L’invaginazione intestinale acuta (IIA) è una delle eziologie più frequenti di sindrome occlusiva nei neonati e nei bambini. Questa è una ben nota emergenza addominale nei reparti di chirurgia pediatrica. Le invaginazioni possono essere classificate in due categorie: IIA idiopatiche del neonato che sono le più frequenti (circa il 90%) e IIA secondarie a una causa locale o in un contesto particolare. Il trattamento iniziale, al di là di alcune controindicazioni, deve prevedere una riduzione radiologica: o un clistere pneumatico o idrostatico sotto controllo scopico o, più recentemente, un clistere con soluzione salina sotto controllo ecografico. La riduzione radiologica può essere facilitata dalla sedazione endovenosa. La chirurgia, a cielo aperto o laparoscopica, è riservata ai casi di fallimento o di controindicazioni alla riduzione radiologica. La diagnosi precoce e la gestione da parte di equipe specializzate dovrebbero ridurre la morbilità dell’IIA.

The incidence of ileal atresia ranges from 1/5000 to 1/12000 births. The incidence of Hirschsprung's disease is 1/5000.The combination of Hirschsprung disease and ileal atresia during infancy seems to be a rare event and the exact incidence of the association of the two malformations remains unknown.Several embryo-pathological theories have been offered to explain the correlation of these two malformations. Some of them state that atresia would be a consequence of aganglionnosis and some others the contrary.The urgency of an early detection of this associations is related to her increased morbidity during management, especially if part of the phenotype remains unidentified.To date, less than 25 case of ileal atresia associated with Hirschsprung's disease are reported in Literature.We present two cases managed in our department initially for ileal atresia. Diagnosis of total colonic Hirschsprung's disease associated was placed later, after the appearance of an intestinal occlusion at the time of the restoration of digestive continuity. The first case was derived three times, and the second twice before becoming digestive self-sufficient.The dysfunction of the anastomosis after restoration of continuity is a dangerous complication which underlines the interest of the early diagnosis of the association ileal atresia and Hirschsprung's disease. It is necessary to have the rectal biopsy early, especially when the patient continues to exhibit poor bowel function after corrective surgery.

A Congenital Diaphragmatic Hernia and 46,XX Disorder of Sex Development Caused by a WT1 Pathogenic Variant Daries M1, Moniez S2, Cartault A3, Sartor A4, Aubert-Mucca M5, Mouttalib S6, Mallet D7 and Pienkowski C8* 1Centre de référence des Pathologies Gynécologiques Rares, Unitéd’Endocrinologie Pédiatrique, Hôpital des Enfants, CHU Toulouse, 31059 Toulouse, France 2Centre de référence des Pathologies Gynécologiques Rares, Unitéd’Endocrinologie Pédiatrique, Hôpital des Enfants, CHU Toulouse, 31059 Toulouse, France 3Centre de référence des Pathologies Gynécologiques Rares, Unitéd’Endocrinologie Pédiatrique, Hôpital des Enfants, CHU Toulouse, 31059 Toulouse, France 4Centre de Diagnostic anténatal, Hôpital Paule de Viguier, CHU Toulouse, 31059 Toulouse, France 5Service de Génétique Médicale, Hôpital Purpan, CHU Toulouse, 31059 Toulouse, France 6Service de Chirurgie viscérale pédiatrique, Hôpital des Enfants CHU Toulouse, 31059 Toulouse, France 7Laboratoire de Biochimie et Biologie Moléculaire Grand Est, UM Pathologies Endocriniennes Rénales Musculaires et Mucoviscidose, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France. 8Centre de référence des Pathologies Gynécologiques Rares, Unitéd’Endocrinologie Pédiatrique, Hôpital des Enfants, CHU Toulouse, 31059 Toulouse, France

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 mitochondria(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. (C) 2017 Elsevier Masson SAS. All rights reserved.

La ginecología infantil se dirige sobre todo a las niñas antes de la pubertad o al comienzo de ésta. Es una consulta frecuente en medicina privada y tiene algunas especificidades. El tratamiento se basa en el conocimiento del desarrollo ginecológico de la recién nacida y de la niña, así como del desarrollo puberal clínico y ecográfico. En este artículo se describen desde un punto de vista anatómico las afecciones que implican a la vulva y al clítoris, las anomalías congénitas de la vagina y del útero y las masas ováricas. La observación clínica del desarrollo somático y la exploración ginecológica externa suelen ser los únicos actos esenciales y suficientes en el tratamiento de las niñas. Los métodos de inspección de la vulva deben efectuarse sin exigencias, con el consentimiento de la paciente y en presencia del adulto que la acompaña. Las vulvitis y vulvovaginitis de la niña son, con mucho, los motivos de consulta más frecuentes. Su diagnóstico es clínico y no necesita toma de muestras. Se detallarán las afecciones infecciosas de la vulva para ayudar al médico a adaptar el tratamiento, detectar la presencia de un cuerpo extraño o tratar de manera correcta una infección bacteriana. Algunas afecciones vulvares no infecciosas y las que afectan al clítoris tienen particularidades pediátricas que no deben ignorarse. El estudio por imagen pélvico, representado en primer lugar por la ecografía, es la exploración decisiva en la detección de las anomalías congénitas de la vagina y del útero, así como para el diagnóstico de las masas ováricas. Éstas son infrecuentes, casi siempre benignas, y el síntoma principal es el dolor. El estudio por imagen hace posible la orientación etiológica. En relación con las diferentes etiologías, se describen el tratamiento de urgencia de una torsión de anexos, las características de un quiste ovárico, el tipo tisular de los tumores orgánicos y su orden de frecuencia.

Background Child health is a key priority on the global health agenda, yet the provision of essential and emergency surgery in children is patchy in resource-poor regions. This study was aimed to determine the mortality risk for emergency abdominal paediatric surgery in low-income countries globally. Methods Multicentre, international, prospective, cohort study. Self-selected surgical units performing emergency abdominal surgery submitted prespecified data for consecutive children aged <16 years during a 2-week period between July and December 2014. The United Nation's Human Development Index (HDI) was used to stratify countries. The main outcome measure was 30-day postoperative mortality, analysed by multilevel logistic regression. Results This study included 1409 patients from 253 centres in 43 countries; 282 children were under 2 years of age. Among them, 265 (18.8%) were from low-HDI, 450 (31.9%) from middle-HDI and 694 (49.3%) from high-HDI countries. The most common operations performed were appendectomy, small bowel resection, pyloromyotomy and correction of intussusception. After adjustment for patient and hospital risk factors, child mortality at 30 days was significantly higher in low-HDI (adjusted OR 7.14 (95% CI 2.52 to 20.23), p<0.001) and middle-HDI (4.42 (1.44 to 13.56), p=0.009) countries compared with high-HDI countries, translating to 40 excess deaths per 1000 procedures performed. Conclusions Adjusted mortality in children following emergency abdominal surgery may be as high as 7 times greater in low-HDI and middle-HDI countries compared with high-HDI countries. Effective provision of emergency essential surgery should be a key priority for global child health agendas. Trial registration number NCT02179112; Pre-results.

The authors report on a case of diaphragmatic hernia occurring in a 3-month-old child affected by Marfan syndrome. Diagnosis was made on a chest X-ray and cardiac ultrasounds, performed because of the association of poor general condition, failure to thrive, and signs of respiratory distress. As a reminder, we emphasize the association between Marfan disease and diaphragmatic hernias as well as the diagnostic approach to reach an appropriate diagnosis. (C) 2015 Published by Elsevier Masson SAS.

L’invagination intestinale aiguë (IIA) iléo-colique du nourrisson et de l’enfant est une urgence thérapeutique de très bon pronostic. L’échographie abdominale est l’examen clé permettant d’affirmer son diagnostic. Sa prise en charge au fil des années est devenue de moins en moins chirurgicale. En effet, la réduction de l’IIA est de plus en plus du domaine de la radiologie interventionnelle, le traitement chirurgical (par laparoscopie en première intention ou laparotomie) étant réservé aux échecs des réductions radiologiques et aux formes évoluées ou d’emblée compliquées (pneumopéritoine, péritonite aiguë, altération importante de l’état général avec état de choc). Les équipes de radiologie disposent de 2 techniques de désinvagination : le lavement aux hydrosolubles ou à la baryte sous contrôle scopique ou échographique et le lavement pneumatique sous contrôle scopique. Il est difficile d’affirmer la supériorité d’une technique par rapport à une autre, le choix dépendant des habitudes, de l’expérience et de l’équipement disponible. La prise en charge actuelle de l’invagination doit être le fait d’équipes pédiatriques radiologiques, anesthésiques et chirurgicales spécialisées.

La schistosomose (bilharziose) est une parasitose d’importation assez fréquente, diagnostiquée le plus souvent chez des voyageurs ou migrants. La contamination se fait classiquement en zones tropicales, après exposition à une forme larvaire, la furcocercaire, présente dans certains cours d’eau ou lacs qui hébergent l’hôte intermédiaire, le bulin. En fonction de l’espèce, cette parasitose peut occasionner des atteintes très variées : uro-génitales, coliques ou hépatiques et être responsable de complications sévères après plusieurs années d’évolution : dysurie, insuffisance rénale, stérilité, cirrhose hépatique et hypertension portale. Son diagnostic n’est pas toujours aisé, car moins de 50 % des patients infectés excrètent des œufs dans les selles ou les urines, mais la mise en évidence de ces éléments par examen microscopique permet un diagnostic de certitude. De par leur meilleure sensibilité, les sérodiagnostics ont contribué à améliorer le diagnostic, même si leur interprétation nécessite une bonne expertise. Sur le plan thérapeutique, le praziquantel, lorsqu’il est prescrit précocement, assure une guérison définitive, sans séquelle. L’identification en Corse, à partir de 2014, d’un foyer de transmission de schistosomose uro-génitale avec plus d’une centaine de cas recensés, ne doit plus nous faire percevoir cette parasitose comme un danger lointain mais comme une menace bien réelle en Europe du Sud.Schistosomiasis (bilharziasis) is a common parasitic disease, most often diagnosed in travelers or migrants. The contamination is usually done in tropical areas, after exposure to the larval stage, the furcocercaria, present in some rivers or lakes, which contains the intermediate host, the bulin. This parasite can cause different types of damage in different organs depending on the species involved: uro-genital, colic or liver. More or less quickly, complications may occur such as dysuria, renal impairment, sterility, liver cirrhosis and portal hypertension. The diagnosis is not always easy as fewer than 50 % of infected patients excrete eggs in the stool or urine, but the evidence of these elements by microscopic examination provides proof of diagnosis. Because of their better sensitivity, immunodiagnosis have contributed to improved diagnosis even if their interpretation requires good expertise. Therapeutically, praziquantel, when prescribed early, ensures a definite cure without sequelae. Identification in Corsica, from 2014, of an outbreak of uro-genital schistosomiasis with more than a hundred cases recorded, should no longer make us perceive this parasitosis as a distant danger but as a real threat in Southern Europe.

Digestive complications related to the ingestion of magnetic foreign bodies in children are increasing,. especially in Asia and North America. In France, several case reports have been reported since 2008. We conducted a retrospective multicentric study to evaluate the frequency of ingestion of magnet foreign bodies and to describe the complicated cases that have occurred in France over the last 5 years. We report 40 cases of which 60% were multiple magnet ingestions. Eighty-eight percent of the children of the group who had swallowed multiple magnets needed interventional management by endoscopy (33%) or surgery (58%). Only two children (12.5%) of the group who swallowed one magnet required removal. This problem is not uncommon in France (2% of the 1132 foreign bodies investigated in the Toulouse center over 5 years), which justifies clear information for healthcare professionals and caregivers in order to avoid potential intestinal complications. We suggest interventional management or very close monitoring in the cases of multiple magnet ingestion. Meanwhile, in the majority of confirmed cases of simple magnet ingestions, we propose home monitoring. (C) 2014 Elsevier Masson SAS. All rights reserved.

Background. - Reconstruction of urethral strictures in children remains a challenge to the pediatric surgeon as these are often related to different rare congenital anomalies with various clinical presentations that endanger renal function and should be repaired in young children. Multiple techniques have been described for their repair. We aimed to determine whether the use of a free tubularised bladder mucosal graft associated to a prior tubeless vesicostomy was feasible and sure, as this technique of reconstruction using tubularised grafts has not been described yet in young children.Results. - Two newborn male patients were referred to our department. Both presented a congenital stenosis of the urethra as a part of a complex urethral malformation. Surgery involved prior tubeless vesicostomy, free bladder mucosal graft for urethral reconstruction, and vesicostomy closure for both children. Postoperative evolution was satisfying in both children and cystourethroscopy showed permeable urethra. Satisfying cosmetic and functional results have been obtained in the two cases.Conclusions. - The prior vesicostomy prevents kidney damage in the context of complex genital and urinary malformations. Bladder mucosa's immunohistological properties are the most similar to those of the urethral tissue, and are appropriate for this type of correction, making our technique feasible and sure. (C) 2014 Elsevier Masson SAS. All rights reserved.

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. (C) 2015 Elsevier Masson SAS. All rights reserved.

Les complications digestives secondaires à l’ingestion de corps étrangers (CE) aimantés chez les enfants sont de plus en plus fréquentes, surtout en Asie et en Amérique du Nord. Quelques cas français ont été rapportés depuis 2008. Nous avons réalisé une étude multicentrique rétrospective afin d’évaluer la fréquence de ce type d’ingestions et d’en décrire les complications digestives survenues au cours des 5 dernières années. Quarante cas ont été colligés dont 60 % d’ingestions d’aimants multiples. Quatre-vingt-huit pour cent des enfants ayant avalé plusieurs aimants avaient nécessité une prise en charge interventionnelle endoscopique (33 %) ou chirurgicale (58 %). Seulement 2 des enfants ayant ingéré un seul aimant (12,5 % des cas) avaient justifié une prise en charge interventionnelle. Cette situation n’est donc pas exceptionnelle (2 % des 1132 CE ingérés en cinq ans dans le centre de Toulouse) ; elle justifie une information des professionnels de santé et du public afin d’éviter les complications digestives. Nous proposons une attitude interventionnelle ou une surveillance extrêmement vigilante en cas d’ingestion de plusieurs aimants. Les cas d’ingestion confirmée d’un seul aimant peuvent être surveillés à domicile dans la majorité des cas.

Introduction. - 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.Material and methods. - We conducted a retrospective, monocentric review of all patients managed for urachal remnants from January 2005 to December 2014.Results. - 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.Conclusion. - 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. (C) 2015 Elsevier Masson SAS. All rights reserved.

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.

Nous avons étudié l'impact de l'éloignement géographique et du mode de consultation (CHU, médecin traitant, hôpital périphérique) sur le taux d'orchidectomie en cas de torsion du cordon spermatique (TCS). Les dossiers des patients de moins de 15 ans opérés pour bourse aiguë douloureuse ont été étudiés rétrospectivement (2007–2010). Sur 76 patients d'un âge médian de 12,5 ans [0,18–14,97], 59 avaient une TCS. Parmi ces patients, le délai médian de consultation par rapport au début de la douleur était de 4,3h [0,5- 48] chez les patients consultant d'emblée au CHU (n=32) et de 11h [2–48] pour les autres (n=27)(p=0,6). Le trajet médian était respectivement de 19 [2,5–113], et 44 km [2,5–393](p=0,007). Le taux d'orchidectomie était respectivement de 25 % et 14,8 % (p=0,52). La durée médiane de la douleur était de 12h [1–72] contre 24h [6–48] pour les enfants ayant eu une orchidectomie (p=0,0001). Dans notre série, le taux d'orchidectomie est dépendant de la durée de la douleur, mais pas du mode de consultation ni de l'éloignement géographique. La suspicion de TCS doit conduire à une exploration chirurgicale rapide, qui implique une continuité des soins entre les hôpitaux périphériques et les centres de chirurgie pédiatrique.

Introduction. 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.Methods. 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.Results. 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.Conclusion. 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. (C) 2014 Elsevier Masson SAS. All rights reserved.