Holoprosencephaly (HPE) consists of a spectrum of malformations related to incomplete separation of the prosencephalon. It occurs between the 18th and the 28th day of gestation and affects both the forebrain and the face. It is estimated to occur in 1/16,000 live births and 1/250 conceptuses. Alobar HPE, the most common and most severe type of HPE, can be diagnosed in the first trimester of pregnancy using ultrasound. Among survivors, the long-term outcome is poor and depends on the type and severity of the pathology as well as associated anomalies.

Keywords : Holoprosencephaly, Alobar, Malformation.

Résumé :

L'holoprosencéphalie (HPE) comprend un spectre de malformations liées à une séparation incomplète du prosencéphale. Elle survient entre le 18ème et le 28ème jour de gestation et touche à la fois le cerveau antérieur et le visage. On estime qu'elle survient chez 1/16 000 naissances vivantes et 1/250 conceptions. L'HPE alobaire, le type d'HPE le plus courant et le plus grave, peut être diagnostiqué au cours du premier trimestre de la grossesse par échographie. Chez les survivants, l’évolution à long terme est médiocre et dépend du type et de la gravité de la pathologie ainsi que des anomalies associées.

Mots cléfs : Holoprosencéphalie, Alobaire, Malformation.

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H. Halim

Service de gynécologie obtstétrique de l’Hôpital maternité Souissi de Rabat