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Le Touquet – Paris-Plage, France

Le Monnier A.,University of Paris Descartes | Le Monnier A.,World Health Organization | Le Monnier A.,Center Hospitalier Of Versailles | Blanot S.,Service de Neurochirurgie Pediatrique | And 4 more authors.
Journal of Clinical Microbiology | Year: 2011

We report a case of ventriculoperitoneal (VP) shunt infection in a 3-year-old boy caused by the food-borne pathogen Listeria monocytogenes, subsequent to acute peritonitis. This unusual presentation of central nervous system (CNS) listeriosis underlines the ability of the bacteria to form and survive within biofilms on indwelling medical devices. Bacterial persistence may lead to treatment failure and spreading. We highlight the helpfulness of specific quantitative real-time PCR for the hly gene (PCR-hly) for the diagnosis and follow-up of such infections in detecting bacterial persistence within medical devices despite effective antibiotic treatment. Only the surgical replacement of the VP shunt will resolve the infection. Copyright © 2011, American Society for Microbiology. All Rights Reserved. Source

" Orbital tumors (OT) are neoplasms of the bony orbit and contents except for the eyeball." Given this definition, we exclude the retinoblastomas, which are the most frequent tumors of this anatomical area in the pediatric population. Although these tumors are rare, there is a great variety of etiologies. Among them, the most frequent OTs in childhood are rhabdomyosarcomas and metastatic tumors: metastatic neuroblastomas and leukemias (chloromas). Moreover, adult and pediatric varieties of OT are made up of two clusters of distinctive histological entities. Examination must be careful: measurement, direction of proptosis, impairment of ocular motility, compressive optic neuropathy, strabismus, etc. Age at onset as well as unilateral or bilateral proptosis are important features. Benign tumors such as dermoid cysts or hemangiomas grow slowly, whereas rapid growth suggests a malignant tumor. Fundi and visual acuity should be checked. The general examination focuses especially on cutaneous abnormalities such as café au lait spots and subcutaneous nodules in neurofibromatosis type 1 (NF1), a hemangioma that often involves the cutaneous portion of the eyelid. When a metastatic tumor is suspected, abdominal palpation and ultrasonography must be performed. Ultrasonography leaves the posterior third of the orbit inaccessible to exploration. The depth to which it can penetrate the orbit is limited (20. mm). Most of the time, standard x-rays, CT, and MR imaging allow for an adequate assessment and usually provide a diagnosis. In children, CT and MRI sometimes require anesthesia to be properly performed. © 2010 Elsevier Masson SAS. Source

Guinet A.,University Pierre and Marie Curie | Audry G.,University Pierre and Marie Curie | Zerah M.,Service de Neurochirurgie Pediatrique | Forin V.,University Pierre and Marie Curie
Progres en Urologie | Year: 2012

Objective: To clarify bladder and bowel function of children with lipomas of the conus, without, before and after neurosurgery. Patients and methods: Retrospective analysis of 114 children with a lipomas of the conus, followed in our pediatric neuro-urology department from 1993 to 2010. Several data were collected: bladder and bowel symptoms, bladder and anorectal continence, neurosurgical indication and age, clinical modification after neurosurgery, investigations carried out in pre- and post-surgery treatment, associated bladder and bowel treatment. Results: Forty-nine of the 77 children (63.6%) operated on had never been seen before surgery in our neuro-urology department. Seventy-seven children (67.5%) underwent a neuro-surgery, 60% indicated due to a neurogenic bladder. Before neurosurgery, 66 children (85.7%) had spontaneous miction. Five children (6.5%) had bladder intermittent catheterization. Forty of these patients (56.3%) were continent. After neurosurgery and a specialized consultation in neuro-urology, 54 children (70.1%) were continent. Thirty-seven children (48%) had spontaneous miction. Thirty-seven children (48%) had bladder intermittent catheterization and drug of overactive detrusor. Fifty-two children (67.5%) were constipated after surgery. Seventy-seven percent of the treatments for bowel symptoms were effective in terms of continence. Conclusion: The existence of a neurogenic bladder was one of the main indications for neurosurgery. These results suggest that the complexity of care requires neurosurgical, urological surgeon and neuro-urology physician to achieve the explorations and urinary and digestive treatment in order to preserve renal function and both continences. © 2012 . Source

Vigneron J.,UF de genetique medicale | Segura P.B.,Service de radiologie | Klein O.,Service de Neurochirurgie Pediatrique
Birth Defects Research Part A - Clinical and Molecular Teratology | Year: 2015

Background: New forms and varieties of craniosynostoses are continuously identified due to the current increased interest of clinicians and geneticists especially since the introduction of microarray-based comparative genomic hybridization (Array-CGH) techniques in the diagnostic setting of patients with craniofacial anomalies. Methods: In this report, we describe the case of an infant who associated the early fusion of the metopic and both the coronal sutures. The interaction of the early fusion of the anterior group of the main cranial sutures gave the infant a particular clinical phenotypes with a Y configuration of the frontal bone and a globally reduced size of the skull. Such a deformity was observed in utero and was subsequently confirmed by the postnatal imaging of the head. Results: This phenotype was never described previously in antenatal period to our knowledge. The array-CGH showed a heterozygous 9.0 Mb deletion in the chromosomal region 7p21.1p21.3 encompassing approximately 25 other genes, spanning from THSD7A to TWIST1/FERD3L. Conclusion: This case further illustrates the variability of the clinical spectrum of craniofacial disorders associated with TWIST1 abnormalities. It is important to note that the Saethre-Chotzen syndrome caused by microdeletion is generally characterized by a mental disability. However, of interest, the postoperative psychomotor development of the child considered hereby was within the normal limits. © 2015 Wiley Periodicals, Inc. Source

Lagaude M.,Caen University Hospital Center | Barreau M.,Caen University Hospital Center | Jokic M.,Caen University Hospital Center | Gerard M.,Caen University Hospital Center | And 4 more authors.
Annales de Dermatologie et de Venereologie | Year: 2014

Background. - Crouzon syndrome with acanthosis nigricans is a rare form of Crouzon syndromein which craniosynostosis and facial dysmorphism are associated with acanthosis nigricans.Patients and methods. - Cutaneous examination of a 9-year-old child presenting bicoronal cra-niosynostosis revealed acanthosis nigricans of the cervical, axillar, inguinal and popliteal regionswhich appeared at the age of two. He had a dysmorphic face including a large forehead, hyperte-lorism, mid-face hypoplasia, prognathism and low-set ears. These clinical anomalies suggesteda case of Crouzon syndrome with acanthosis nigricans, which was later confirmed by the findingof a mutation in the FGFR3 gene.Discussion. - Acanthosis nigricans in children is often a cutaneous marker of insulin resistance.However, it may also form part of diverse diseases, notably those of genetic origin. The associa-tion of craniosynostosis and acanthosis nigricans allows incrimination of the FGFR3 gene fromthe outset and diagnosis of Crouzon syndrome with acanthosis nigricans. In the present case,dermatological examination allowed an aetiology of craniosynostosis to be determined. © 2014 Elsevier Masson SAS. All rights reserved. Source

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