urinary lithiasis in four (5%). The underlying physiopathology included bladder dysfunction in 15 patients (17%), urethral stenosis in six (7%), and ureterovesical junction stenosis in five (6%). On univariate analysis, posterior bladder wall dissection (p = .001), bladder neck trigone dissection (p = .010), and partial prostatectomy (p = .023) were significantly associated with an increased risk of bladder dysfunction; on multivariate analysis, only age ≤2 years (p = .028) at operation and posterior bladder wall dissection (p = .006) were found to be significant.The conservative surgical approach combined with brachytherapy for bladder-prostate RMS leads to long-term urological complications in 26% of survivors. Optimizing brachytherapy doses for young children and establishing a clear and long-term follow-up protocol could help to reduce these complications.

The umbilical hernia is common in children. Most of the cases have a spontaneous regression around the age of 3 years. Complications are very rare, and thus surgery is not routinely indicated before the age of 3 years. We report an exceptional case of spontaneous rupture of an umbilical hernia with emphasis on the management of this rare complication and a literature review of similar cases.

INTRODUCTION Necrotizing Enterocolitis (NEC) remained a dramatic complication leading to death or neonatal morbidities in preterms. For some, Intra-Abdominal Hypertension (IAH) and Abdominal Compartment Syndrome worsened the multi-organ failure. An open abdomen surgery could be an alternative to conventional surgical treatment to move beyond this stage. OBJECTIVES To retrospectively describe the clinical course, pre- and post-operative features of preterms suffering from severe NEC with IAH treated by open abdomen surgery and referred to our center from October 2007 to September 2019. Our secondary objective is to identify various risk factors for mortality in this population. METHODS Data on neonatal, clinical, biological, pre and post-operative features and outcome were collected. Univariate analyses were performed to compare their pre and post-operative features stratifying on outcome. RESULTS Among 29 included patients, 14 (48%) survived to discharge without short bowel syndrome. Death was associated with an earlier postnatal age at NEC (16.3 ± 9.1 versus 31.3 ± 25.9 days; p = 0.004) and followed a withdrawal of treatment in 60% of cases. Surgery was associated with a significant improvement of respiratory and hemodynamic features (decrease of mean ventilator pressure from 13.1 ± 5.4 to 11.3 ± 4.0 cmH2O, p

Background: Outcome of children with bladder-prostate rhabdomyosarcoma has improved with multimodal therapies, including surgery and/or radiotherapy for local treatment. Our aim was to report the long-term urological complications after a conservative approach combining conservative surgery and brachytherapy. Patients and methods: Eighty-six patients, free of disease, were retrospectively reviewed. Symptoms related to urinary tract obstruction, incontinence, infection and lithiasis were reported and graded according to CTCAE classification. Only symptomatic patients underwent urodynamic studies. Risk factors for complications were analyzed. Results: There were 76 males and 10 females. The median follow-up was 6.3 years (18 months-24 years). Complications occurred after a median follow up 5 years (0-21). Twenty-two patients had long-term urological complications. Urinary tract obstruction was found in 15 patients, urinary incontinence in 14 patients. Recurrent urinary tract infection and urinary lithiasis were found in 5 patients respectively. Beyond symptom, 3 etiologies were identified: bladder dysfunction in 15 patients, urethral stenosis in 6 and uretero-vesical junction stenosis in 5. Posterior bladder wall dissection used in large prostatic tumors, operation at age less than 2 years and partial prostatectomy were identified as risk factors for these complications. Conclusion: The conservative surgical approach combined with brachytherapy for BPRMS leads to long-term urological complications in 22% of patients free of their disease. Optimizing brachytherapy doses for young children and establishing a clear and long term follow-up protocol, could help to reduce these complications.

Rationale Three-dimensional reconstruction (3DR) of preoperative images may improve the presurgical assessment of tumours prior to removal. We aimed to analyse the advantages and discrepancies of preoperative 3DR in paediatric tumours. Methods We conducted a prospective observational study from 2016 to 2019, including patients with thoraco-abdominal tumours having predictable surgical risks on preoperative images (encasement of vessels posing vascular risks, ie, neuroblastic and soft tissue tumours or parenchyma preservation of the invaded organ, ie, liver and kidney). A comparison of 2D/3DR and surgical findings was performed. Results Twenty-four patients, with a median age at surgery of 68.2 months (13 days-203 months), were operated on for neuroblastoma (n = 7), renal tumour (n = 7), hepatic tumour (n = 4) and others (n = 6; bone sarcoma of the iliac branch, abdominal lymph nodes of a recurrent testicular germ cell tumour, pseudoinflammatory tumour of the omentum, thoracic lipoblastoma, desmoplastic tumour, solid and pseudopapillar tumour of the pancreas). Reconstruction was of poor quality in two patients with renal tumours because computed tomography (CT) had no excretory phase. Discrepancies between 3DR and surgical findings occurred in two patients, one because of poor assessment of caliceal infiltration by renal nodules and the other because of inadequate reconstruction of renal vein thrombosis. For all the other tumours, 3DR improved the visualisation and precise location of vessels during surgery. Conclusion High-quality preoperative images are mandatory to provide the best 3DR. In the majority of cases, 3DR is of significant help during surgery to better identify vascular structures within tumours and preserve parenchyma.

Background Liver metastases are rare in children with Wilms tumor (WT), and their impact on the outcome is unclear. Patients and methods The French cohort of patients with WT presenting liver metastases at diagnosis and enrolled in the International Society of Pediatric Oncology (SIOP) 2001 study was reviewed. Results From 2002 to 2012, 906 French patients were enrolled in the SIOP2001 trial. Among them, 131 (14%) presented with stage IV WT and 18 (1.9%) had liver metastases at diagnosis. Isolated liver metastases were displayed in four of them. After preoperative chemotherapy, persistent liver disease was reported in 14/18 patients, and 13 of them underwent metastasectomy after nephrectomy. In resected liver lesions, the same histology of the primary tumor was reported for three patients, blastemal cells without anaplasia were identified in one patient with DA-WT, and post-chemotherapy necrosis/fibrosis was identified for the other 10 patients. For the four patients who had liver and lung surgery, both sites had nonviable cells with post-chemotherapy necrosis/fibrosis. Six patients had hepatic radiotherapy. Sixteen patients achieved primary complete remission and were alive at the last follow-up (median follow-up: 6.4 years). The only two deceased patients presented diffuse anaplasia histology. The five-year EFS and OS were 83% (60%-94%) and 88% (66%-97%), respectively. Conclusion Liver involvement does not appear to be an adverse prognostic factor in metastatic WT. The role of hepatic surgery and radiotherapy remains unclear, and should be carefully considered in case of persistent liver metastases, according to histology and radiological response to other metastatic sites.

Purpose:Human chorionic gonadotropin stimulates fetal testosterone production and contributes to normal development of male genitalia. Using population based data we hypothesized that differences in maternal free beta human chorionic gonadotropin may be associated with hypospadias.Materials and Methods:Data were obtained from the Paris Registry of Congenital Malformations (REMAPAR) (2011 to 2016). The initial study population included 3,172 pregnant women who gave birth to a singleton live born male infant with a congenital malformation. After exclusion of cases with unknown beta human chorionic gonadotropin and those with chromosomal or genetic abnormalities, the study population included 194 boys with isolated hypospadias and 1,075 controls. For cases with operative notes (125) we obtained data on type (proximal/distal) of hypospadias. Using quantile regression we compared median values of multiple of median beta human chorionic gonadotropin measured for first trimester Down syndrome screening (10th to 13th gestational weeks) for overall as well as by type of hypospadias vs controls. We also considered possible effects of placental dysfunction (maternal age, intrauterine growth retardation and preterm births) as potential confounding factors.Results:Overall the median beta human chorionic gonadotropin multiple of median was comparable for women who had an infant with hypospadias vs controls (0.99 vs 0.95, p=0.3). However, proximal hypospadias was associated with a statistically significant higher median multiple of median than distal hypospadias or unspecified (1.49 vs 0.92 vs 1.05, p=0.02). The estimates were comparable after adjustment for placental dysfunction.Conclusions:Our findings support the hypothesis that an alteration in maternal beta human chorionic gonadotropin levels is associated with hypospadias. However, this association appears to be limited to proximal hypospadias.

The radiological distinction of Wilms tumor (WT) nodules from nephrogenic rests (NR) in patients with multifocal unilateral WT or bilateral disease is challenging. The study aims to compare the radiology assessment of kidney nodules with their final histology in 48 patients. The final histology of 118 nodules corresponded to the initial radiological diagnosis while 40 (25%) nodules were misdiagnosed, 20 being initially diagnosed WT on imaging were proved to be NR at histology. The size of nodules at diagnosis might help to distinguish WT from NR before surgery. Homogeneity did not seem to be a key feature.

Aim Definitions of nephron-sparing surgery (NSS) procedures in Wilms tumor (WT) are not clear. The new UMBRELLA protocol offers a formula (NSS(X)-SRM(n)-PRM(n)-RRP(n%)) to better define the different NSS parameters. We aimed to assess the advantages and limits of this new formula. Methods This retrospective monocentric study included patients operated by NSS for WT from 1975 to 2018. We reviewed the medical records and applied the NSS formula to all included patients. Results Eighty kidneys were operated on 56 patients at a mean age of 19.2 months (4 days-7.5 years), with 49 partial nephrectomies and 31 tumorectomies. The assessment of the surgical resection margins (SRM) showed a doubt in six cases and one tumor breach. An intact pseudocapsule along the resection margin with no renal parenchyma was found in four cases at pathological resection margins (PRM) assessment, whereas a tumor breach was described in seven cases. Among the six patients with a surgical doubt, only one had a pathological stage III. There were no surgical doubts in the seven patients with tumor breach at pathology. At a mean follow-up of eight years (15 days-28.6 years), eight patients had elevated blood pressure levels. Ten had proteinuria. These two parameters were significantly increased in patients with a remaining renal parenchyma (RRP) of less than half of the initial total renal parenchyma. The serum creatinine level was normal for all except two patients. Conclusion The new NSS formula described all the crucial elements of NSS. RRP seemed essential for the evaluation of long-term renal function.

Circulating tumor DNA (ctDNA) is a powerful tool for the molecular characterization of cancer. The most frequent pediatric kidney tumors (KT) are Wilms' tumors (WT), but other diagnoses may occur. According to the SIOP strategy, in most countries pediatric KT have a presumptive diagnosis of WT if they are clinically and radiologically compatible. The histologic confirmation is established after post-chemotherapy nephrectomy. Thus, there is a risk for a small fraction of patients to receive neoadjuvant chemotherapy that is not adapted to the disease. The aim of this work is to perform molecular diagnosis of pediatric KT by tumor genetic characterization based on the analysis of ctDNA. We analyzed ctDNA extracted from plasma samples of 18 pediatric patients with KT by whole-exome sequencing and compared the results to their matched tumor and germline DNA. Copy number alterations (CNAs) and single nucleotide variations (SNVs) were analyzed. We were able to detect tumor cell specific genetic alterations-CNAs, SNVs or both-in ctDNA in all patients except in one (for whom the plasma sample was obtained long after nephrectomy). These results open the door to new applications for the study of ctDNA with regards to the molecular diagnosis of KT, with a possibility of its usefulness for adapting the treatment early after diagnosis, but also for disease monitoring and follow up.

A heterozygous intragenic duplication within the repeated area (CTGCAGCTG)×2 of the NR5A1 gene was found in a 15-year-old 46,XY DSD (disorders/differences of sex development) patient with micropenis and severe proximal hypospadias. This heterozygous duplication has already been described twice in boys with a similar phenotype, whereas a deletion of 3 amino acids at the same position in the protein SF-1 has been described in a 46,XX patient with primary ovarian failure and short stature. These data suggest that this region within the NR5A1 gene has an important role for SF-1 protein function in gonads and is a hotspot for intragenic rearrangements.

Introduction Long-gap esophageal atresia (LGEA) leads to multifactorial morbidity. The aim of this study is to analyze midterm gastrointestinal and respiratory morbidities with special assessment of feeding difficulties. Secondary goals were to determine risk factors of malnutrition and the impact of surgical techniques on morbidity. Methods We conducted a prospective observational national study. Using French national database, we reviewed medical charts of every patient treated for LGEA between 2008 and 2010 in France. Phone contact was proposed to assess orality disorders using Functional Oral Intake Scale (FOIS). Patients with complete data were included. We compared conservative management with esophageal replacement. Results We included 31 cases with a median age of follow-up of 9 years [7–10]. Median z-score for weight was −0.97 [−3.52–2.50], Conclusion Midterm morbidity in LGEA concerns 80% of our population. Gastrointestinal morbidity includes mainly dysphagia, GER, and orality disorders. This study suggests that conservative management provides more orality disorders. Other studies are mandatory to confirm it.

Background Patients with Beckwith–Wiedemann syndrome (BWS) or isolated hemihypertrophy (HH) treated for a Wilms tumor (WT) carry an increased risk of developing metachronous lesion. There are no guidelines on precise indications for nephron sparing surgery (NSS) in unilateral WT (UWT). The objective of this retrospective study was to delineate the indications of NSS in patients with BWS/HH treated for WT and to evaluate their outcome. Procedure All cases of BWS/HH treated for a WT according to SIOP protocols from 1980 to 2013 were reviewed. Patients were divided into two groups (G): isolated UWT (G1) and bilateral lesions (G2) with two subgroups: bilateral tumors suspected of malignancy (G2a), and unilateral tumor suspected of malignancy with contralateral nephroblastomatosis (G2b). Results Forty-six patients were included (34 G1, three G2a, and nine G2b). Nine NSS and 25 total nephrectomies (TN) were performed in G1, two bilateral NSS and one NSS with contralateral TN in G2a, and eight NSS and one TN in G2b. The 3-year event-free survival was 92.3% (95% CI [77.9–97.5%]). One death occurred after a local relapse following a TN for a stage III stromal WT (G1) and another after a combined local and distant relapse following a NSS for a stage I diffuse anaplastic WT (G2b). There were two metachronous WT (4%), 3 years after a TN (G1) and 12 years after a NSS (G2b). Conclusions NSS is recommended in bilateral WT and may be an option in selected UWT patients with BWS/HH because it was not associated with an increased risk of local relapse.

Summary Background The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure. Objective The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure. Design, setting, and participants From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5–37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8–33m]. Intervention RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction. Outcome measurements The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage. Results and limitations All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3–30] follow-up. Complications Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1–3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery. Conclusion The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy. Table . Primary delayed closure + Kelly Redo closure + Kelly n 20 7 Males/females 13/7 5/2 Age (mo.) 3.0 [0.5–37] 10 [8–33] Bladder dehiscence 0 0 Urethral stenosis 1/20 3/7 Urethral fistula 3/20 2/7

Background/Purpose To implement resident curriculum in France based on theoretical teaching and bed side training, the national council known as the “College Hospitalier et Universitaire de Chirurgie Pediatrique” examined the relevance and feasibility of systematically introducing simulation program in the pediatric surgery resident training. Material and methods A national simulation training program was developed and took place in a 2-day session organized in 7 simulation centers in France. The program included technical (laparoscopic/suturing technique on low-fidelity models) and nontechnical (6 scenarios for standardized consultation, and a team work scenario based on errors prevention in the operative room) skills. Evaluation of the program (Likert scale from 1 (bad) to 5 (excellent) and notation on 20 points) concerned trainees and trainers. Results 40 residents (95% of all pediatric surgery French residents) attended with a ratio of trainees/trainer of ½. The training objectives earned a score of 4.46/5. The pedagogical value of the seminar scored 4.7/5, teaching quality 17.95/20, and the overall seminar score was 17.35/20. Conclusion This program, unique nationally, was assessed very favorably by the participating residents and by the involved trainers. To our knowledge, it represents the first mandatory national simulation training program included within a surgical training model. Level of evidence Level IV.

The objective of this article is to inform health-care personnel, especially those of pediatric hospitals (pediatricians, surgeons, anesthetists, etc.), about the incidence of latex allergy in children and the adverse effects that may result if avoidance measures are not taken. The prevalence of this allergy is increasing because of repeated exposure to this ubiquitous material (in medical equipment or household products such as bottle teats or balloons). The risks are allergic reactions, ranging from benign local dermatitis to anaphylactic shock. This problem is well known and progress has been made: there is increased production of latex-free products and screening for latex-related allergy or sensitization during the pre-anesthetic evaluation to take preventive actions when planning surgery. The younger the patient exposed to latex, the higher the risk of sensitization. Therefore, the main issue raised in this article is the potential benefit of moving to latex-free health-care facilities so as to minimize the risk of allergic incidents. Many studies showed a significant decrease in the risk of allergy (sensitization and allergic reaction) when avoiding latex, but we must also consider the feasibility, the cost, and the effectiveness of such a policy.

Circulating tumor DNA (ctDNA) is a powerful tool for the molecular characterization of cancer. The most frequent pediatric kidney tumors (KT) are Wilms' tumors (WT), but other diagnoses may occur. According to the SIOP strategy, in most countries pediatric KT have a presumptive diagnosis of WT if they are clinically and radiologically compatible. The histologic confirmation is established after post-chemotherapy nephrectomy. Thus, there is a risk for a small fraction of patients to receive neoadjuvant chemotherapy that is not adapted to the disease. The aim of this work is to perform molecular diagnosis of pediatric KT by tumor genetic characterization based on the analysis of ctDNA. We analyzed ctDNA extracted from plasma samples of 18 pediatric patients with KT by whole-exome sequencing and compared the results to their matched tumor and germline DNA. Copy number alterations (CNAs) and single nucleotide variations (SNVs) were analyzed. We were able to detect tumor cell specific genetic alterations-CNAs, SNVs or both-in ctDNA in all patients except in one (for whom the plasma sample was obtained long after nephrectomy). These results open the door to new applications for the study of ctDNA with regards to the molecular diagnosis of KT, with a possibility of its usefulness for adapting the treatment early after diagnosis, but also for disease monitoring and follow up.

Summary Introduction Fistulas are a common complication of hypospadias surgery; they are more frequent after mid-shaft and posterior hypospadias repair. Surgical treatment of fistula still remains challenging with a significant failure rate. The basic principle is to add layers between skin and neourethra in order to decrease the incidence of recurrent urethrocutaneous fistula (UCF). We report our experience of UCF repair using a vascularized tunica vaginalis flap (TVF) after posterior and mid-shaft hypospadias surgery. Material and methods A retrospective review of all patients operated on using TVF for UCF in our institution between December 2005 and July 2017 was performed. Results Among 36 cases, TVF was used at a first attempt in 22 patients; 14 children had a prior attempt to close the fistula, and four of them had two surgeries before TVF repair. UCF was respectively penoscrotal (n = 3, 8%), posterior (n = 19, 53%), midshaft (n = 9, 25%) and anterior (n = 5, 14%). The size of the fistula was more than 5 mm in 26 patients. The UCF was treated successfully in every case after one single procedure. In the three children with two fistulas, both fistulas were successfully treated by the same TVF. After an average follow-up time of 45 months there was no recurrence of the initial UCF. In four cases of undescended testis, it was possible to dissect the flap through an inguinal incision and perform an orchydopexy in the same time. One patient presented a testicular atrophy after undescended testis surgery. Discussion Area review of published series shows excellent results in UCF repair including recurrent fistula (Table). TVF can aspire to some advantages with regard to a dartos flap (DF). First of all, a nearly 2.5-fold lower incidence of fistula after fistula repair with TVF than with DF (5.1% vs. 12.2%) has been shown. Secondly, TVF allows treating multiple fistulas, and can also be brought to the anterior part of the penis until the balano preputial furrow, allowing curing anterior fistula. Furthermore, it doesn't lead to aesthetic complications such as penile rotation or distal skin necrosis, which can occur during DF procedures. Conclusion TVF is a simple and reproductive technique for UCF repair, with a high success rate. The risk of testicular atrophy has to be considered in case of associated undescended testis surgery, and careful attention must be given to the TVF dissection. This technique should be considered as first choice treatment for any UCF. Table . Literature review. Authors Date Cases (n) Including recurrent UCF Average length of urethral stent (days) Average follow-up (months) Fistula recurrence after TVF Landau et al. 2003 14 14 (100%) 5.5 44 0% Routh et al. 2006 16 12 (75%) 7 18 0% Xie'eryazidan et al. 2009 26 ? ? ? 15% Muruganandham et al. 2010 13 4 (31%) 7–10 28 0% Kadian et al. 2011 14 14 (100%) 10 ? (maximum 2 years) 0% Pescheloche et al. 2017 36 14 (39%) 7 45 0%

Resume Les enfants et les adolescents peuvent etre confrontes a des evenements qui menacent leur vie ou leur integrite. Suite a ces expositions, l’etat de stress post-traumatique (ESPT) est la consequence psychopathologique la plus frequente. L’âge n’etant pas un facteur de protection, ce trouble peut se reveler grave et durable dans le temps. La prise en charge psychotherapeutique de l’ESPT chez l’enfant est loin d’etre systematique et les recherches sur le sujet sont rares chez l’enfant. Cet article expose la resolution d’un ESPT chez une jeune fille de 12 ans victime d’un accident de manege dans sa petite enfance, par une prise en charge par hypnose dans le cadre d’une therapie breve. Ce choix psychotherapeutique a ete fait sur la base des caracteristiques communes partagees par l’hypnose et les symptomes post-traumatiques que presentait l’enfant, en particulier les phenomenes de dissociation. Les manifestations cliniques de l’ESPT ont disparu en quatre semaines, sans recidive a un an. Ce fait clinique suggere que l’hypnose pourrait etre une therapie efficace et innovante pour la prise en charge des enfants presentant un ESPT. Des recherches prospectives sur un grand nombre de patients seront necessaires pour valider cette hypothese.

Resume Introduction La prise en charge urologique des vessies neurologiques d’origine medullaire de l’enfant a pour objectif d’obtenir une continence sociale tout en preservant le haut appareil, en combinant le catheterisme vesical intermittent aux anticholinergiques. L’objectif de cette etude etait de rapporter les resultats sur la continence de la prise en charge endoscopique vesico-sphincterienne des enfants avec intolerance ou echec du traitement de premiere intention. Patients et methodes Sur les 364 enfants suivis pour vessie neurologique dans notre institution, 22 ont necessite une prise en charge endoscopique entre 2000 et 2012. L’incontinence urinaire etait liee a une hyperactivite detrusorienne pour 16 et a une insuffisance sphincterienne pour 13, necessitant une ou plusieurs injections de toxine botulique A (TBA) intradetrusorienne et/ou de dextranomere-acide hyaluronique (Dx/Ha) dans le col vesical. La continence etait reevaluee entre six et huit semaines par le score de Schulte-Baukloh. Resultats Au terme d’un suivi moyen de quatre ans, 16 enfants avaient recu 54 injections de TBA et 13 enfants avaient recu 24 injections de Dx/Ha. La continence sociale, definie par un score 0 ou 1, etait acquise rapidement apres injection de TBA et necessitait de repeter les injections tous les 8,7 mois (6–12) avec une tres faible morbidite. Concernant le Dx/Ha, 69 % des enfants ont ete ameliores de maniere significative apres la premiere injection et avec de meilleurs resultats chez les filles, passant d’un score 1 a un score 0 ou d’un score 2–3 a un score 1. Conclusion Une prise en charge endoscopique adaptee a permis une amelioration de la continence chez deux tiers des enfants en echec apres un traitement de premiere intention de leur vessie neurologique, permettant de differer ou d’eviter une intervention chirurgicale lourde.

Objectives According to the Renal Tumor Study Group (RTSG) of the International Society of Paediatric Oncology (SIOP), diagnostic biopsy of renal tumors prior to neoadjuvant chemotherapy is not mandatory unless the presentation is atypical for a Wilms tumor (WT). This study addresses the relevance of this strategy as well as the accuracy and safety of image-guided needle biopsy. Methods Clinical, radiological, and pathological data from 317 children (141 males/176 females, mean age: 4 years, range, 0-17.6) consecutively treated in one SIOP-affiliated institution were retrospectively analyzed. Results Presumptive chemotherapy for WT was decided for 182 patients (57% of the cohort), 24 (8%) were operated upfront, and 111 (35%) were biopsied at diagnosis. A non-WT was confirmed after surgery in 5/182 (3%), 11/24 (46%), and 28/111 (25%), respectively. Age at diagnosis was the most commonly (46%) used criterion to go for biopsy but a nine-year threshold should be retrospectively considered more relevant. Tumor volumes of clear cell sarcoma of the kidney and WT were significantly higher than those of other tumors (P = 0.002). The agreement between core-needle biopsy (CNB) and final histology was 99%. No significant morbidity was associated with CNB. Conclusion The use of SIOP criteria to identify patients eligible for presumptive WT neoadjuvant chemotherapy or upfront surgery avoided biopsy in 65% of children and led to a 97% rate of appropriate preoperative chemotherapy. Image-guided CNB is a safe and accurate diagnostic procedure. The relevance of SIOP biopsy criteria might be improved by using an older age threshold (9 years instead of 6 years) and by adding initial tumor volume.

Background The three sequential SIOP MMT studies provide the largest dataset available to date, to define the patient and tumour characteristics, treatment modalities and event-free and overall survival for children with non metastatic rhabdomyosarcoma (RMS) of the bladder and/or prostate (BP). Procedure The combined dataset of 172 patients with BP RMS treated on the SIOP MMT 84, 89 and 95 studies was reviewed to determine tumour characteristics, details of treatment and outcome. Results Median age at diagnosis was 2.5 years (range 2 months–17.8 years) and 138 (79%) were males. Median follow-up was 11.4 years (range 3 months–22 years). The 5-year overall survival of the combined cohort was 77% (CI 70–83%). The 5-year event-free survival was 63% and included 7 patients (4%) who did not achieve complete remission (CR), and 57 (33%) who relapsed. Age ≥ 10 years (RR 3.7) and alveolar pathology (RR 3.3) were identified as independent prognostic factors on multivariate analysis. Fifty-nine (50%) of the 119 survivors were cured without significant local therapy, improving from 31% in MMT84 study to 61% in MMT95 study. Conclusion The clinical strategy of the MMT studies aims to minimise the burden of therapy whilst maintaining survival rates. Overall survival is comparable to that of other international groups, despite the lower use of radiotherapy and or radical surgery, although number of events experienced is higher. Further assessment of the late effects of therapy is required to confirm whether this approach results in lower morbidity in the long-term. Pediatr Blood Cancer 2014;61:217–222. © 2013 Wiley Periodicals, Inc.

Resume L’objectif de ce travail etait de realiser une mise au point sur la place de la chirurgie video-assistee en chirurgie viscerale pediatrique et de discuter ses perspectives d’avenir. Les techniques de videochirurgie ont permis de minimiser l’impact esthetique et traumatique des interventions en chirurgie viscerale et sont en constant developpement chez l’adulte comme chez l’enfant. Une formation plus precoce des chirurgiens a ces techniques, associee aux progres anesthesiques et aux ameliorations technologiques, a ameliore le savoir-faire, permettant de pratiquer des interventions plus complexes par cette voie d’abord. Si la plupart des interventions viscerales ont deja ete realisees par voie cœlioscopique, la litterature peine a la definir comme un gold standard , notamment en raison des difficultes a demontrer des benefices esthetiques ou de diminution des douleurs. L’interet de la cœlioscopie pour la cholecystectomie est desormais admis en termes de suites operatoires sans augmentation des complications. Pour le traitement chirurgical du reflux gastro-œsophagien et pour la splenectomie, aucune etude pediatrique ne conclut a la superiorite de la cœlioscopie mais des donnees chez l’adulte la suggerent. L’abord par lomboscopie permet de realiser des interventions urologiques en evitant de larges lombotomies. La cœlioscopie peut servir egalement d’outil diagnostique, comme pour les testicules non palpables, suivi d’un geste therapeutique si necessaire. L’appendicectomie par cette technique reste encore sujette a debat. Les etudes sont contradictoires et la chirurgie ouverte garde une place importante, mais la proportion de cœlioscopie dans cette indication semble augmenter. La cœlioscopie reste controversee pour la chirurgie oncologique pediatrique.

Purpose To report the results of a conservative strategy based on partial surgery combined with brachytherapy in a prospective cohort of children with bladder–prostate rhabdomyosarcoma (BP RMS). Methods and Materials We prospectively documented the outcome of children treated in our department between 1991 and 2015 for BP RMS and undergoing a multimodal approach combining conservative surgery (partial cystectomy and/or partial prostatectomy) and perioperative interstitial low-dose-rate or pulse-dose-rate brachytherapy. Before brachytherapy, children had received chemotherapy with modalities depending on their risk group of treatment. Results A total of 100 patients were identified, with a median age of 28 months (range, 5.6 months-14 years). According to the Intergroup Rhabdomyosarcoma Study (IRS) group, 84 were IRS-III, and 12 were IRS-IV tumors. Four patients were treated at relapse. The median number of chemotherapy cycles before local therapy was 6 (range, 4-13). After surgery, 63 patients had a macroscopic tumor residuum. Five patients underwent a brachytherapy boost before pelvic external beam radiation therapy because of nodal involvement, and 95 had exclusive brachytherapy. Median follow-up was 64 months (range, 6 months-24.5 years). Five-year disease-free and overall survival rates were 84% (95% confidence interval 80%-88%) and 91% (95% confidence interval 87%-95%), respectively. At last follow-up most survivors presented with only mild to moderate genitourinary sequelae and a normal diurnal urinary continence. Five patients required a secondary total cystectomy: 3 for a nonfunctional bladder and 2 for relapse. Conclusion Brachytherapy is effective as part of a conservative strategy for BP RMS, with a relatively low delayed toxicity as compared with previously published studies using external beam radiation therapy. Longer follow-up is required to ensure that the functional results are maintained over time.

Patients with Beckwith-Wiedemann syndrome (BWS) or isolated hemihypertrophy (HH) treated for a Wilms tumor (WT) carry an increased risk of developing metachronous lesion. There are no guidelines on precise indications for nephron sparing surgery (NSS) in unilateral WT (UWT). The objective of this retrospective study was to delineate the indications of NSS in patients with BWS/HH treated for WT and to evaluate their outcome.All cases of BWS/HH treated for a WT according to SIOP protocols from 1980 to 2013 were reviewed. Patients were divided into two groups (G): isolated UWT (G1) and bilateral lesions (G2) with two subgroups: bilateral tumors suspected of malignancy (G2a), and unilateral tumor suspected of malignancy with contralateral nephroblastomatosis (G2b).Forty-six patients were included (34 G1, three G2a, and nine G2b). Nine NSS and 25 total nephrectomies (TN) were performed in G1, two bilateral NSS and one NSS with contralateral TN in G2a, and eight NSS and one TN in G2b. The 3-year event-free survival was 92.3% (95% CI [77.9-97.5%]). One death occurred after a local relapse following a TN for a stage III stromal WT (G1) and another after a combined local and distant relapse following a NSS for a stage I diffuse anaplastic WT (G2b). There were two metachronous WT (4%), 3 years after a TN (G1) and 12 years after a NSS (G2b).NSS is recommended in bilateral WT and may be an option in selected UWT patients with BWS/HH because it was not associated with an increased risk of local relapse.

Abnormalities of the male genitalia have increased in the last 2 decades in numerous developed countries and remain a frequent reason of consultation in pediatric surgery. The diagnostic spectrum is wide, and surgeons should pay particular attention to these abnormalities because of their potential psychological effect. Anatomically, these abnormalities can affect one of three parts of the penis. First, the foreskin may not be fully retracted. This is normal at birth and can be caused by prepuce adherents that can continue until adolescence. Today, true phimosis is treated with topical corticoids from the age of 3 years. If medical treatment fails, a surgical procedure is required. Second, the urethra can be affected by hypospadia, which is the most frequent abnormality of the urethra. It is associated with ectopic urethral meatus, hypoplastic foreskin, and penis curvature. Its pathogenic background is not clearly understood. Surgery options differ according to the type of hypospadia and according to the surgeon's experience. It is sometimes hard to deal with, especially in a perineal form, where genetic and hormonal studies are recommended. These interventions can lead to complications ranging from stenosis to fistula. Therefore, parents have to be informed of the benefits and risks of the surgical procedures. Epispadias is rare but more serious because of the increasing risk of urinary incontinence. Finally, abnormalities of the corpora cavernosa - often associated with hypospadias - can include penis curvature and micropenis, for which an endocrinological analysis is essential.

International audience; Introduction After surgical repair of gastroschisis, most neonates exhibit severe intestinal dysmotility. We hypothesized that impaired development of the enteric nervous system or interstitial cells of Cajal (ICC) network contributes to impaired intestinal motility in gastroschisis. We evaluated this hypothesis in a rat model of gastroschisis.Material and Methods Gastroschisis was created surgically in rat fetuses on gestational day 18, under general anesthesia, and small bowel was harvested on day 22. Intestinal weight-to-length (IW/L) ratio, and small-bowel wall thickness were assessed. Specimens were processed for hematoxylin-eosin staining or immunohistochemistry with specific markers for neuronal cells (Hoxb5), glial cells (GFAP, S100), and ICCs (c-kit). Myenteric plexus maturation was assessed morphologically and compared with sham and control fetuses. Stage of development of the myenteric plexus was graded from 1 (mature) to 3 (very immature) comparatively with specimens from E16 to E22 control fetuses.Results Compared with sham-operated or control fetuses, gastroschisis was associated with increases in mean intestinal weight/intestinal length (IW/L) ratio, and mean thicknesses of the total, muscular, and submucosal layers of the small-bowel wall. The myenteric plexus were present in the small bowel from fetuses with gastroschisis, however all exhibited abnormal myenteric plexus maturation. Thus, of the gastroschisis fetuses, 55% had an aspect similar to the immature myenteric plexus of E19-E20 fetuses and 45% to the very immature mesenteric plexus observed in E16-E18 fetuses. When compared with sham and control groups, ICCs were less abundant in eviscerated small bowel in the gastroschisis group and often exhibited weak c-kit staining or an abnormally round shape without branches. Hoxb5, a marker for enteric neuroblasts and neuronal precursors, was expressed similarly in myenteric plexuses in all groups. S100 or GFAP staining showed the presence of glial cells within the myenteric plexuses in all groups. The S100 expression level was similar in all groups. In contrast, the GFAP expression level was higher in the gastroschisis group than in the sham and control groups.Conclusion Our results suggest that delayed maturation of the enteric nervous system combined with impaired ICC network development may largely explain the intestinal dysmotility seen in neonates with gastroschisis.

Resume Objectifs Le volvulus du grele est une cause rare d’occlusion prenatale. Les objectifs de notre travail etaient d’evaluer le pronostic du volvulus prenatal du grele. Patients et methodes Nous avons mene une etude retrospective sur les volvulus prenatals operes entre mai 2004 et mai 2010. Les volvulus avec ou sans cause identifiable et dont la survenue prenatale ne faisait aucun doute ont ete inclus. Les elements diagnostiques, les donnees neonatales, le bilan lesionnel peroperatoire, la conduite therapeutique et l’evolution ont ete analyses. Resultats Dix cas ont ete identifies. Un diagnostic prenatal de dilatation intestinale ou de peritonite meconiale avait ete porte dans 3 cas sans signe specifique de volvulus. Il s’agissait d’un volvulus primitif dans 5 cas. Pour les 5 autres, il existait une cause : une breche mesenterique isolee, 2 atresies intestinales primitives, un mesentere etroit et une malrotation. Un nouveau-ne avec volvulus total du grele sur malrotation est decede. Les 9 autres enfants ont survecu. Le delai median d’autonomie digestive a ete de 29 j (6–667 j). La majorite des patients enfants avaient un etat nutritionnel normal a 3 et 12 mois. A l’issu d’un suivi median de 16 mois, aucun des enfants vivants n’avait de sequelle digestive ou neurologique. Conclusion Malgre une grande gravite a court terme en cas de volvulus total sur malrotation, nos resultats suggerent que le pronostic a long terme des volvulus prenatals est bon.

Background Ovarian teratoma (OT) is the most common ovarian neoplasm in children. Oophorectomy has been the standard treatment but may impair fertility. The aim of this study was to investigate the feasibility and outcome of ovarian-sparing surgery (OSS) for OT. Procedure We retrospectively studied all children treated for OT at a pediatric teaching hospital in Paris, France, between March 1992 and July 2006. OSS was performed when deemed technically feasible in patients who had no lymphadenopathy by preoperative imaging or surgical exploration, normal tumor marker levels, and calcifications on radiographs. Results We identified 30 patients, including 29 with unilateral OT and 1 with synchronous bilateral OT. Emergent surgery was performed in five patients, among whom four had ovarian torsion requiring oophorectomy and one underwent OSS. Of the 26 OTs in the 25 remaining patients, 10 were managed with OSS and 16 with oophorectomy. Subsequently, ultrasound monitoring detected OT development in the contralateral ovary in 4 (14%) patients, after a median of 3 years (range, 1–14 years); OSS was performed in all four cases. The patient with bilateral synchronous OT, managed by OSS initially, underwent unilateral oophorectomy 3 years later for a recurrence. Overall OSS was performed for 15 (42%) OTs. Conclusions Our results suggest recommendations for preserving fertility whenever possible without compromising the oncological prognosis. In particular, OSS should be reserved for patients who meet all criteria for localized mature teratoma. Long-term follow-up is crucial. Pediatr Blood Cancer 2011; 57: 429–434. © 2011 Wiley-Liss, Inc.

Purpose: Germline hSNF5/INI1 mutations are responsible for hereditary cases of rhabdoid tumors (RT) that constitute the rhabdoid predisposition syndrome (RPS). Our study provides the first precise overview of the prevalence of RPS within a large cohort of RT. Experimental Design: hSNF5/INI1 coding exons were investigated by sequencing and by multiplex ligation-dependent probe amplification. Results: Seventy-four constitutional DNAs from 115 apparently sporadic RT were analyzed from 1999 to 2009. Germline mutations were found in 26 patients (35%). Data from 9 individuals from 5 RPS families (siblings) were also studied. The median age at diagnosis was much lower (6 months) in patients with germline mutation (P < 0.01) than in patients without (18 months). Nevertheless, 7 of 35 patients with germline mutation (20%) developed the disease after 2 years of age. The mutation could be detected in only 1 parent whereas germline blood DNA was wild type in the 20 other parent pairs, therefore indicating the very high proportion of germ-cell mosaicism or of de novo mutations in RPS. The former hypothesis could be clearly documented in 1 case in which prenatal diagnosis was positive in a new pregnancy. Finally, the 2 years' overall survival was 7% in mutated and 29% in wild-type patients, mainly due to the worse outcome of RT in younger patients. Conclusions: Our results show a high proportion of germline mutations in patients with RT that can be found at any age and up to 60% in the youngest patients. Genetic counseling is recommended given the low but actual risk of familial recurrence. Clin Cancer Res; 17(1); 31–8. ©2011 AACR.

Infant small-bowel intussusceptions, most of the time idiopathic, may exceptionally reveal a severe digestive disease. We report the case of a 4-month-old infant who presented multiple and simultaneous ileal intussusceptions associated with severe acute gastroenteritis. Initially, the infant showed a protein-losing enteropathy with a clear alteration of the general state of health and undocumented fever, resistant to broad-spectrum antibiotic therapy. Skin and splenic nodules associated with hepatosplenomegaly and pancytopenia set in progressively. The etiologic evaluation led to the diagnosis of a Mycobacterium bovis BCG infection related to severe combined immune deficiency. The treatment consisted in anti-tuberculosis quadruple therapy in association with immunoglobin supplementation. Secondarily, the patient underwent gene therapy in a clinical trial. An early BCG vaccine in the first weeks of life, before the outbreak of infection revealing the immune deficiency, is a risk factor in triggering a disseminated BCG infection in immunodepressed infants. This clinical case is the first reported of severe combined immune deficiency revealed by small-bowel intussusceptions related to a disseminated BCG infection.

Prune-belly sequence (PBS) usually results from early urethral obstruction. In rare cases, PBS seems to be due to a faulty primary development of the parietal mesenchyme leading to underdevelopment of the abdominal wall musculature, and disorganization of the smooth muscles in the urinary tract. We report on two patients with segmental, unilateral wall musculature deficiency associated with homolateral agenesis of ribs. One patient also had hemivertebrae and the other one ipsilateral diaphragmatic eventration and aplasia cutis. This combination of anomalies may represent a localized deficiency in the development of somitic mesoderm mesenchyme during early embryogenesis.

Résumé Introduction Les traumatismes du testicule sont rares chez l’enfant, et la littérature est très pauvre sur ce sujet. Le but était de les analyser à partir d’une étude multicentrique rétrospective. Matériel et méthodes Un questionnaire sur les traumatismes du testicule survenus entre 2000 et 2009 a été envoyé dans les différents centres de chirurgie pédiatrique français. L’analyse a été effectuée à l’aide du questionnaire et du compte rendu opératoire, et n’ont été retenus que les dossiers de traumatisme vrai du testicule, excluant les plaies simples du scrotum, les traumatismes « révélateurs » d’une torsion de testicule ou d’hydatide, ou encore les dossiers incomplets. Résultats Quinze centres ont bien voulu nous transmettre leurs observations et parmi ceux-ci, deux ont répondu n’avoir opéré aucun cas; cette étude n’est pas exhaustive, car beaucoup d’équipes ont envoyé des dossiers d’enfants opérés et aucun cas de traumatisme bénin. Parmi les 60 observations reçues, 45 ont été retenues. L’âge moyen était de 12,3 ans (2 jours–18 ans). Les circonstances du traumatisme étaient 23 coups (pied, genou, poing), 13 chutes (vélo, banc, luge), quatre accidents de la voie publique, quatre traumatismes de nature inconnue et un traumatisme obstétrical. Le traumatisme était fermé 41 fois et ouvert quatre fois. L’échographie-doppler couleur a été réalisée dans 34 cas sur 45 (75,5 %); un diagnostic exact a été possible 30 fois, alors qu’il y a eu trois faux-positifs (deux fractures du testicule et un hématome intratesticulaire non confirmés à l’intervention) et un faux-négatif (contusion de l’épididyme non vue à l’échographie). Les lésions testiculaires étaient les suivantes: 15 fractures, huit hématomes intratesticulaires, six contusions testiculaires bénignes (dont une associée à une tumeur), six hématomes du cordon, six contusions de l’épididyme, trois hématocèles isolées et une avulsion. Le traitement a été chirurgical 33 fois et non opératoire 12 fois. Parmi les 15 fractures du testicule, 13 ont été opérées (huit sutures et cinq orchidectomies partielles) et deux ont été surveillées avec de bons résultats, sans atrophie secondaire. Pour les hématomes intratesticulaires, trois ont été surveillés et cinq opérés. Seuls 20 enfants ont été suivis au-delà de quatre mois, essentiellement les fractures testiculaires, et aucune atrophie secondaire n’a été constatée. Conclusion Les traumatismes du testicule chez l’enfant sont rares, mais leur pronostic est plutôt bon. L’échographie-doppler permet désormais de faire un diagnostic précis dans un grand nombre de cas, devant permettre d’éviter certaines explorations chirurgicales systématiques; mais l’intervention reste indispensable en cas de doute.

Surgical indications for vesico-ureteral reflux have become more selective and endoscopic treatment has now become part of therapeutic arsenal. However, it is important to know when to operate with uretero-vesical replantation if the ureters are severely dilated, before the acute pyelonephritis becomes recurrent in the setting of persisting reflux in girls or higher grade reflux.

Resume But Etudier l’efficacite et la morbidite de la voie lombaire posterieure (VLP) horizontale dans les nephro-ureterectomies polaires (NUP) pour duplication pyelo-ureterale chez le nourrisson. Materiel et methodes Trente-trois NUP pour duplication pyelo-ureterale ont ete realisees par VLP chez 31nourrissons, âges de 6.7 ± 5.5 mois (1-26) entre Janvier 1994 et Decembre 2005. Resultats La duree operatoire moyenne etait de 121 ± 35 minutes (60-19). Il n’y a pas eu de complication per-operatoire. Les pertes sanguines etaient minimales dans tous les cas ( Conclusion La VLP horizontale a ete une technique sure et reproductible, offrant un benefice esthetique par rapport a la voie classique antero-laterale. Elle peut representer une voie d’abord privilegiee dans la chirurgie ouverte des NUP pour duplication pyelo-ureterale chez le nourrisson, dans une tranche d’âge pour laquelle la videochirurgie reste encore peu evaluee.

RESUME La transplantation renale est le seul traitement de l’insuffisance renale de l’enfant qui permette a ce dernier de retrouver une vie et une croissance normales. En cas d’anomalie du bas appareil urinaire, le risque est de voir recidiver l’insuffisance renale en raison des perturbations urodynamiques. Afin d’isoler ce facteur, les auteurs comparent les resultats obtenus apres greffe renale en cas d’anomalie du bas appareil urinaire a ceux obtenus en cas d’anomalie du haut appareil urinaire, a l’exclusion de toute anomalie parenchymateuse susceptible de recidive. Depuis 1988, cent vingt-sept greffes renales ont ete realisees sur cent dix-huit enfants. Dans dix-sept cas (groupe A), il existait une anomalie du bas appareil : valves de l’uretre posterieur (onze cas), vessie neurologique (quatre cas), exstrophie vesicale (un cas), Prune Belly (un cas), et dans dix-sept cas (groupe B) une anomalie du haut appareil : hypoplasie ou dysplasie (douze cas), jonction pyeloureterale bilaterale (trois cas), dysplasie multikystique bilaterale (deux cas). Dans le groupe A, cinq enfants ont eu une enterocystoplastie, et deux une ureterostomie cutanee. Dans neuf cas la transplantation a ete realisee sans intervention prealable. Les complications chirurgicales et infectieuses ont ete colligees, ainsi que la survie du greffon et du malade. L’analyse statistique a utilise le test de Mann-Whitney. Dans le groupe A, sept enfants ont eu une ou plusieurs complications urologiques : pyelonephrites recidivantes (deux cas), abces renal (deux cas), dilatation du haut appareil urinaire (trois cas), lithiase (un cas), pyelonephrite incrustante a Corynebacterie (un cas). Dans le groupe B, trois complications seulement sont survenues : pyelonephrite aigue (deux cas), epididymite avec prostatite (un cas), mais la difference entre les groupes n’est pas significative (p = 0,246). Dans le groupe A, la survie moyenne du greffon est de 5,29 ans et dans le groupe B de 5,97 ans. (p = 0,76). La comparaison entre les deux groupes A et B ne montre aucune difference significative dans le taux de creatinine a un an (p = 0,77), 5 ans (p = 0,81), et actuelle (p = 0,75). Il est possible de realiser une transplantation renale en cas d’anomalie du bas appareil urinaire chez l’enfant sans compromettre a court et moyen terme la fonction du greffon, la survie du greffon ni du malade.

Resume Le diagnostic le plus frequent de l'anomalie de la jonction pyelo-ureterale chez l'enfant est la decouverte, par echographie antenatale, d'une dilatation pyelocalicielle. La plupart des pyeloplasties par resection-anastomose de la jonction pyelo-ureterale est effectuee chez le nourrisson, pour lequel la voie lombaire posterieure est tres adaptee. Le drainage urinaire intrapyelique ne fait l'unanimite ni sur son indication, bien qu'il semble etre une securite, ni sur son type : nephrostomie simple ou par sonde double J. L'anastomose calico-ureterale est une technique a connaitre pour les rares reprises chirurgicales, en particulier avec dilatation pyelocalicielle majeure. La videochirurgie par lomboscopie pourrait trouver son interet, en particulier chez le grand enfant. L'endopyelotomie offre des resultats interessants en cas de stenose postoperatoire.

Resume Le megaœsophage idiopathique ou achalasie œsophagienne est une affection rare chez l'enfant. Le but de ce travail est d'etudier le profil clinique et l'evolution a long terme de 20 enfants pris en charge pour un megaœsophage a l'hopital Armand-Trousseau depuis 1980. Patients. – Sur les 20 enfants (sept filles dont deux sœurs), âges au moment du diagnostic de huit mois a 18 ans (mediane = 6,4 ans), sept avaient une maladie associee (sequelle d'ictere nucleaire, encephalopathie, trisomie 21, syndrome 3 A ou 4 A, dysautonomie familiale). Le diagnostic, evoque devant des symptomes apparus 1 a 62 mois auparavant (mediane = 8 mois) associant des regurgitations, des infections respiratoires et un amaigrissement, a ete confirme par la radiographie pulmonaire et l'opacification œsophagienne qui etaient anormales chez respectivement 11 et 18 enfants, mais surtout par la manometrie œsophagienne qui montrait de facon constante une dyskinesie du corps de l'œsophage associee a un defaut de relaxation du sphincter inferieur (SIO) lors des deglutitions, et dans la majorite des cas, a une hypertonie du SIO. Evolution. – En premiere intention, neuf enfants ont ete traites par nifedipine (un perdu de vue) et un enfant a ete traite par dilatations au ballonnet avec une efficacite nulle ou transitoire. Une intervention chirurgicale (myotomie extramuqueuse de Heller associee a un montage antireflux) a donc ete effectuee chez 19 enfants. Pendant la duree du suivi (extremes : six mois–21 ans) un enfant est decede d'une hypoglycemie (syndrome 3 A), et des symptomes sont reapparus chez cinq enfants, dont un ayant necessite une reintervention. L'analyse actuarielle a revele un pourcentage d'enfants asymptomatiques de 72 % a un an et de 46 % a cinq et dix ans apres l'intervention. La manometrie postoperatoire effectuee chez neuf enfants a montre la normalisation du tonus du SIO mais la persistance d'un defaut de relaxation du SIO et d'une dyskinesie du corps de l'œsophage, justifiant une surveillance prolongee de ces enfants.

Resume Grâce aux progres de l'echographie fœtale le diagnostic de certaines tumeurs est possible avant la naissance. Nous rapportons cinq cas de neuroblastomes surrenaliens de diagnostic antenatal. Le diagnostic a toujours ete effectue au troisieme trimestre de grossesse. A la naissance aucun retentissement fonctionnel n'a ete observe et les dosages des catecholamines urinaires etaient normaux. L'echographie montrait une tumeur kystique dans un cas, solide dans deux cas et d'echostructure mixte dans les deux derniers. Les cinq enfants ont ete operes sans chimiotherapie pre- ou postoperatoire. Dans chaque cas il s'agissait d'un neuroblastome de stade I selon la classification d'Evans. Tous sont vivants sans recidive avec un recul de 32 mois a 14 ans.

Total nephrectomy (TN) remains the standard treatment of unilateral Wilms tumors (uWT). The SIOP WT-2001 protocol allowed Nephron Sparing Surgery (NSS) for polar or peripherally non-infiltrating tumors.Inventory of the current SIOP NSS-experience.2,800 patients with a unilateral, localized or metastatic and an unequivocal surgical technique recorded were included. All had neo-adjuvant chemotherapy and delayed surgery. In 91 (3%) NSS was performed and in 2709 TN. Data was retrieved from the SIOP WT 2001 database.NSS group contained 65% stage I tumours and the TN group 48%. Tumor volume (at diagnosis and surgery) was significantly smaller in the NSS group. Within stage III, after NSS, 7/12 (58%) had positive margins (M +), 5 with tumor negative lymph nodes (LN-). After TN, 355/712 (55%) had M + , 182 were LN-. Treatment of M+ in the NSS group resulted in two conversions to TN (one combined with radiotherapy), three patients had radiotherapy only and in two patients local therapy, if given, was not recorded. After NSS, four recurrences occurred. For localized disease the 5-year overall (OS) and event free survival (EFS) in NSS group was 100 and 94.8 (95% CI:89.9-99.9), respectively, while OS and EFS in the TN group were 94.4 (95% CI: 93.2-95.5, log-rank test P = 0.06) and 86.5 (95% CI:85.0-88.1, log-rank test P = 0.06), respectively.NSS was only performed in 3% of patients with uWT. Despite excellent survival with few relapses, the gain of nephrons needs to be weighed against the risk to induce stage III with intensified therapy.

Resume Devant une anomalie du contenu scrotal chez un nouveau-ne, le diagnostic de torsion du cordon spermatique est frequent. La vitalite du testicule tordu et son devenir apres exploration chirurgicale ont ete etudies retrospectivement. L'indication d'une intervention en urgence est discutee. Patients et resultats. – L'exploration chirurgicale de 30 nouveau-nes presentant une bourse aigue a retrouve : 18 torsions du cordon spermatique dont une bilaterale, six traumatismes obstetricaux avec hematome intrascrotal, cinq vaginalites, et une ischemie transitoire sans torsion. La torsion etait le plus souvent antenatale (16 fois sur 18) et deux fois postnatale (a deux et 20 jours de vie). Une orchidopexie controlaterale a toujours ete effectuee. Le type anatomique de la torsion etait supravaginal, sauf une fois ou il etait intravaginal. Excepte le cas d'une torsion postnatale a j20, opere dans les quatre heures, tous les testicules tordus ont ete perdus. Conclusion. – Malgre le mauvais pronostic des torsions antenatales, l'intervention est justifiee par la necessite de confirmer le diagnostic et de realiser une fixation controlaterale en raison de formes bilaterales. En cas de torsion postnatale, seule la rapidite de l'exploration chirurgicale permet de sauver le testicule comme cela a deja ete rapporte dans d'autres series.