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      • Record: found
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      Is Open Access

      Holoprosencephalie alobaire dans un contexte de syndrome polymalformatif: apport de l'imagerie, à propos d'un cas

      case-report

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          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          L'holoprosencéphalie est une malformation cérébrale rare, d’étiologies multiples et souvent associée à des anomalies faciales évocatrices. Cette pathologie, résultant d'un défaut de développement précoce du prosencéphale, est de pronostic fœtal extrêmement réservé en particulier pour la forme alobaire. Nous rapportons à travers ce cas clinique, une holoproséncéphalie alobaire diagnostiquée à l'imagerie (ETF, tomodensitométrie) et relevée par un syndrome polymalformatif chez un nouveau-né de 03 mois.

          Most cited references7

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          The etiopathologies of holoprosencephaly

          R.C. Dwight, T Minal, A.H. Jill … (2005)
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            [Medial cleft lip: one diagnosis masking another].

            A Bouaïchi, N Mansouri, A Hiroual … (2011)
            Median cleft lip and palate are one of the facial manifestations of holoprosencephaly, a brain malformation resulting from a defect in the development of the prosencephalon, the embryonic precursor of the forebrain. It can occur in isolation or as part of a broader pattern of malformation in many different syndromes. The diagnosis of holoprosencephaly requires a complete malformative assessment. a newborn baby seen at birth for a median cleft lip associated with a complex craniofacial malformation. The search for malformations revealed the absence of the median cerebral structure and trisomy 13. The baby died at 48 h. A 28-day-old newborn baby consulting for a median cleft lip and palate. The brain ultrasounds showed no anomalies of the median cerebral structure. The malformative assessment revealed no other malformation. Holoprosencephaly is a complex developmental brain malformation. From the advances in neuroimaging and genetics, our understanding of the etiology and pathogenesis of this condition has advanced dramatically. This advance should lead to improved management of common medical complications and more optimal family counseling. Surgical treatment is the preferred therapy for viable malformations. Copyright © 2010 Elsevier Masson SAS. All rights reserved.
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              [Holoprosencephaly with neurogenic hypernatremia].

              J-C Cuvellier, J.-M. Cuisset, G Soto … (1998)
              Semi-lobar holoprosencephalies can be seldom complicated by neurogenic hypernatremia, which must be distinguished from other causes of hypernatremia. In two admitted children with semi-lobar holoprosencephaly, 7 months and 4 years old, biological data revealed chronic hypernatremia and hyperosmolarity without clinical signs of dehydration, which were finally attributed to a neurogenic hypernatremia. Neurogenic hypernatremia must be clearly differentiated from other causes of hypernatremia since it never causes specific complications.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Pan Afr Med J
                Journal ID (iso-abbrev): Pan Afr Med J
                Journal ID (publisher-id): PAMJ
                Title: The Pan African Medical Journal
                Publisher: The African Field Epidemiology Network
                ISSN (Electronic): 1937-8688
                Publication date (Electronic): 30 June 2013
                Publication date Collection: 2013
                Volume: 15
                Electronic Location Identifier: 83
                Affiliations
                [1 ]Service de radiologie de l'hôpital Saint-Jean de Dieu/UFR des Sciences de la Santé, Université de Thiès, Sénégal
                [2 ]Service de pédiatrie de l'hôpital Saint-Jean de Dieu/UFR des Sciences de la Santé, Université de Thiès, Sénégal
                [3 ]Département de biophysique de l'UFR des Sciences de la Santé, Université de Thiès, Sénégal
                [4 ]Département de médecine interne de l'UFR des Sciences de la Santé, Université de Thiès, Sénégal
                Author notes
                [& ]Corresponding author: Dr. Aliou Amadou Dia, radiologue, Assistant Chef de Clinique à l'UFR de Santé de l'université de Thiès, BP 22254 Dakar-Ponty, Sénégal
                Article
                Publisher ID: PAMJ-15-83
                DOI: 10.11604/pamj.2013.15.83.2797
                PMC ID: 3810221
                PubMed ID: 24171066
                SO-VID: 9a9881e4-54c9-4bd6-8c3a-cf35fd02cd7c
                Copyright © © Dia Aliou Amadou et al.
                License:

                The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 05 May 2013
                Date accepted : 23 June 2013
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Medicine
                Keywords: syndrome polymalformatif,holoprosencéphalie,fente labio-palatine,echographie trans-fontanellaire,tomodensitométrie,polymalformative syndrome,holoprosencephaly,cleft lip and palate,transfontanellar ultrasound,ct
                Data availability:
                ScienceOpen disciplines: Medicine
                Keywords: syndrome polymalformatif, holoprosencéphalie, fente labio-palatine, echographie trans-fontanellaire, tomodensitométrie, polymalformative syndrome, holoprosencephaly, cleft lip and palate, transfontanellar ultrasound, ct

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