Search

El estrechamiento del prepucio (a menudo denominado fimosis) se define como una abertura prepucial estrecha que impide la retraccion manual del prepucio por detras del surco balanoprepucial y que hace que la exposicion del glande sea dificil, si no imposible. Con mucha frecuencia, el prepucio no es retractil durante los primeros anos de vida del nino (estrechamiento fisiologico del prepucio), pero ello se suele corregir espontaneamente durante los primeros 3-4 anos de vida. El estrechamiento patologico o adquirido del prepucio, que tambien se encuentra en el adulto, se define como la imposibilidad de efectuar la retraccion del prepucio debido a lesiones de liquen escleroatrofico (o balanitis xerotica obliterans), a episodios recurrentes de infeccion del prepucio o a maniobras de retraccion forzada del prepucio. Las indicaciones de tratamiento del estrechamiento del prepucio siguen resultando controvertidas actualmente. La mayoria de autores propone la abstencion terapeutica (en espera de una resolucion espontanea, casi siempre antes de la pubertad) para el estrechamiento fisiologico del prepucio y el tratamiento quirurgico para el estrechamiento del prepucio complicado. Sin embargo, las complicaciones (episodios recurrentes de infeccion prepucial, parafimosis e infecciones urinarias) pueden constituir una indicacion para el tratamiento del estrechamiento fisiologico del prepucio en una edad temprana. En el pasado, la unica alternativa para el tratamiento del estrechamiento del prepucio era la circuncision o postectomia y, mas recientemente, la plastia del prepucio. El tratamiento medico con corticoides topicos constituye actualmente una alternativa valida al tratamiento quirurgico, en caso de estrechamiento del prepucio que persiste mas alla de la edad de 4-5 anos. De este modo, el tratamiento quirurgico se reserva para los fracasos del tratamiento medico y como profilaxis de las infecciones urinarias en el nino portador de una enfermedad urologica subyacente. Las complicaciones del tratamiento quirurgico se han estimado entre el 0,1-3,5% e incluyen hemorragia, ulceracion del meato, estenosis del meato, infecciones locales postoperatorias, fistulas uretrales, amputacion parcial o total del pene, efectos secundarios de la anestesia y repercusiones psicosexuales para el nino y sus padres.

To provide an adequate lifelong urological care in the complex period of adolescence, a transition consultation conducted by a paediatric surgeon and an urologist was developed in our institution. As a real rite of passage, it allows the follow-up and the adapted care of urological conditions, sometimes complex, and permits the transition between childhood and the world of grown-ups. We reported our experience at the Children Hospital of our institution (paediatric surgery and urology departments). During a 6 months period (January-July 2015), forty-five young adults with a mean age of 17.8±3.6 years were seen in transition consultation. Eight patients had neurogenic voiding disorders (4 spina bifida, 1 multiple sclerosis, 1 mitochondrial encephalopathy, 1 metachromic leucodystrophy, 1 paraplegia), 9 patients had idiopathic voiding disorders, 1 patient had a non obstructive malformative uropathy; and 30 patients had surgery during infancy and childhood: hypospadias in 17 young men and malformative uropathy in 13 patients. This consultation occurred within 4.6±4.5 years after the last consultation with paediatric surgeon. For 6 patients, the transition consultation was the first for the urological problem. After this consultation, 8 patients stayed in paediatric surgery and 37 patients were referred to adult urologist. Among those 8 patients: 2 patients had cognitive and psychiatric disorders; 4 patients refused to be transferred to adult unit; 2 patients wanted to come back at transition consultation. Among the 37 patients transferred in adult urological care: 6 patients had urological surgery, and one patient was referred to a sexology consultation. The remaining 30 patients have initiated long-term monitoring. All reconvened patients came back at the follow-up visit (at least 12 months follow-up). A 16-year-old patient (spina bifida with polymalformative syndrome) developed a depressive syndrome at the end of the consultation, in the motive of an awareness of the definitive nature of his handicap and the need of medical follow-up throughout his life. Transition consultation makes easier the passage from paediatric care to adult urological care. It allows a smooth change of interlocutors, facilitates subsequent care and improves compliance to medical follow-up. It requires a good collaboration between paediatric and adult care units. Transition responds to an increasing request of adolescents, families, and medical teams, since care rupture during adolescence can have functional and psychological consequences. Level of proof 4.

International audience; We report the natural history and prognosis of tumors after augmentation enterocystoplasty, with a molecular analysis using an oncogene panel to search for potential targeted therapies.Materials and methods: this multicenter, nationwide, retrospective study included 16 patients. A panel of 21 clinically relevant oncogenes was tested on archival tumor specimens using next-generation sequencing. Survival rate was the main clinical outcome and sequences were compared to the reference genome for the genetic outcome.Results: augmentation enterocystoplasties were performed mainly for congenital neurogenic bladder and bladder exstrophy at a median patient age of 17 years (range 4 months to 45 years). Most of the malignancies were diagnosed because of clinical manifestations, with a median latency period of 20 years. Adenocarcinomas were mainly found after gastrocystoplasty, whereas urothelial cell carcinomas were typically found after colocystoplasty. Of the 16 patients 13 were diagnosed at an advanced stage of the disease (positive lymph nodes in 7, distant metastases in 6). The overall 1-year survival rate was 56%. Only 3 patients remained disease-free at a median followup of 70 months. Of the 9 tumors with analyzable DNA 4 were wild-type and 5 harbored missense mutations (KIT-p.Pro573Ser, PDGFRA-p.Glu587Lys, KRAS-p.Gly12Asp, ERBB4p.Arg484Lys, CTNNB1-p.Ser37Phe and p.Ser47Asn).Conclusions: malignancy after augmentation enterocystoplasty is diagnosed late with frequent metastases and a very low 1-year survival rate. More than half the tested samples harbored missense mutations in oncogenes accessible to targeted therapies. An international collaboration to enlarge the genetic panel analysis of these tumors may offer new therapeutic hope to patients.

INTRODUCTION AND OBJECTIVES:The aim of this study is to describe the natural history, the prognosis and the molecular analysis of a panel of cancer genes on a large series of patients with malignan...

This research studied the effects of additional fiber in the rearing phase diets on egg production, gastrointestinal tract (GIT) traits, and body measurements of brown egg-laying hens fed diets varying in energy concentration from 17 to 46 wk of age. The experiment was completely randomized with 10 treatments arranged as a 5 × 2 factorial with 5 rearing phase diets and 2 laying phase diets. During the rearing phase, treatments consisted of a control diet based on cereals and soybean meal and 4 additional diets with a combination of 2 fiber sources (cereal straw and sugar beet pulp, SBP) at 2 levels (2 and 4%). During the laying phase, diets differed in energy content (2,650 vs. 2,750 kcal AMEn/kg) but had the same amino acid content per unit of energy. The rearing diet did not affect any production trait except egg production that was lower in birds fed SBP than in birds fed straw (91.6 and 94.1%, respectively; P < 0.05). Laying hens fed the high energy diet had lower feed intake (P < 0.001), better feed conversion (P < 0.01), and greater BW gain (P < 0.05) than hens fed the low energy diet but egg production and egg weight were not affected. At 46 wk of age, none of the GIT traits was affected by previous dietary treatment. At this age, hen BW was positively related with body length (r = 0.500; P < 0.01), tarsus length (r = 0.758; P < 0.001), and body mass index (r = 0.762; P < 0.001) but no effects of type of diet on these traits were detected. In summary, the inclusion of up to 4% of a fiber source in the rearing diets did not affect GIT development of the hens but SBP reduced egg production. An increase in the energy content of the laying phase diet reduced ADFI and improved feed efficiency but did not affect any of the other traits studied.

Background: Multiple sclerosis (MS) is the most common disabling neurological disorder affecting young adults. Lower urinary tract dysfunction (LUTD) is common during the course of MS and is responsible for an organic impact on the upper urinary tract and for impaired quality of life. Experimental autoimmune encephalomyelitis (EAE), an animal model of MS, is characterized in mice by paralysis of the tail and hind limbs progressing to paraplegia, but urinary parameters and voiding behavior are not well understood. We aimed to study micturition behavior of both healthy and EAE mice to determine changes induced by EAE. Methods: Bladder function study included micturition behavior and filling cystometry. Results: EAE mice developed profound bladder dysfunction characterized by a decrease in urine volume per micturition (138.8 ± 20 vs. 213 ± 17.5 µL, P = 0.018), significantly more frequent postvoid residual urine (30% vs. 61%, P = 0.0496), and increased postvoid residual volume (260 ± 15 vs. 197.5 ± 12 µL, P = 0.045). Cystometric analysis showed significantly more frequent detrusor overactivity (69% vs. 1%, P < 0.0001). Conclusions: Our study, characterizing bladder dysfunction in EAE mice by clinical and cystometric examination, showed that dysfunction was similar to neurological bladder disorders found in human multiple sclerosis and makes this model promising to assess new compounds. World J Nephrol Urol. 2016;5(1):4-10 doi: http://dx.doi.org/10.14740/wjnu245w

Resume But de l’etude Le but de ce travail a ete de determiner la prevalence des variations des veines renales (variations de nombre et de trajet). Patients et methodes Nous avons mene une etude retrospective unicentrique (CHU de Toulouse – laboratoire d’anatomie de la faculte de medecine de Toulouse-Purpan) a partir de scanners abdomino-pelviens avec injection de produit de contraste et en coupes fines (1 a 2 mm) sur une periode de 2 mois. Le groupe etudie comportait 121 patients, âges de 21,7 a 93,4 ans (âge moyen 60,9 ± 15,4 ans). Le sex-ratio etait de 2/1, avec 80 hommes et 41 femmes. Resultats Quatre-vingt-huit patients (73 %) n’avaient pas de variantes des veines renales. En effet, 48 patients, soit de 40 % de l’effectif de l’etude, avaient une artere renale et une veine renale de chaque cote avec un trajet classique, et 40 patients (33 %) avaient une variante de nombre ou de trajet des arteres renales. Nous avons mis en evidence une prevalence de 9,1 % de variation de la veine renale gauche : 5 cas de veine renale gauche retro-aortique (4,1 %) et 6 cas de veine renale gauche circumaortique (5 %). Nous n’avons decele aucun cas de veine renale gauche multiple. Nous avons mis en evidence 25 cas (20,6 %) de veine renale droite multiple (double dans 23 cas, triple dans 2 cas). Trois patients avaient une variante de trajet de la veine renale gauche associee a une veine renale droite double. Le risque d’avoir une variation de la veine renale droite etait statistiquement plus eleve que le risque d’avoir une variation gauche (OR = 2,6, p = 0,01). Nous avons mis en evidence de facon significative un risque plus important d’avoir une variante veineuse dans la population feminine (OR = 2,4, p = 0,04). Nous n’avons pas decele de variante de la veine cave inferieure. Conclusion D’apres notre etude, la prevalence d’une veine renale gauche retro- ou circumaortique (9,1 %) est plus elevee que celle rapportee dans la litterature. Les variantes de trajet et de nombre des vaisseaux renaux ne sont pas si rares et doivent etre connues par les radiologues et les chirurgiens. Leur connaissance a des implications cliniques majeures, en particulier dans le cadre de prelevement d’organe sur donneur vivant.

Resume Nous rapportons le cas d’une nephrectomie partielle polaire superieure gauche avec ureterectomie totale laparoscopique robot assistee dans le cadre d’un systeme double urinaire symptomatique chez une adolescente. Une patiente âgee de 14 ans avait une incontinence urinaire marquee par des fuites a l’effort. Le bilan morphologique (echographie et uro-IRM) montrait un systeme urinaire gauche double avec un pole superieur dysplasique et quasi non fonctionnel, dont le mega-uretere d’implantation ectopique expliquait la symptomatologie. Le premier temps de l’intervention etait la nephrectomie partielle superieure, avec la section du mega-uretere en arriere des vaisseaux polaires superieurs permettant d’emporter l’uretere proximal dans la piece de nephrectomie. Le second temps etait l’ureterectomie, le mega-uretere a ete disseque puis sectionne a la radicelle au contact du parametre uterin gauche. La patiente a ete repositionnee entre les deux temps operatoires : passage du decubitus lateral droit au decubitus dorsal. La voie d’abord trans-peritoneale avait ete choisie car elle offre un grand espace de travail et permet la dissection des ureteres jusque dans leur portion pelvienne par un simple repositionnement des trocarts du robot sans incision supplementaire et sans modification du champ operatoire. Les suites operatoires ont ete simples avec une disparition des troubles urinaires. La chirurgie laparoscopique robot assistee est en cours de developpement et d’evaluation en urologie pediatrique et a notre connaissance, nous rapportons le premier cas en France de nephro-ureterectomie polaire superieure robot-assistee selon cette technique chez un enfant. Dans le cas rapporte, nous montrons que la chirurgie mini-invasive robotique chez l’enfant pour traiter des uropathies malformatives est une technique sure.

Introduction/Background Intradetrusor injections of botulinum toxin type A (IDBTX-A) have become the gold standard in adults for treatment of neurogenic detrusor overactivity refractory to anticholinergics. Data regarding IDBTX-A in pediatric patients with spinal dysraphism are scarce. The purpose of this study was to assess the effectiveness of IDBTX-A in children with spina bifida. Material and method All patients aged under 16 years old who underwent IDBTX-A between 2002 and 2016 at six institutions were included in a retrospective study. Our primary endpoint was the injection's success defined subjectively as both clinical improvement (no incontinence episodes between clean intermittent catheterization (CIC), absence of urgency, less than 8 CIC per day) and urodynamic improvement (resolution of detrusor overactivity, normal bladder compliance for age), lasting ≥ 12 weeks. Predictive factors of success were assessed through univariate analysis. Results Fifty-three patients with a mean age of 8.5 years were included. Thirty-three (62.3%) had repeated injections (from 2 to 8 injections) resulting in a total number of 141 IBTX-A performed. The global success rate of the first injection (clinical and urodynamic) was 30%. Patients with closed spinal dysraphism had a significantly better success rate than patients with myelomeningocele (P = 0.002). The clinical success rate was 66% and was significantly associated with maximum urethral closure pressure (34 cm vs. 54.4 cm H2O; P = 0.02). The urodynamic success rate was 34%. Patients with closed spinal dysraphism had higher success rate vs. patients with myelomeningocele (48.2% vs. 17.4%; P = 0.02). Patients in whom the first IDBTX-A succeeded urodynamically had higher baseline bladder compliance (15.5 ml/cm H2O vs. 7 ml/cm H2O; P = 0.02). Maximum cystometric capacity (P Conclusion Despite IBTX-A enabled clinical improvement in most patients (66%), urodynamic outcomes were poor, resulting in a low global success rate (30%).

Resume Introduction Les valves de l’uretre posterieur (VUP) representent la plus grave des uropathies obstructives meme si leur pronostic a ete notablement ameliore par une prise en charge multidisciplinaire des la periode antenatale. L’objectif de cette etude etait de savoir si les valves de diagnostic tardif dans notre centre avaient une evolution similaire a celles diagnostiquees en antenatal. Patients et methode Nous avons mene une etude retrospective, monocentrique des patients operes pour VUP dans le service de chirurgie pediatrique de l’hopital des enfants de Toulouse entre 1990 et 2010. Resultats Au cours des 20 dernieres annees, 69 patients ont ete operes pour cette pathologie dans notre centre. Trente-huit ont ete diagnostiques lors d’une evaluation prenatale a 30,6 SA et d’une prise en charge neonatale avec une prise en charge au cinquieme jour en moyenne avec un suivi moyen de 7,2 ± 0,5. Trente et un patients etaient diagnostiques secondairement au cours de l’enfance a l’âge de 6,3 ans avec un suivi de 2,3 ± 0,7 annees. Dix alterations de la fonction renale sur 38 patients (26,32 %) ont ete mises en evidence dans le groupe diagnostic antenatal contre deux dans le groupe de diagnostic tardif (6,45 %, p = 0,029) (sans necessite d’avoir recours a une transplantation). Aucune difference entre les deux groupes n’a ete mise en evidence concernant des troubles mictionnels chez les patients de plus de cinq ans ( p = 0,68). Conclusion Contrairement a certaines series de la litterature, les VUP de diagnostic tardif etaient de meilleur pronostic dans notre serie. Nous pensons que cela peut etre attribue au caractere moins obstructif de ces valves. Neanmoins, un suivi plus prolonge s’avere necessaire. Dans tous les cas, un suivi nephrologique et urologique concomitant reste primordial tout au long de la croissance.

To provide an adequate lifelong urological care in the complex period of adolescence, a transition consultation conducted by a paediatric surgeon and an urologist was developed in our institution. As a real rite of passage, it allows the follow-up and the adapted care of urological conditions, sometimes complex, and permits the transition between childhood and the world of grown-ups. We reported our experience at the Children Hospital of our institution (paediatric surgery and urology departments). During a 6 months period (January-July 2015), forty-five young adults with a mean age of 17.8±3.6 years were seen in transition consultation. Eight patients had neurogenic voiding disorders (4 spina bifida, 1 multiple sclerosis, 1 mitochondrial encephalopathy, 1 metachromic leucodystrophy, 1 paraplegia), 9 patients had idiopathic voiding disorders, 1 patient had a non obstructive malformative uropathy; and 30 patients had surgery during infancy and childhood: hypospadias in 17 young men and malformative uropathy in 13 patients. This consultation occurred within 4.6±4.5 years after the last consultation with paediatric surgeon. For 6 patients, the transition consultation was the first for the urological problem. After this consultation, 8 patients stayed in paediatric surgery and 37 patients were referred to adult urologist. Among those 8 patients: 2 patients had cognitive and psychiatric disorders; 4 patients refused to be transferred to adult unit; 2 patients wanted to come back at transition consultation. Among the 37 patients transferred in adult urological care: 6 patients had urological surgery, and one patient was referred to a sexology consultation. The remaining 30 patients have initiated long-term monitoring. All reconvened patients came back at the follow-up visit (at least 12 months follow-up). A 16-year-old patient (spina bifida with polymalformative syndrome) developed a depressive syndrome at the end of the consultation, in the motive of an awareness of the definitive nature of his handicap and the need of medical follow-up throughout his life. Transition consultation makes easier the passage from paediatric care to adult urological care. It allows a smooth change of interlocutors, facilitates subsequent care and improves compliance to medical follow-up. It requires a good collaboration between paediatric and adult care units. Transition responds to an increasing request of adolescents, families, and medical teams, since care rupture during adolescence can have functional and psychological consequences.4.

L’invaginazione intestinale acuta (IIA) e una delle eziologie piu frequenti della sindrome occlusiva nel lattante e nel bambino. E un’urgenza addominale ben conosciuta dai servizi di chirurgia pediatrica. Si possono classificare le invaginazioni in due categorie: le IIA idiopatiche del lattante, che sono le piu frequenti (circa il 90%), e le IIA secondarie a una causa locale o che insorgono in un contesto particolare. Il trattamento iniziale, al di fuori di alcune controindicazioni, deve fare ricorso a una riduzione radiologica: un clisma pneumatico o idrostatico sotto controllo scopico oppure, tecnica piu recente, un clisma con una soluzione salina sotto controllo ecografico. La riduzione radiologica puo essere facilitata da una sedazione endovenosa. La chirurgia, a cielo aperto o per via laparoscopica, e riservata ai casi di insuccesso o di controindicazioni a una riduzione radiologica. Una diagnosi e una gestione precoce da parte di equipe specializzate devono permettere di ridurre la morbilita dell’IIA.

Lactobezoar is a compact mass of undigested milk concretions and mucous secretions in the gastrointestinal tract. It is usually located in the stomach, resulting in various degrees of gastric outlet obstruction. Lactobezoar is the most common type of bezoar in infancy. We report the case of rare and complicated gastric outlet obstruction secondary to lactobezoar. A female infant, 35weeks and 4days' gestation, one of dichorionic, diamniotic twins (birth weight, 1.890kg), was referred to our center at 5days of life for shock and food intolerance. She was on discontinuous oral feedings with a maltodextrin-enriched infant formula. On examination on day 4, there was a tender mass palpable in the left hypochondrium and on day 5, there was abdominal distension with signs of hemodynamic instability and sepsis. Plain abdominal X-ray showed a pneumoperitoneum associated with a heterogeneous mass in a distended stomach, consistent with a bezoar. An emergency laparotomy revealed a gastric perforation secondary to a large lactobezoar, with necrosis of the greater curvature and anterior wall of the stomach. Surgical treatment consisted of extraction of the lactobezoar, partial gastrectomy (resection of necrotic areas), and gastrostomy. Pathological examination confirmed the necrosis of the gastric mucosa. The postoperative course was complicated by prolonged sepsis. The child was kept NPO for 21days. On day 21 postsurgery, an upper gastrointestinal contrast study showed a well-dimensioned stomach, with a good pyloric passage. Gastrostomy and oral feedings were then initiated with good outcome at 6months. Etiopathogenic factors of lactobezoar are prematurity, low birth weight, altered gastric secretions and disturbed gastric emptying, hypercaloric and predominantly casein-based formulas, and inadequate milk composition. Lactobezoar should be considered in infants with symptoms of gastrointestinal obstruction with evocative images. Conservative management with nil per os, parenteral nutrition, and regular saline gastric washes has a good prognosis with rapid resolution of symptoms. Surgical indications are rare, and early and appropriate diagnosis should help limit and reduce the morbidity of lactobezoar.

Resume Le syndrome de Protee est une maladie genetique rare et sporadique qui associe une croissance disproportionnee de multiples tissus, des malformations vasculaires ainsi que des naevus epidermiques. En raison d’une distribution en mosaique, les phenotypes sont variables. Les malformations vasculaires font partie des principaux criteres utilises pour definir et de diagnostiquer ce syndrome. Elles peuvent concerner le tractus gastro-intestinal, la rate ou les voies urinaires, mais les atteintes vesicales sont rares. Nous rapportons ici un cas de malformation vasculaire de la vessie chez un garcon de 12 ans connu pour avoir un syndrome de Protee. Une revue de la litterature sur les malformations de la vessie ou des tumeurs dans ce syndrome a en outre ete realisee.

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

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

Abnormal persistence of the processus vaginalis in children predisposes to a complication that is strangulated inguinal hernia. The inguinal hernia is a frequent pathology because global incidence varies from 0,8 to 4,4% for children of any age and reaches about 30% in premature children. Strangulated inguinal hernia is rare (from 1,5 to 8%) and constitutes a surgical emergency. More frequently, reduction following sedation is possible (from 25 to 33%), and normally leads to delayed surgery. Current practice is to operate early in these children, including preterm infants, before any complication arises. This early surgery requires use of regional anaesthetic techniques such as spinal anaesthesia, ileo-inguinal ileohypogastric nerve blocks and caudal anaesthesia. However, although this pathology may appear to be routine commonplace it requires a specialized surgical and anaesthetic approach and specialized environment.

The purpose of this study was to evaluate safety and tolerability of transcutaneous tibial nerve stimulation (TENS) in patients under 15years of age with refractory overactive bladder.A retrospective analysis was conducted on outcomes of TENS (1daily 20-minute session, 10Hz) in patients with refractory overactive bladder, excluding patients with neurogenic bladder. Treatment efficacy was evaluated on symptomatic improvement and voiding schedule. Healing was defined as following: no recurrence of urinary tract infection, normal urodynamic voiding parameters, no nighttime continence disorder, normal uroflowmetry.Nineteen consecutive patients with refractory overactive bladder were treated from November 2010 to March 2012 (11girls, 8boys, age 12.1±2.7 years). Three patients reported only daytime voiding disorders, the others reported daytime and nighttime voiding disorders. Ten patients reported febrile urinary tract infection (1 boy, 9 girls). The average length of treatment was 6 months. Two patients were lost to follow-up. Thirteen patients had only tibial TENS; 3 patients had tibial TENS and trospium chloride or desmopressin. At 1-month assessment, 16 patients out of 17 (94%) reported symptomatic improvement. At the end of treatment, 12 patients out of 17 (70%) met healing criteria (5 boys, 7girls), without relapse within 9 months. Three boys (18%) had partial improvement (no daytime wetting, but increased daytime frequency). No patient reported side effects.Tibial TENS is a safe, non invasive and effective treatment in refractory overactive bladder in children. The success rate is 70%, with no side effect and no relapse at the end of the treatment in our study.

To evaluate outcomes and long-term sexual quality of life after hypospadias surgery. Seventeen-years-old patients operated for a posterior hypospadias in childhood were included in a transversal study.Fifteen patients, among the forty children treated since 1997, accepted to participate. These young men (mean age at the first surgery was 27.9±20months) were clinically reviewed and responded to questionnaires (EUROQOL 5, IIEF15 and non-validated questionnaire). This study arises about 8.4±5years after the last visit in paediatric department.Mean study age was 21.2±4.7years. One third of patients thought that global quality of life was distorted. Although 33% of the patients had erectile dysfunction, 80% were satisfied with their sexual quality of life. The most important complains were relative to the penile appearance. Number of procedures was not predictive of patient's satisfaction about penile function and appearance. Thirty-three percents of the patients would have been satisfied to have psychological and medical support. They would be interested in having contact with patients who suffered from the same congenital abnormality.These patients had functional and esthetical disturbances. This visit leads to a specific visit in 20% cases. In this study, medical follow-up does not seem to be counselling and had to be adapted. Adequate follow-up transition between paediatric and adult departments especially during adolescence seems to be necessary.

Objectifs Le spina bifida est la premiere cause de neurovessie congenitale. Les injections intra-detrusoriennes de toxine botulique (IDBTX) sont devenues le traitement de reference de l’hyperactivite detrusorienne neurogene resistante aux anticholinergiques chez l’adulte. Cependant, il existe tres peu de donnees sur les resultats des IDBTX chez l’enfant spina bifida. L’objectif de cette etude etait d’evaluer les resultats des IDBTX chez l’enfant spina bifida. Methodes Tous les patients spina bifida ayant eu au moins une IDBTX entre 2002 et 2016 dans 14 centres francophones ont ete inclus dans une etude retrospective. Les patients âges de plus de 16 ans ont ete exclus pour ne garder qu’une population pediatrique. Le critere de jugement principal etait le succes de l’injection defini comme une disparition des urgenturies, de l’incontinence urinaire et de l’hyperactivite detrusorienne et un nombre d’autosondages Resultats Cinquante-trois patients ont ete inclus pour un nombre total de 133 injections (1–8/patient). Les caracteristiques des patients sont resumees dans le Tableau 1 . Le taux de succes global de la premiere injection etait de 62 % avec une resolution de l’incontinence urinaire chez 66 %. Trente-trois patients (62 %) ont recu une deuxieme injection et la duree mediane entre la premiere et la deuxieme injection etait 9 de mois. Le taux de succes etait similaire qu’il y ait ou non un trouble de la compliance (64 % vs. 61 % ; p = 0,85) et qu’il s’agisse d’un dysraphisme ouvert ou ferme (60,9 % vs. 63 % ; p = 0,88). L’âge n’avait pas non plus d’impact sur l’efficacite (OR = 1,4 ; p = 0,76). Il n’y avait pas de facteur predictif du succes. Il y a eu 4 complications (3 %). Conclusion Les IDBTX constituent un traitement efficace chez les enfants spina bifida quel que soit le type de dysraphisme (ouvert/ferme). Contrairement a ce qui a ete observe chez l’adulte, l’efficacite est similaire sur les troubles de la compliance et sur l’hyperactivite detrusorienne. L’âge de debut des injections n’avait pas d’impact sur l’efficacite.

Resume La prise en charge endoscopique du reflux vesico-ureteral (RVU) de l’enfant par injection acide de dextranomer/hyaluronique (Dx/Ha, Deflux®) se complique dans moins de 0,6 % des cas d’une obstruction ureterale necessitant une reprise chirurgicale. Dans la majorite des cas, cela survient lors de la periode postoperatoire immediate. Nous presentons ici le cas d’un garcon de neuf ans ayant une obstruction ureterale distale a plus de deux ans du geste chirurgical initial. C’est le deuxieme cas d’obstruction symptomatique retardee en raison de Dx/Ha rapporte dans la litterature.

The classical management of urachal remants consists in surgical resection, in order to prevent infections and long term malignancies. However, some reports have recently spread a wait and see management. The aim of our study was to report the results of the surgical management in our center.We conducted a retrospective, monocentric review of all patients managed for urachal remnants from January 2005 to December 2014.Thirty-five patients have been operated during the study period (18 girls and 17 boys). Mean age at surgery was 4,9±4,4 years old. Twenty-seven patients were referred due to symptoms whereas 8 were discovered incidentally (4 by ultrasound scan and 4 during laparoscopy). Among them, 10 were urachal cysts, 15 were urachus sinusa and 10 were patent urachus. Thirty were operated using an open approach and 5 using a laparoscopic approach. Mean length of stay was 3,8±1,7days (1-10) with a mean duration of bladder drainage of 2,5±1 days. No major complications occurred. No abnormal tissue was discovered at the histological analysis.Presentation of urachal remnants is variable but surgical outcomes remain excellent in our experience. When symptoms occur, the surgical decision is easy, but when the diagnosis is incidental, the decision is much more complicated. Official guidelines could ease the decision process and the management of urachal anomalies.

Ileocolic intussusception in infants and children requires emergency treatment and has a very good prognosis. Abdominal ultrasonography imaging has a high sensitivity and specificity in its detection. Management of ileocolic intussusceptions now requires fewer surgical procedures because interventional radiology is usually efficient. Surgery (laparoscopy or laparotomy) is reserved for failed radiological reductions and advanced or immediately complicated intussusceptions (pneumoperitoneum, acute peritonitis, shock). Radiology teams have two reduction techniques: hydrostatic barium enema under fluoroscopic or sonographic guidance and air enema under fluoroscopic guidance. The superiority of one radiological reduction technique over the other cannot be asserted, and the choice depends on experience and available equipment. Current management of intussusceptions should be performed by pediatric radiology, anesthesiology and surgery teams.

Ectopic intrathoracic kidney is a rare congenital anomaly, usually asymptomatic. This anomaly is sometimes associated with a diaphragmatic hernia. Few cases of this combination have been described, often in the absence of a prenatal diagnosis. We report on the case of a female newborn infant who was diagnosed with an ectopic intrathoracic right kidney and a diaphragmatic hernia upon 33 weeks of gestation. The patient underwent surgery on the first day of life and the respiratory and renal outcomes were simple. We review the literature and discuss the seemingly good prognosis of this combination.

Choledochal cysts are rare congenital malformations of the biliary tract. Traumatic rupture of a choledochal cyst can be misleading. An 11-year-old boy was admitted for peritonitis and intestinal occlusion after blunt abdominal trauma, evolving over 48 h. Laparotomy revealed bile ascites and a suspected duodenal perforation. After referral to our center, a CT scan showed a perforated choledochal cyst. Six months later, a complete excision of the cyst was successfully performed. This treatment is mandatory because of the risk of further complications such as lithiasis, pancreatitis, cholangitis, biliary cirrhosis, and malignant transformation (cholangiocarcinoma).

Lower pole vessels are a classical cause of PUJO even in children. The "gold standard" in the management of PUJ obstructions remains a dismembered pyeloplasty as described by Anderson and Hynes. However, some authors have developed an alternative procedure to this approach with encouraging results. The aim of our study was to evaluate our preliminary results concerning laparoscopic vascular hitch for crossing vessels.We conducted a retrospective, monocentric study of all patients managed by this technique from January 2010 to December 2012.Eleven patients (7 boys, 4 girls) were managed by laparoscopy at a mean age of 10.7 years (5.4-17). They were referred to our center for clinical symptoms (intermittent pain 7, high blood pressure 1, UTI 1), antenatal diagnosis or accidental discovery. Obstruction was confirmed by MAG3 nephrogram and the presence of obstructive vessels by tomodensitometry or MRI. Mean operative time was 90.2minutes (48-184). Seven patients over 11 were strictly managed by laparoscopic transposition of lower pole vessels. Four required a classical video-assisted dismembered pyeloplasty due to a potential intraluminal stenosis. The latter were suspected by a distension test with furosemid in all four cases. Mean follow-up was 12.9±3 months. Nine patients over 11 were totally non symptomatic, whereas 2 still present mild intermittent pain. In all cases, ultrasound scans show an improvement of the pelvic dilatation.Laparoscopic transposition of lower pole vessels is a suitable and feasible alternative for the management of obstructive PUJ. Our preliminary experience emphasizes the need for a precise preoperative selection of patients along with a per operative evaluation of the obstruction. Further experience seems required to improve our criteria in this indication.5.

We report the case of a laparoscopic robot assisted left upper polar partial nephrectomy with total ureterectomy performed in a teenager. A 14 year-old girl was referred to our institution for stress urinary incontinence. The morphological assessment (ultrasound scan and uro-MRI) showed a double collecting system with a complete ureteral duplication complicated by a dysplasia of the upper moiety of the duplex left kidney and a mega ureter. The surgery started on a lateral decubitus position by the upper polar partial nephrectomy and the ureter section behind superior polar renal vessels. The patient was placed in a supine position and the mega ureter was released and sectioned at the level of the distal adynamic segment in the left uterine parameter. The transperitoneal route was chosen as it provides a large workspace and allows the dissection of the ureters into their pelvic portion by a simple repositioning of the robot ports without additional incision and without any modification of the operative field. No intraoperative and postoperative complication was noticed. Laparoscopic robotic assisted surgery in pediatric urology is increasing, and to our knowledge, we reported this technique and surgery for the first time in France and in children. In the reported case, we showed that the robotic minimally invasive surgery in children is an innovative and safe technique for the treatment of symptomatic upper urinary tract malformations.

Intussusceptions in infants and children are a medical and surgical emergency. A seasonal pattern, with fall and winter predominance, is usually taught in medical schools, but in France the epidemiological characteristics of intussusceptions are not clearly described.We conducted a retrospective study concerning children with idiopathic ileocolic intussusceptions admitted and treated at our institution (Emergency Department and Pediatric Surgery Department, Toulouse University Hospital) between January 2002 and December 2011. The main purpose was to evaluate the seasonality of intussusceptions. We describe the sex ratio, age of occurrence, rate of recurrence, and rate of failed enema reduction.A total of 306 idiopathic ileocolic intussusceptions (280 patients) were included over a 10-year period with an average of 31 cases per year [21-42]. No seasonal pattern of intussusceptions or fall/winter predominance was shown (P=0.6) in the cumulative number of monthly cases: 24% of the intussusceptions occurred during fall, 21% during winter, 29% during spring, and 27% during summer. There were two peaks: April (35 cases over 10 years) and June (34 cases). The mean age was 18.7 months [2.3-159.4]: 19.5 months [3-159.4] in boys and 14.8 months [2.3-77.5] for girls (P=0.02). Two peaks were shown: one between six and nine months, the other one between 18 and 21 months. Male children had a higher incidence than female children: the sex ratio was 2:1, with 65.4% boys and 34.6% girls (P=0.0003), with an increasing sex ratio for older children. The recurrence rate was 8.2% among all (26 recurrences): 10.1% in boys and 4.9% in girls (P=0.08). The rate of surgery after failed enema reduction was 7.5% (21/280 children). There was no case of recurrence after operation.These results were consistent with previous reports. We did not show any seasonal pattern of idiopathic ileocolic intussusceptions in our patients. A new survey of childhood intussusceptions would be relevant to confirm these results and would make it possible to modify academic teachings about seasonality in intussusceptions.

Introduction Nitric oxide synthases (NOSs) and estrogen receptors are expressed in the vagina. Aim We aimed to assess the impact of sildenafil on vaginal lubrication according to the hormonal status and to determine the role of the neuronal isoform of NOS (nNOS). Methods Fourweekold C57/BL6 female mice were sham operated or ovariectomized. At 10 weeks of age, they were injected intraperitoneally by any combination of sildenafil, 7nitroindazole (7NI)—a potent selective nNOS inhibitor—or the corresponding vehicles. Vaginal lubrication was induced in a physiological manner by cervical vaginal probing and quantified depending on the hormonal and pharmacological conditions. The animals were then sacrificed for vaginal histomorphometry. Main Outcome Measures The main outcome measure is the quantification of vaginal transudate after cervicovaginal stimulation and vaginal histomorphometry. Results Sildenafil increased cervicovaginal probinginduced vaginal lubrication in ovariectomized and shamoperated animals. Ovariectomized mice exhibited decreased vaginal lubrication as compared with shamoperated mice. When taking into account the presence of severe vaginal atrophy, a threefold increase in transudate per gram of vagina wet weight was revealed in ovariectomized animals. Castration markedly reduced the thickness of the vaginal wall. nNOS inhibition by 7NI had no impact on vaginal lubrication. Conclusions Irrespective of the hormonal status, sildenafil increased vaginal lubrication. The vaginal effect of sildenafil was independent of the nNOS pathway and more pronounced in ovariectomized animals.

The objective of this study was to evaluate the clinical outcome of primary megaureters and to evaluate predictive factor for surgery need based on ultrasound values.A total of 43 primary obstructive megaureters detected in 41 patients have been evaluated between January 2000 and may 2010.Mostly boys (73%) were concerned by megaureters, mainly on the left side (66%). Twenty patients were prenatally diagnosed. Surgical indications were: pyonephrosis (3), recurrent pyelonephritis (14), scintigraphic damage (3), ureteric diameter aggravation (7), disease occurring on unique kidney (2). Regarding the 30 patients who benefit surgery, the retrovesical ureter measured, before surgery 19.15 mm (± 7.17) on average and 3.18 mm, 44 months later. Over the 10 patients treated medically, the initial diameter was 9.91 mm and at the end of the study, five patients had megaureter completely regressed, three patients had a diameter greater than 10mm at the end of the study and two faced a worsening evolution with sudden and complete renal damage on scintigraphic nephrogram, after recurrent pyelonephritis despite antibioprophylaxis.We recommend a careful watch-fulling of primary megaureters; mainly for those with recurrent infections and whenever the retrovesical ureter diameter exceeds 14 mm at first ultrasound.

Acute scrotal pain is a true surgical emergency as patients presenting with acute scrotal pain may suffer from spermatic cord torsion and gonadal loss. We assessed whether the type of consultation (first consultation in our center or secondary transfer from a peripheral hospital or primary care practice), distance from home to hospital, and duration of pain had an impact on the orchiectomy rate.We retrospectively reviewed the medical records of all patients under 15years of age suffering from acute scrotal pain who had surgical exploration between January 2007 and January 2010 in our center. Patient demographics, transfer status, time to consultation in our center, time to surgery, operative findings and clinical outcome were reviewed.Of the 76 patients with acute scrotal pain in whom surgical exploration was performed, 59 had acute spermatic cord torsion, 16 had torsion of the testicular appendage, and 1 had orchitis. In patients with acute spermatic cord torsion, the median age was 13 years (range: 0.18-14.97). In patients with acute spermatic cord torsion, 32 came straight to our center (direct admission group, 54.2%), and 27 (45.8%) came after a prior consultation out of side the center (transfer group). The median journey was 19km (range: 2.5-113) in the direct admission group and 44km (range: 2.5-393) in the transfer group (P=0.0072). The median time between pain onset and consultation at our center was 4.3h (range: 0.5-48) in the direct admission group, and 11h (range: 2-48) in the transfer group (P=0.6139). The median time between admission at our center and surgery was 2.5h, with no difference between the 2 groups (P=0.8789). The orchiectomy rate was 25% in the direct admission group and 14.8% in the transfer group (P=0.5177). In children who underwent orchiectomy, the duration of pain was consistently over 6h. The duration of pain was greater in patients with orchiectomy (12h [range: 1-72]) than in patients without orchiectomy (12h [range: 6-48]; P=0.0001).In this study, the orchiectomy rate depended on the duration of pain but not on transfer status. Acute scrotal pain must lead to surgical exploration as soon as possible, requiring close collaboration between peripheral hospitals, primary care physicians, and referral centers.

Posterior urethral valves (PUV) diagnosed during childhood have classically been associated with a better outcome than antenatally diagnosed PUV. The aim of our study was to compare long-term outcome of these two patients' groups.We retrospectively reviewed the medical records of boys with PUV managed between 1990 and 2010. Patient demographics, clinical background, radiographic data (including prenatal ultrasonography data when available), renal and bladder functional outcomes, surgical procedures and urinary tract infections (UTI) were abstracted. Impaired renal function (IRF) was defined as glomerular filtration rate less than 90 mL/min/1.73 m(2) at last follow-up.We identified 69 patients with confirmed PUV. Thirty-eight were diagnosed prenatally (group 1) at 30.5 weeks of gestation and 31 had a delayed diagnosis (group 2) at a median age of 6.31 years. At diagnosis, 20 patients in group 1 had renal insufficiency versus two in group 2 (P0.05). At the end of mean follow-up of 7.2 ± 0.5 years, in group 1, 26.3% developed IRF versus 6.3% in group 2 (mean follow-up 2.3 years). Mean age at last follow-up was 7.3 years in group 1 versus 8.3 in group 2 (P0.05). In group 1, 27% had voiding dysfunction versus 30% in group 2 (NS). In group 1, 35% had UTI during follow-up versus 10% (P=0.01).During the follow-up, the patients with delayed diagnosis VUP have developed fewer complications related to the initial obstruction than the population who was detected antenatally and managed from the early hours of life. However, the rate of IRF and voiding disorders in our study, associated with the data of the literature, highlights the potential persistence and worsening of these conditions. That is why, whatever the age at diagnosis, VUP patients require a close monitoring.

To determine the prevalence of renal vein variants. To investigate the distribution of renal veins.We retrospectively reviewed spiral computed tomography (CT) scans of the abdomen performed during a two-month period. The same protocol was used for all CT scans: same multidetector-row CT scanner (Siemens(®)), 1 to 2-mm section thickness, injection of intravenous iomeprol. The study group included 121 patients, aged 21.7 to 93.4 years (mean age 60.9 ± 15.4 years). The sex ratio was 2/1, with 80 men and 41 women.Seventy-three percent of the study group (88 patients) had no variants of the renal veins. Indeed almost 40% (48 patients) had one artery and one vein on each side, with typical course, and 33% (40 patients) had course and/or number variants of the renal arteries. Variants of the right renal vein consisted in multiple veins in 20.6% (25 cases). We detected no case of multiple left renal veins, but we described variations of its course in 9.1% (11 cases): 5 cases of retroaortic left renal vein (4.1%) and 6 cases of circumaortic left renal vein (5%). Three of these 11 patients had an associated double right renal vein. The probability to have a right renal vein variant was significantly higher than a left one (OR = 2.6, P = 0.01). And we found a significantly higher risk of having a venous variant in women (OR = 2.4, P = 0.04). We detected no case of inferior vena cava variant.In our study, prevalence of a circum- or retroaortic left renal vein appeared higher than previously reported in the literature (9.1%). Knowledge of anatomical variants of renal vasculature is crucial and this study puts the emphasis on variations of course and number of renal vessels. Those variations are not so uncommon and should be known by radiologists and also by surgeons. Their knowledge has major clinical implications in practice and it contributes to the safety of renal and retroperitoneal surgery.

Lactobezoar is a compact mass of undigested milk concretions and mucous secretions in the gastrointestinal tract. It is usually located in the stomach, resulting in various degrees of gastric outlet obstruction. Lactobezoar is the most common type of bezoar in infancy. We report the case of rare and complicated gastric outlet obstruction secondary to lactobezoar. A female infant, 35weeks and 4days' gestation, one of dichorionic, diamniotic twins (birth weight, 1.890kg), was referred to our center at 5days of life for shock and food intolerance. She was on discontinuous oral feedings with a maltodextrin-enriched infant formula. On examination on day 4, there was a tender mass palpable in the left hypochondrium and on day 5, there was abdominal distension with signs of hemodynamic instability and sepsis. Plain abdominal X-ray showed a pneumoperitoneum associated with a heterogeneous mass in a distended stomach, consistent with a bezoar. An emergency laparotomy revealed a gastric perforation secondary to a large lactobezoar, with necrosis of the greater curvature and anterior wall of the stomach. Surgical treatment consisted of extraction of the lactobezoar, partial gastrectomy (resection of necrotic areas), and gastrostomy. Pathological examination confirmed the necrosis of the gastric mucosa. The postoperative course was complicated by prolonged sepsis. The child was kept NPO for 21days. On day 21 postsurgery, an upper gastrointestinal contrast study showed a well-dimensioned stomach, with a good pyloric passage. Gastrostomy and oral feedings were then initiated with good outcome at 6months. Etiopathogenic factors of lactobezoar are prematurity, low birth weight, altered gastric secretions and disturbed gastric emptying, hypercaloric and predominantly casein-based formulas, and inadequate milk composition. Lactobezoar should be considered in infants with symptoms of gastrointestinal obstruction with evocative images. Conservative management with nil per os, parenteral nutrition, and regular saline gastric washes has a good prognosis with rapid resolution of symptoms. Surgical indications are rare, and early and appropriate diagnosis should help limit and reduce the morbidity of lactobezoar.

Introduction Nitric oxide synthases (NOSs) and estrogen receptors are expressed in the female urethra. Aim We aimed to assess the impact of sildenafil on micturition behavior, urethral tone according to the hormonal status and to determine the implications of the neuronal isoform of NOS (nNOS). Methods Four‐week‐old C57/BL6 female mice were sham‐operated or ovariectomized. Six weeks later, they were injected intraperitoneally by any combination of sildenafil, 7‐nitroindazole (7‐NI)—a potent selective nNOS inhibitor—or the corresponding vehicles. The mice were then subjected to micturition behavior and leak point pressure studies. Urethral histomorphometry was performed. Main Outcome Measures The main outcome measures were micturition behavior, leak point pressure, and histomorphometry. Results In sham‐operated and ovariectomized animals, sildenafil did not impact micturition, although it decreased urethral resistance 10‐fold. nNOS inhibition by 7‐NI reduced the number of micturitions and increased residual volume and leak point pressure. It abrogated sildenafil‐induced drop in urethral resistances. Hormonal status did not influence the structure of the urethral layers. Conclusions. Irrespective of the hormonal status, sildenafil decreased leak point pressure by a nNOS‐mediated mechanism. Game X, Bouali O, Allard J, Gourdy P, Escourrou G, Tack I, Rischmann P, Arnal J‐F, and Malavaud B. Influence of sildenafil on micturition and urethral tone in ovariectomized and non‐ovariectomized mice. J Sex Med 2012;9:466–471.

Surgically relevant obstruction after dextranomer/hyaluronic acid injection (Dx/Ha, Deflux(®)) for the treatment of vesicoureteral reflux (VUR) is rare with a 0.6% incidence. It occurs usually during the early postoperative period. We report here the case of a 9-year-old boy with a history of VUR who was previously treated with Deflux(®) and was referred more than 2 years later with acute flank pain (as he already did 2 weeks after surgery with a spontaneous relief under medical treatment). Initial radiological investigations showed hydronephrosis caused by distal ureteral obstruction which required open surgery removal of the Dx/Ha and Cohen procedure. This is the second case of delayed symptomatic obstruction due to Dx/Ha reported in the literature.

Proteus syndrome is a rare, sporadic disorder consisting of disproportionate overgrowth of multiple tissues, vascular malformations, and connective tissue or epidermal nevi. Due to mosaic pattern of distribution, the phenotypes are variable and diverse. Vascular malformations are part of the major criteria used to define and diagnose this syndrome. It can involve the gastrointestinal tract, spleen, or the urinary tract but bladder malformations are rare. We report here a case of bladder vascular malformation in a 12-year-old boy known to have Proteus syndrome and review the literature on bladder malformations or tumors in this syndrome.

The accidental discovery of a retrotracheal left pulmonary artery in a 4-month-old infant encouraged us to review the various embryologic theories concerning this very rare anomaly and perform an anatomic update in order to better define surgical treatment. Nathan underwent surgery for a bilateral inguinal hernia at the age of 4 months. The postoperative period was marked by malaise associated with dyspnoea, stridor, tachycardia and sweating. A X-ray of the thorax, oesophageal transit and angio scan presented an intertracheo-oesophageal left pulmonary artery and a reimplantation of the left pulmonary artery was successfully performed. A retrotracheal left pulmonary artery is a very rare malformation. From development of pulmonary vascularisation, three embryologic theories have been advanced to explain this anomaly. From an anatomic point of view, Landing et al. proposed in 1982 a classification system of retrotracheal left pulmonary artery. Today, current radiological techniques not only provide a precise diagnosis but also make it possible to define appropriate care for the different types of this malformation.

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

Arteriovenous malformations are seldom in children but raise important therapeutic problems. Apart from intracranial arteriovenous malformations, few observations have been described in the literature. We report the case of a superficial perineal arteriovenous malformation in a 5-year-old child. Tailored embolization followed shortly by thorough surgery is the best attitude. Surgery must totally eradicate the lesion to allow hope for a cure. Surgery should be reserved for forms that are extensive or a source of complications. Even total resection does not ensure non-recurrence.

Abnormal persistence of the processus vaginalis in children predisposes to a complication that is strangulated inguinal hernia. The inguinal hernia is a frequent pathology because global incidence varies from 0,8 to 4,4% for children of any age and reaches about 30% in premature children. Strangulated inguinal hernia is rare (from 1,5 to 8%) and constitutes a surgical emergency. More frequently, reduction following sedation is possible (from 25 to 33%), and normally leads to delayed surgery. Current practice is to operate early in these children, including preterm infants, before any complication arises. This early surgery requires use of regional anaesthetic techniques such as spinal anaesthesia, ileo-inguinal ileohypogastric nerve blocks and caudal anaesthesia. However, although this pathology may appear to be routine commonplace it requires a specialized surgical and anaesthetic approach and specialized environment.

induite (grade 3—4 vs 0—2) etait significativement correlee a la SG (respectivement 34 vs 10mois p < 0,0001) et la SSP (respectivement 10 vs 4mois, p < 0,0001). Par ailleurs, une insuffisance renale (clairance < 60mL/min) a l’initiation du traitement etait associee a une meilleure SG et SSP (respectivement 35 vs 11mois, p = 0,0002 pour la SG et 12 vs 5mois, p = 0,0002 pour la SSP). En analyse multivariee, la toxicite induite (p = 0,01) et le score MSKCC (p = 0,02) ont ete identifies comme des facteurs independants de SG. Conclusion.— Le score MSKCC et la toxicite induite apparaissaient comme des facteurs independants de survie en premiere ligne de TC du CRM. Par ailleurs, nos resultats suggerent qu’une insuffisance renale aurait potentiellement un impact significatif sur la survie.