Le syndrome de Pallister–Hall associe une polydactylie, une épiglotte bifide, des anomalies génito-urinaires et anales et un hamartome hypothalamique. Celui-ci est l’élément clé du diagnostic, et conditionne le pronostic vital par le syndrome tumoral et l’hypopituitarisme qui l’accompagnent. Nous relatons le cas d’un nouveau-né dont la présentation clinique évocatrice d’un syndrome de Pallister–Hall a fait rechercher un hamartome hypothalamique.

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.

Abstract 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.

Introduction In France, all classical Hodgkin Lymphoma (cHL) children and adolescents cases are presented in seven multidisciplinary pediatric regional tumor boards (RCPP) to define treatment plan. Between January 2013 and December 2016, no trial was open for recruitment and treatment recommendations were based on the preliminary results of the EuroNet-PHL-C1 protocol. This retrospective work aims to describe RCPP decisions, delivered treatment and outcome of cHL patients during this period for one region (Ile-de-France).

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 18 years) 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 < 13 years (OR: 10.7; 95% CI: 1.3-148.2) (P= 0.022) and a whirlpool sign at MRI (OR: 61.0; median unbiased estimate, 7.2) (P< 0.0001). When a mass was present, the best quantitative MRI criteria for AT were mass volume and ovary-corrected volume >= 30 cm(3) (kappa = 0.72 and 0.61, respectively), mass axis length >= 5 cm (kappa = 0.90), and mass surface area > 14 cm(2) (kappa = 0.58), with moderate to almost perfect interobserver agreement. The overall sensitivity, specificity and accuracy of MRI for the diagnosis of AT were 100% (10/10; 95% CI: 69-100), 94% (17/18; 95% CI: 73-100) and 96% (27/28; 95% CI: 82-100) respectively, with perfect interobserver agreement (kappa = 1). Conclusion: In pediatric patients with suspected AT and inconclusive initial ultrasound examination, a strategy including MRI as a second-line imaging modality should be considered if MRI does not delay a potential surgery. (C) 2020 Societe francaise de radiologie. Published by Elsevier Masson SAS. All rights reserved.

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

Objective: The goal of the study is to investigate the association of pertinent preoperative temporal bone computed tomography (CT) and brain magnetic resonance imaging (MRI) results and intraoperative surgical findings and complications of pediatric cochlear implantation reported in academic settings. Methods: This is a retrospective review of cochlear implant patients who received a pre-operative temporal bone CT and MRI of the brain between 2005 and 2012 at academic pediatric otolaryngology practices within children's hospitals in the United States and France. Scans were reviewed in a double-blind fashion and compared to intraoperative findings. Results: 91 children were analyzed (mean age 5.54 +/- 0.58 years). A small facial recess identified on CT was associated with difficult insertion of electrodes (P = 0.0003). A prominent sigmoid sinus noted on CT was associated of difficult insertion of electrodes (P = 0.01), iatrogenic tegmen dehiscence (P = 0.005), as well as difficult round window access (P = 0.025). No specific CT finding was found to be associated with external auditory canal injury, perilymphatic gusher, or iatrogenic facial nerve injury. MRI brain and internal auditory canal findings were not predictive of surgical outcomes. Conclusions: Preoperative CT and MRI remain an important planning tool for pediatric cochlear implantation, particularly in academic institutions. The findings of our study demonstrate that a detailed assessment of both preoperative CT and MRI are valuable for teaching and surgical planning.

Lipoblastoma is a rare, benign tumor of embryonic fat occurring in infants and young children. Generally found in the trunk or extremities, it is rarely located in other areas such as the head and neck, where it may cause respiratory symptoms. A median, pretracheal location is extremely atypical and, to our knowledge, has never been described. Here we describe such a tumor in an 11-month-old infant. The heterogeneous, fatty content of the tumor is very suggestive of lipoblastoma, whatever its location, particularly before age 3 years.

En pédiatrie, l’indication la plus fréquente de la scintigraphie osseuse est la suspicion d’infection ostéoarticulaire. Cette pathologie, particulièrement fréquente avant 5 ans, est parfois de diagnostic difficile. Le radiopharmaceutique de référence dans cette population est un diphosphonate marqué par le Technetium-99m. Les avantages de l’examen sont une sensibilité élevée, l’absence de sédation, et la possibilité de visualiser l’ensemble du squelette. En cas de suspicion de spondylodiscite, l’imagerie par résonance magnétique (IRM) est l’examen de première intention.

Le nombre de luxations congénitales de hanches découvertes à l’âge de la marche est en augmentation. L’échographie de hanche est l’examen clé du dépistage de la luxation de hanche après l’examen clinique. Dans cette fiche technique, nous rappelons les indications et les bonnes pratiques pour le dépistage, mais également pour le diagnostic échographique de la luxation congénitale de hanche.

Décrire la présentation clinique et radiologique ainsi que le traitement et l’évolution d’enfants présentant une atteinte pulmonaire d’histiocytose langerhansienne. Les enfants avec une atteinte pulmonaire définie par des anomalies radiologique à la radiographie ou au scanner ont été inclus à partir du registre français de l’histiocytose (1983–2016). Les données démographiques, la symptomatologie clinique, les résultats radiologiques, les épreuves fonctionnelles respiratoires, l’analyse du lavage broncho-alvéolaire (LBA), la prise en charge thérapeutique et l’évolution ont été analysés. Ces caractéristiques ont été comparées en fonction de l’âge des enfants (moins de 10 ans et plus de 10 ans) et de l’atteinte des organes « à risque » (moelle, foie et rate). Parmi les 1749 enfants inclus dans la cohorte, 166 avaient une atteinte pulmonaire (soit 9,5 %). Le groupe des enfants de moins de 10 ans (n = 137) avait un âge médian au diagnostic de 1,03 an, une activité de la maladie plus importante avec une médiane de 4 organes atteints et des organes à risques plus souvent impliqués. Le groupe des plus de 10 ans (n = 29) avait un âge médian au diagnostic de 15,47 ans, moins d’organes impliqués (notamment moins d’organes à risques) et 35 % d’entre eux étaient tabagiques. Les scanners de 51 patients ont été revus (146 scanners) retrouvant des nodules dans 62 % (18/29) avec un score moyen (allant de 0 à 12) et des kystes dans 45 % (13/29). Un LBA a été réalisé chez 54 patients avec 48 % des patients présentant un taux de CD1A > 10 %. Avant 1998, 92 % des enfants ont reçu une chimiothérapie (essentiellement vinblastine et corticoïdes) et, après 1998, une 2e induction (53 %) puis une 2e ligne de chimiothérapie par anti-2CDA et Aracytine® (30 %) en cas de réponse insuffisante. Ce nouveau schéma a amélioré le pronostic avec une augmentation de la survie à 5 ans de 56 à 88 % en cas d’atteinte des organes à risques mais le taux de séquelles reste élevé, surtout en cas d’atteinte des organes à risques (46 %). Cette étude décrit l’atteinte pulmonaire de l’histiocytose langerhansienne pédiatrique, avec une cohorte importante pour une pathologie rare. L’expression de la gravité est variable, sans corrélation avec les scores radiologiques. L’évolution de la prise en charge thérapeutique a permis une amélioration significative du pronostic.

Introduction. 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.Observation. 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 3 cm in diameter, which led to the suspicion of Burkitt's lymphoma. Laparoscopy showed Meckel's diverticulum with a mass of 3 x 2.8 x 2 cm. 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.Conclusion. 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. (C) 2016 Elsevier Masson SAS. All rights reserved.

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.