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International audience; Purpose: The purpose of this study was to assess the performance of magnetic resonance imaging (MRI) in children and adolescents with suspected adnexal torsion (AT) after inconclusive initial ultrasound examination.Materials and methods: Twenty-eight girls with a mean age of 12±4 (SD) years (range: 1 month to 18years) were included. All had clinically suspected AT and inconclusive initial ultrasound findings followed by pelvic MRI as a second-line imaging modality. The final diagnosis was obtained by surgery or follow-up. Two radiologists blinded to the clinical, ultrasound and surgical data, retrospectively and independently reviewed MRI examinations. Clinical and MRI features associated with AT were searched for using univariate analyses.Result: Among the 28 patients, 10/28 patients (36%) had AT and 22/28 (79%) had an ovarian or tubal mass. AT was associated with an age

Background Health workers exposed to ionizing radiation (IR) account for +50% of workers exposed to artificial IR in France. Over the last 10 years, the use of IR in medicine has developed due to the introduction of new practices. The EXPERTS study aims to evaluate and characterize the trends in IR exposure of health workers in France between 2009 and 2019. Methods The study includes all health workers with at least one dosimetric record in the System for occupational dosimetry registration (SISERI) database for each of the years 2009, 2014, and 2019, in the 6 hospitals included in the study (in Paris & Bordeaux). Individual passive external doses, professional activity, age and gender were collected via SISERI and occupational medicine units. Doses were estimated from badges worn at chest level under the lead apron. Results 1,999 workers were included. Mean Hp(10) doses significantly decreased between 2009 and 2019 (-0.004 mSv/year; p < 0.05). Workers in Bordeaux hospitals were more exposed than those in Paris in 2009 and 2014, while trend was reversed in 2019 (p > 0.05). Doses in men were significantly higher than women's doses in 2009 and 2014 (p < 0.02), but were similar in 2019 (p = 0.99). Physicians had the highest mean IR doses between 2009 and 2019 (0.14mSv ±1.09), and more specifically surgeons and cardiologists, but their exposure tended to decrease in recent years. Radiological technologists have similar mean doses (0.14mSv ±0.38), relatively stable over the study period - the most exposed in the nuclear medicine unit (0.60mSv ±0.58). Conclusions IR exposure of health workers in France decreased significantly between 2009 and 2019, despite an increase in practices using IR - this decrease was partly recorded in orthopaedic surgeons. This may be related to radiation protection rules' application. However, badges may not always be worn properly. This study could be extended to other healthcare centres to study local specificities and to adapt radiation protection policies Key messages Despite the increase in medical practices using ionising radiations (IR), the average exposure of health workers to IR has decreased over the 10 last year, mainly in Bordeaux hospitals. Differences in average IR exposure were found by occupation, gender, health care units, and hospitals. Policies should use these results to promote radiation protection rules.

Resume Introduction Cet article a pour but de donner les elements essentiels que tout praticien doit connaitre pour repondre aux interrogations legitimes d’une femme enceinte exposee aux rayonnements ionisants. Messages principaux Il comporte une mise au point des connaissances actuelles sur les effets deterministes (deces prenatal, malformation, retard mental) et stochastiques (cancers et leucemies) des rayonnements ionisants sur l’embryon et le fœtus. Les effets dependent du niveau d’exposition et du terme de la grossesse auquel elle se produit. Les niveaux d’exposition aux rayonnements ionisants lies a la realisation des principaux examens d’imagerie sont rappeles. L’attitude pratique a adopter devant l’exposition accidentelle d’une femme enceinte qui ignorait son etat est expliquee en se fondant sur les positions consensuelles des organismes internationaux. La protection legale de la femme enceinte travaillant sous les rayonnements ionisants est egalement exposee. Conclusion Dans la tres grande majorite des cas, il est heureusement possible de rassurer la patiente tout en l’informant.

Resume En pediatrie, l’indication la plus frequente de la scintigraphie osseuse est la suspicion d’infection osteoarticulaire. Cette pathologie, particulierement frequente avant 5 ans, est parfois de diagnostic difficile. Le radiopharmaceutique de reference dans cette population est un diphosphonate marque par le Technetium-99m. Les avantages de l’examen sont une sensibilite elevee, l’absence de sedation, et la possibilite de visualiser l’ensemble du squelette. En cas de suspicion de spondylodiscite, l’imagerie par resonance magnetique (IRM) est l’examen de premiere intention.

International audience; Objective: To investigate cerebral anomalies other than Chiari type 2 malformation in fetuses with myelomeningocele (MMC).Design: A retrospective cohort study in a single tertiary centre.Setting: A review of associated cerebral anomalies in cases with prenatal diagnosis of myelomeningocele.Population: Seventy cases of fetal myelomeningocele.Methods: Ultrasound and MRI images were blindly reviewed. Postnatal imaging and results of the postmortem results were also reviewed. The association between cerebral anomalies and the following ultrasound findings was measured: level of the defect, ventriculomegaly, microcephaly and fetal talipes.Main outcome measures: A microcephaly was observed in 32/70 cases (46%) and a ventriculomegaly was observed in 39/70 cases (56%). Other cerebral anomalies were diagnosed in 47/70 (67%).Results: Other cerebral anomalies were represented by 42/70 cases with abnormal CC (60%), 8/70 cases with perinodular heterotopia (PNH; 11%), 2/70 cases with abnormal gyration (3%). MRI performed only in fetal surgery cases confirmed the ulltrasound findings in all cases and provided additional findings in two cases (PNH). Risk ratios of fetal cerebral anomalies associated with MMC did not reach significance for microcephaly, ventriculomegaly, talipes or the level of the defect There was an overall good correlation between pre- and postnatal findings with a Kappa value of 0.79 [95% CI 0.57-1] and 82% agreement.Conclusion: Fetal brain anomalies other than Chiari type 2 malformation are frequently observed in fetuses with myelomeningocele, predominantly represented by CC anomalies. Whether these associated cerebral anomalies have an impact on selecting cases eligible for fetal surgery needs further evaluation.Tweetable abstract: Fetal cerebral anomalies other than Chiari type 2 malformation, microcephaly, and ventriculomegaly may be associated with MMC in up to 67% of the cases.

Resume Le nombre de luxations congenitales de hanches decouvertes a l’âge de la marche est en augmentation. L’echographie de hanche est l’examen cle du depistage de la luxation de hanche apres l’examen clinique. Dans cette fiche technique, nous rappelons les indications et les bonnes pratiques pour le depistage, mais egalement pour le diagnostic echographique de la luxation congenitale de hanche.

Resume Le diverticule de Meckel est une malformation frequente chez l’enfant, le plus souvent asymptomatique, se compliquant dans 20 % des cas. De facon exceptionnelle, il peut etre le siege de developpement d’une tumeur, notamment un lymphome de Burkitt chez l’enfant. Plus rarement, comme chez l’adulte, des tumeurs stromales gastro-intestinales, des leiomyosarcomes ou des tumeurs neuro-endocrines de type carcinoide peuvent etre observees. Le diagnostic de pseudo-tumeur inflammatoire secondaire a une perforation est rare. Nous rapportons le cas d’une pseudo-tumeur inflammatoire developpee sur la pointe d’un diverticule de Meckel avec une heterotopie gastrique chez un nourrisson de 14 mois. Cette lesion avait fait evoquer une tumeur maligne. L’evolution a ete favorable apres exerese chirurgicale. La perforation d’un diverticule de Meckel secondaire a une ulceration sur heterotopie gastrique est un mode de revelation frequent. La decouverte secondaire d’une pseudo-tumeur inflammatoire reactionnelle a cette perforation doit faire avant tout discuter les diagnostics differentiels de tumeurs malignes.

Objectives To identify at prenatal ultrasound (US) the features of apparently isolated subependymal pseudocysts (SEPC) that may indicate underlying pathology and should lead to further investigations. Methods This was a retrospective study of cases with SEPC detected on prenatal US and/or magnetic resonance imaging (MRI). Those with apparently isolated SEPC at US were classified into two groups as follows: Group 1 (n = 29): normal prenatal US and MRI (except for SEPC) and normal outcome; Group 2 (n = 12): normal prenatal cerebral US (except for SEPC) and abnormal prenatal cerebral MRI with or without abnormal outcome. A third group (n = 9) included cases with abnormal prenatal US and MRI. The latter cases with obvious cerebral abnormalities at US were excluded from the statistical analysis as they do not represent a diagnostic dilemma for clinicians. Groups 1 and 2 were analyzed, comparing them with respect to their SEPC characteristics (size, number, location in relation to the caudothalamic notch and the ventricular horns and morphology) and extracerebral abnormalities. Results The mean ± SD SEPC great axis was longer in Group 2 (11.67 ± 5.82 mm) than it was in Group 1 (8.00 ± 5.64 mm) (P = 0.021), suggesting an optimal cut-off for size of SEPC of ≥ 9 mm (sensitivity = 75%, specificity = 62%) to maximize sensitivity for predicting pathological outcome. SEPC adjacent to the temporal horns and SEPC located posterior to the caudothalamic notch were observed more frequently in Group 2, indicating their association with poor outcome (P = 0.003 and P = 0.003, respectively). Atypical morphology and extracerebral abnormalities were observed more frequently in Group 2 (P = 0.013 and P = 0.044, respectively). There was no statistically significant difference between groups for either number or location of cysts along the inferior wall or adjacent to the lateral wall of the frontal horns (P = 0.591 and P = 0.156, respectively). Conclusion When apparently isolated SEPC are observed at prenatal US, further investigations should be performed under the following circumstances: (1) SEPC great axis ≥ 9 mm; (2) SEPC adjacent to the occipital and temporal horns; (3) SEPC located posterior to the caudothalamic notch; (4) SEPC with atypical morphology. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Objective To compare the residual cholesteatoma detection accuracy of diffusion-weighted (DW) and T1 delayed sequences for magnetic resonance at one year postoperative with second-look surgery in pediatric patients who have undergone primary middle ear surgery for cholesteatoma. Methods This was a prospective monocentric consecutive study conducted in a tertiary academic referral center. Children were referred for MR imaging (MRI) one year after surgery. A 1.5 T MRI was utilized, using nonecho-planar DW images and delayed gadolinium-enhanced T1-weighted images. Accuracy of magnetic resonance imaging was assessed by two radiologists before surgery. Interobserver and intraobserver agreements were assessed using the κ test. Magnetic resonance imaging data were compared with surgery, which was considered as the gold standard. Results Twenty-four consecutive unselected pediatric patients were included. Sensitivity, specificity, positive predictive value, and negative predictive value for the first observer were of 40%, 86%, 67%, and 67%, respectively, and those for the second observer were 30%, 86%, 60%, and 63%, respectively. The only two cholesteatoma with a size superior to 3 mm were diagnosed before surgery, but the majority of small cholesteatoma were not detected. Conclusions MRI is a key examen to diagnosed the residual cholesteatoma but is limited by the size of the lesion under 3 mm. Delaying the realization of MRI during follow-up could increase sensitivity, thus avoiding misdiagnosis as well as unnecessary second look surgery.

Ninety genes have been identified to date that are involved in non-syndromic hearing loss, and more than 300 different forms of syndromic hearing impairment have been described. Mutations in SOX10, one of the genes contributing to syndromic hearing loss, induce a large range of phenotypes, including several subtypes of Waardenburg syndrome and Kallmann syndrome with deafness. In addition, rare mutations have been identified in patients with isolated signs of these diseases. We used the recent characterization of temporal bone imaging aspects in patients with SOX10 mutations to identify possible patients with isolated hearing loss due to SOX10 mutation. We selected 21 patients with isolated deafness and temporal bone morphological defects for mutational screening. We identified two SOX10 mutations and found that both resulted in a non-functional protein in vitro. Re-evaluation of the two affected patients showed that both had previously undiagnosed olfactory defects. Diagnosis of anosmia or hyposmia in young children is challenging, and particularly in the absence of magnetic resonance imaging (MRI), SOX10 mutations can mimic non-syndromic hearing impairment. MRI should complete temporal bones computed tomographic scan in the management of congenital deafness as it can detect brain anomalies, cochlear nerve defects, and olfactory bulb malformation in addition to inner ear malformations.

Resume Une etude prospective multicentrique nationale a ete menee incluant les luxations congenitales de hanche diagnostiquees apres l’âge de 3 mois et impliquant outre la Societe francaise d’orthopedie pediatrique, l’Association francaise des pediatres ambulatoires et la Societe francophone d’imagerie pediatrique et prenatale. Elle revele une insuffisance de depistage clinique, evident sur le plan quantitatif et probablement qualitatif, requerant une campagne de communication et d’enseignement pour rappeler les bonnes pratiques. L’interet de l’echographie systematique n’est pas demontre. Des recommandations relatives au depistage de la LCH ont ete elaborees a la lumiere des resultats presentes dans cet article par les auteurs avec la Haute Autorite de sante. La realisation d’une etude nationale prospective randomisee multicentrique est indispensable, a laquelle il semble fondamental d’integrer les medecins generalistes.

Objectives To describe the prenatal ultrasound and magnetic resonance imaging (MRI) findings suggestive of periventricular nodular heterotopia (PNH). Methods This retrospective case series included fetuses referred to our institution for brain MRI between 2007 and 2012, which were diagnosed with PNH and confirmed by postnatal MRI or autopsy. The type of PNH, associated ventriculomegaly and associated malformations are reported. Results We included 11 fetuses (nine female, two male) with a mean gestational age at diagnosis of 31 (range, 23–34) weeks. PNH lesions were small and diffuse (n = 7), large and multiple (n = 1) or single (n = 3). A targeted ultrasound examination performed before fetal MRI missed the diagnosis in four cases (one diffuse and three single); a further ultrasound examination performed after MRI diagnosed PNH in two of these four cases. Ventriculomegaly was present in six cases (four unilateral and two bilateral). PNH appeared in all cases as nodules of intermediate echogenicity protruding into the ventricular lumen. In all cases of diffuse PNH, the frontal horns and bodies of the lateral ventricles appeared square in shape on coronal view, with irregular borders on axial view. Associated cerebral malformations were observed in seven cases and included corpus callosal agenesis (n = 4, with additional malformations in two) and retrocerebellar cyst (n = 3). Extracerebral malformations were also present in two cases. Maternal MRI was performed in five of the six cases of isolated small and diffuse PNH in female fetuses, and demonstrated PNH in two of these. Conclusion PNH is underdiagnosed at prenatal ultrasound, even on targeted scans. Irregular ventricular borders on axial view and irregular square-shaped lateral ventricles on coronal view are suggestive of PNH at prenatal ultrasound. Copyright © 2012 ISUOG. Published by John Wiley & Sons Ltd.

International audience; To investigate the role of cancer predisposing factors (PFs) on the associations between paediatric computed tomography (CT) scan exposures and subsequent risk of central nervous system (CNS) tumours and leukaemia. A cohort of children who underwent a CT scan in 2000-2010 in 23 French radiology departments was linked with the national childhood cancers registry and national vital status registry; information on PFs was retrieved through hospital discharge databases. In children without PF, hazard ratios of 1.07 (95% CI 0.99-1.10) for CNS tumours (15 cases) and 1.16 (95% CI 0.77-1.27) for leukaemia (12 cases) were estimated for each 10 mGy increment in CT x-rays organ doses. These estimates were similar to those obtained in the whole cohort. In children with PFs, no positive dose-risk association was observed, possibly related to earlier non-cancer mortality in this group. Our results suggest a modifying effect of PFs on CT-related cancer risks, but need to be confirmed by longer follow-up and other studies.

Lung diseases associated with surfactant metabolism disorders represent a significant but heterogeneous group of rare disorders. Intra-alveolar accumulation of protein related to surfactant dysfunction leads to cough, hypoxemia and radiological diffuse infiltration. Inherited deficiency of pulmonary surfactant protein B (SP-B) was initially described in term newborns who develop severe respiratory failure at birth. More recently, mutations in surfactant protein C (SP-C) or in proteins required for surfactant synthesis such as ATP-binding cassette, sub-family A, member 3 (ABCA3) or NK2 homeobox 1 (NKX2-1) were identified in newborns with respiratory distress but also in children with diffuse infiltrative pneumonia. The aim of this review is to describe the clinical presentation of these diseases but also the diagnostic tools and the treatments options available.

Objectives To evaluate the diagnostic accuracy of fetal cerebral magnetic resonance imaging (MRI) on a large cohort and to compare pre- and postnatal MRI data. Methods This prospective study included all cases referred to our unit for fetal cerebral MRI examination between June 2006 and December 2009 and which underwent at least one postnatal MRI examination. Cases in which there was termination of pregnancy, fetal death or stillbirth were excluded. The pre- and postnatal diagnoses established by MRI were compared and divided into five subgroups: same diagnosis on pre- and postnatal MRI (Group 1); same diagnosis but different appearance related to the natural course of the disease (Group 2); different diagnosis (related to limitations of fetal MRI) (Group 3); same diagnosis but with additional findings discovered on postnatal MRI examination (Group 4); or same diagnosis but different appearance related to the natural course of the disease (as in Group 2) and associated with additional findings discovered on postnatal MRI examination (Group 5). The prognostic impact of a possible disagreement between pre- and postnatal findings was evaluated. Results One hundred fetuses were included. Fetal MRI was performed at a mean gestational age of 33 (range, 24–39) weeks and postnatal MRI at a mean age of 3.5 months. There were 53 cases classified as Group 1, 32 in Group 2, four in Group 3, 10 in Group 4 and one in Group 5. Thus, in 15 cases (Groups 3–5), there were discrepancies between pre- and postnatal findings (mostly related to corpus callosum anatomy, cortical and migration disorders). The discrepancy was judged to have a prognostic impact in 9/15 cases. Two postnatal MRI examinations were performed in eight cases, in one of which the second examination showed subependymal heterotopia which were not detectable on the first examination. Conclusion Pre- and postnatal MRI data showed good agreement in 85% of cases. There was disagreement with a prognostic impact in 9% of cases. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.

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.

Minor salmonellosis is due to Gram-negative bacilli, which usually cause enterocolitis with potentially severe complications. We report on a case of a clinically uncommon presentation of Salmonella enterica serovar typhimurium infection in an 8-year-old child who presented with acute abdominal pain. We discuss clinically uncommon presentations of salmonella disease in children, as well as its pathology and radiology.

Resume Le cholesteatome est une pathologie connue depuis 1683 mais dont l’etiopathogenie reste inexpliquee. Chez l’enfant, il se presente sous deux formes : le cholesteatome acquis, proche de la forme de l’adulte et le cholesteatome congenital. La frequence de la forme acquise a diminue ces dernieres annees grâce a une meilleure prise en charge des pathologies otitiques de l’enfant. A l’inverse, celle du cholesteatome congenital est en augmentation notamment par une meilleure information des professionnels de sante et de meilleurs outils diagnostiques. Des preuves cliniques et histologiques ont affirme la plus grande agressivite du cholesteatome chez l’enfant, mais il n’existe actuellement aucune explication precise de cette difference. Le diagnostic est clinique mais les examens d’imagerie, tomodensitometrie (TDM) et imagerie par resonance magnetique (IRM) sont indispensables pour le bilan preoperatoire et le suivi postoperatoire. Le developpement recent de nouvelles sequences d’IRM (T1 tardif avec injection de gadolinium et diffusion) permet un diagnostic radiologique de plus en plus precis. Le traitement est chirurgical mais des alternatives therapeutiques en particulier le laser ont ete essayees sans succes. Les complications sont liees a l’atteinte des structures de voisinage et sont le plus souvent de nature infectieuse. Certaines engagent le pronostic vital et doivent etre recherchees systematiquement.

Aim of the Study Determine high-resolution tomography (HRCT) scan characteristics in children with SFTPC mutation and correlate them to histological findings. Patients and Methods This retrospective multicenter study included 15 children (7 females and 8 males) with SFTPC mutations. HRCT scans have been performed in all the children and lung biopsies in 8 children. Results From all signs assessed on initial HRCT scans, ground-glass opacities (n = 14, 93%) and lung cysts (n = 6, 40%) were predominant. Interlobular septal thickening (n = 1, 7%), air space consolidation (n = 1, 7%), paraseptal emphysema (n = 2, 13%), and pulmonary nodules (n = 1, 7%) were also found. Histological analysis revealed accumulation of macrophages in the alveolar lumen, type II pneumocyte hyperplasia, and alveolar septal thickening. Dilatation of the respiratory bronchiole and alveolar duct associated with muscular hyperplasia were also described. Interestingly, lung cysts on HRCT scans were associated with dilatation of terminal bronchioli and alveolar duct in lung biopsies. Conclusion In children with SFTPC mutations, HRCT scan finding was highly correlated to the histological findings and, as such, represent a useful tool to identify patients that may require SFTPC gene sequencing. Pediatr Pulmonol. 2010; 45:1021–1029. © 2010 Wiley-Liss, Inc.

En 2007, le service de radiologie de l’Hopital Trousseau (Paris) et l’IRSN ont initie une analyse detaillee des doses delivrees lors d’examens scanographiques chez l’enfant. Cette etude a porte sur trois types d’examen (thorax, abdomen-pelvis et rochers) et trois tranches d’âge (nouveau ne a 1 an, 4 a 6 ans et 9 a 11 ans). Une premiere analyse des doses a ete realisee a posteriori sur l’activite du second semestre 2006. Elle a montre que les protocoles d’acquisition du scanner, qui satisfaisaient aux recommandations dosimetriques en vigueur, etaient mal respectes dans le service : les pratiques etaient heterogenes et les valeurs moyennes de PDL superieures aux recommandations. Apres ces premiers resultats, une action de sensibilisation a l’optimisation des doses a ete menee aupres du personnel et les protocoles d’acquisition du scanner ont ete optimises. L’analyse dosimetrique reprise en 2008 suivant le schema de 2006 a montre une harmonisation des pratiques au sein du service, une diminution significative des PDL moyens (-50 % par exemple en scanographie thoracique) et le respect systematique des recommandations dosimetriques pour les trois examens etudies.

RADIOLOGIE ET IMAGERIE MEDICALE : Musculosquelettique - Neurologique - Maxillofaciale - 31-203-A-10

We report a rare case of polymicrogyria diagnosed at 27 weeks' gestation on ultrasound examination and associated with cytomegalovirus (CMV) infection. The ultrasound finding suggesting this diagnosis was the direct visibility of the overfolded cortical ribbon. The cerebral surface was clearly visible because of a markedly enlarged pericerebral space associated with micrencephaly secondary to CMV infection. Bilateral opercular dysplasia was also present. Very few sonographic markers of infectious fetopathy were observed other than periventricular cysts located behind both ventricular horns. Magnetic resonance imaging (MRI) of the fetal brain confirmed the ultrasound findings and also showed the presence of marked micrencephaly, whereas cephalic measurements acquired on ultrasound examination (biparietal diameter and head circumference) were within the normal range. This case emphasizes the complementary roles of sonography and MRI in the prenatal diagnosis of cerebral abnormalities. Moreover, it illustrates the fact that polymicrogyria is easier to diagnose on ultrasound examination during the second trimester, before the development of secondary sulci.

Meckel's diverticulum is a common malformation in children, usually asymptomatic, with complications in only 20% of cases. Exceptionally, a tumor can develop in Meckel's diverticulum in children, particularly Burkitt's lymphoma; in adults it can develop into a gastrointestinal stromal tumor, a leiomyosarcoma, or a neuroendocrine tumor such as a carcinoid tumor. The diagnosis of inflammatory pseudotumor following an insidious perforation is rare.We report the case of a 14-month-old boy who presented with fever, asthenia, food refusal, and digestive complaints such as vomiting and tender abdomen suggesting appendicitis. Computed tomography showed an ileal mass of 3cm in diameter, which led to the suspicion of Burkitt's lymphoma. Laparoscopy showed Meckel's diverticulum with a mass of 3×2.8×2cm. Histological examination confirmed the diagnosis of Meckel's diverticulum with gastric heterotopia and showed a proliferation of spindle cells in a myxoid background, with an inflammatory infiltrate made of lymphocytes and plasmocytes. Immunostaining ruled out a malignant tumor. The diagnosis of an inflammatory pseudotumor developing on a Meckel diverticulum with gastric heterotopias was made. The outcome was favorable after surgical resection.While perforation of a Meckel diverticulum with gastric heterotopia is a common finding, the discovery of an inflammatory pseudotumor following a perforation is rare; the differential diagnosis should include Burkitt's lymphoma.

Resume L'evaluation des tumeurs osseuses en imagerie peut faire appel a toutes les methodes de radiodiagnostic. La radiographie simple reste incontournable. Elle evalue la dynamique evolutive de la lesion et permet d'envisager un ou plusieurs diagnostics grâce notamment a des criteres morphologiques et topographiques. La tomodensitometrie est utilisee pour sa resolution spatiale en explorant des regions difficilement accessibles en radiographie, et est egalement interessante pour l'etude de la corticale osseuse et de la matrice tumorale. Les principaux atouts de l'imagerie par resonance magnetique sont sa resolution en contraste et ses nombreux plans de coupe possibles, utiles pour l'extension locoregionale d'une tumeur. La scintigraphie est surtout employee pour savoir si une lesion est unique ou si elle s'integre dans une pathologie polyostotique. Echographie, angiographie, radiologie interventionnelle ont des indications tres precises. Selon les caracteristiques radiographiques d'une lesion, differentes attitudes sont preconisees allant de l'absence de toute investigation complementaire lorsque la benignite est evidente, au bilan d'extension locoregional et general, avant biopsie, en cas de suspicion de malignite.

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.

Resume Les urgences des membres chez l’enfant regroupent principalement trois situations : les traumatismes, les infections et les douleurs de hanche. En urgence, l’exploration repose principalement sur les radiographies simples completees dans de rares situations par une echographie. La tomodensitometrie et l’IRM sont rarement necessaires. La connaissance des particularites pediatriques est indispensable pour le diagnostic et la prise en charge des patients. Les traumatismes des membres de l’enfant se distinguent par le fait qu’il s’agit d’os en croissance a forte composante cartilagineuse et par une resistance biomecanique differente de celle de l’adulte. Les lesions infectieuses pediatriques s’expliquent par les caracteristiques de la vascularisation metaphyso-epiphysaire, la situation intra-articulaire de la metaphyse et le faible attachement du perioste a la corticale. Les boiteries (synovite aigue transitoire, osteochondrite primitive de hanche et epiphysiolyse) sont une cause frequente de consultation dont le diagnostic repose en fonction de l’âge sur l’interpretation des radiographies simples.

Resumen Para el estudio por imagen de los tumores oseos se puede recurrir a todos los metodos de radiodiagnostico. La radiografia directa sigue siendo ineludible. A partir de criterios morfologicos y topograficos, permite evaluar la dinamica evolutiva de la lesion y pensar en uno o mas diagnosticos. La tomografia computarizada se emplea por su resolucion espacial al explorar regiones de dificil acceso para la radiografia, y resulta igualmente interesante para el estudio de la cortical osea y de la matriz tumoral. Las principales ventajas de la resonancia magnetica son la resolucion de contraste y los multiples planos de corte posibles, utiles para determinar la extension locorregional de un tumor. La gammagrafia se emplea sobre todo para saber si una lesion es unica o forma parte de un proceso poliostotico. La ecografia, la angiografia y la radiologia intervencionista tienen indicaciones precisas. Las caracteristicas radiograficas de una lesion determinan la conducta ulterior, desde la ausencia absoluta de otro examen complementario cuando la benignidad es evidente, hasta el estudio de extension locorregional y general, con biopsia previa, en caso de sospecha de malignidad.

Archives de pediatrie - In Press.Proof corrected by the author Available online since mercredi 10 juillet 2013

Acute or rapidly progressive visual loss in children needs urgent attention and treatment. It may be unilateral orbilateral. Etiology depends upon the involved areas: eye ball, optic nerve, retro-chiasmatic pathways. Psychogenic origin is quite common in school-age children, however, it has to be considered last. Unilateral visual loss may be overlooked. Acute total transitory visual loss may be due to epilepsy or to migraine. Rapidly progressive visual loss may be due to retinal disease, optic neuritis or cortical blindness. Management of visual loss depends on clinical features, associated symptoms, and aspect of the optic disc. It needs collaboration between ophthalmologist,pediatrician and neuropediatrician. Retinal hemorrhages first call to mind a traumatic origin. Swelling of the optic disc may be due to increased intracranial pressure or due to optic neuritis. When the optic disc is normal it is necessary to rule out organic diseases before establishing the diagnosis of a psychogenic vision disturbance. In emergency, brain neuroimaging is the best way to diagnose intracranial mass and visualize optic pathways.

International audience; Background Recent epidemiological results suggested an increase of cancer risk after receiving computed tomography (CT) scans in childhood or adolescence. Their interpretation is questioned due to the lack of information about the reasons for examination. Our objective was to estimate the cancer risk related to childhood CT scans, and examine how cancer-predisposing factors (PFs) affect assessment of the radiation-related risk. Methods The cohort included 67 274 children who had a first scan before the age of 10 years from 2000 to 2010 in 23 French departments. Cumulative X-rays doses were estimated from radiology protocols. Cancer incidence was retrieved through the national registry of childhood cancers; PF from discharge diagnoses. Results During a mean follow-up of 4 years, 27 cases of tumours of the central nervous system, 25 of leukaemia and 21 of lymphoma were diagnosed; 32% of them among children with PF. Specific patterns of CT exposures were observed according to PFs. Adjustment for PF reduced the excess risk estimates related to cumulative doses from CT scans. No significant excess risk was observed in relation to CT exposures. Conclusions This study suggests that the indication for examinations, whether suspected cancer or PF management, should be considered to avoid overestimation of the cancer risks associated with CT scans. © 2015 Cancer Research UK.

To identify at prenatal ultrasound (US) the features of apparently isolated subependymal pseudocysts (SEPC) that may indicate underlying pathology and should lead to further investigations.This was a retrospective study of cases with SEPC detected on prenatal US and/or magnetic resonance imaging (MRI). Those with apparently isolated SEPC at US were classified into two groups as follows: Group 1 (n = 29): normal prenatal US and MRI (except for SEPC) and normal outcome; Group 2 (n = 12): normal prenatal cerebral US (except for SEPC) and abnormal prenatal cerebral MRI with or without abnormal outcome. A third group (n = 9) included cases with abnormal prenatal US and MRI. The latter cases with obvious cerebral abnormalities at US were excluded from the statistical analysis as they do not represent a diagnostic dilemma for clinicians. Groups 1 and 2 were analyzed, comparing them with respect to their SEPC characteristics (size, number, location in relation to the caudothalamic notch and the ventricular horns and morphology) and extracerebral abnormalities.The mean ± SD SEPC great axis was longer in Group 2 (11.67 ± 5.82 mm) than it was in Group 1 (8.00 ± 5.64 mm) (P = 0.021), suggesting an optimal cut-off for size of SEPC of ≥ 9 mm (sensitivity = 75%, specificity = 62%) to maximize sensitivity for predicting pathological outcome. SEPC adjacent to the temporal horns and SEPC located posterior to the caudothalamic notch were observed more frequently in Group 2, indicating their association with poor outcome (P = 0.003 and P = 0.003, respectively). Atypical morphology and extracerebral abnormalities were observed more frequently in Group 2 (P = 0.013 and P = 0.044, respectively). There was no statistically significant difference between groups for either number or location of cysts along the inferior wall or adjacent to the lateral wall of the frontal horns (P = 0.591 and P = 0.156, respectively).When apparently isolated SEPC are observed at prenatal US, further investigations should be performed under the following circumstances: (1) SEPC great axis ≥ 9 mm; (2) SEPC adjacent to the occipital and temporal horns; (3) SEPC located posterior to the caudothalamic notch; (4) SEPC with atypical morphology.

Ninety genes have been identified to date that are involved in non-syndromic hearing loss, and more than 300 different forms of syndromic hearing impairment have been described. Mutations in SOX10, one of the genes contributing to syndromic hearing loss, induce a large range of phenotypes, including several subtypes of Waardenburg syndrome and Kallmann syndrome with deafness. In addition, rare mutations have been identified in patients with isolated signs of these diseases. We used the recent characterization of temporal bone imaging aspects in patients with SOX10 mutations to identify possible patients with isolated hearing loss due to SOX10 mutation. We selected 21 patients with isolated deafness and temporal bone morphological defects for mutational screening. We identified two SOX10 mutations and found that both resulted in a non-functional protein in vitro. Re-evaluation of the two affected patients showed that both had previously undiagnosed olfactory defects. Diagnosis of anosmia or hyposmia in young children is challenging, and particularly in the absence of magnetic resonance imaging (MRI), SOX10 mutations can mimic non-syndromic hearing impairment. MRI should complete temporal bones computed tomographic scan in the management of congenital deafness as it can detect brain anomalies, cochlear nerve defects, and olfactory bulb malformation in addition to inner ear malformations.

A prospective multi-centre nationwide study of patients with congenital dislocation of the hip (CDH) diagnosed after 3 months of age was conducted with support from the French Society for Paediatric Orthopaedics (Société Française d’Orthopédie Pédiatrique [SoFOP]), French Organisation for Outpatient Paediatrics (Association Française de Pédiatrie Ambulatoire [AFPA]), and French-Speaking Society for Paediatric and Pre-Natal Imaging (Société Francophone d’Imagerie Pédiatrique et Prénatale [SFIPP]). The results showed inadequacies in clinical screening for CDH that were patent when assessed quantitatively and probably also present qualitatively. These findings indicate a need for a communication and educational campaign aimed at highlighting good clinical practice guidelines in the field of CDH screening. The usefulness of routine ultrasound screening has not been established. The findings from this study have been used by the authors and French National Health Authority (Haute Autorité de Santé [HAS]) to develop recommendations about CDH screening. There is an urgent need for a prospective randomised multi-centre nationwide study, which should involve primary-care physicians.