Abstract

EXENCEPHALY: A MAJOR FORM OF ENCEPHALOCELE, ABOUT A CASE AND REVIEW OF THE LITERATURE

*M. Azerki, N. Tijani, Z. Kabba, M. H. Alami, Z. Tazi, A. Filali and R. Bezad

Abstract

Exencephaly is defined as the exteriorization of brain tissue and/or meninges outside the cranial cavity through a congenital bony defect. Its prevalence varies from 0.8 to 4 per 10,000 live births with a female predominance. Risk factors are increased by hyperthermia, valproic acid intake, folic acid and vitamin B12 deficiency during the periconceptional period and diabetes. Antenatal diagnosis is based mainly on ultrasound, which is the imaging of choice in antenatal screening for brain malformations, which will be done in the 1st trimester. The treatment is essentially surgical: by reintegration of the hernia contents, the vital and functional prognosis of encephaloceles depends on several factors. As for prevention, it is based essentially on the intake of folic acid in the pre-conceptional period. We report in our observation the case of a pregnancy carried to term in a 28 year old primigravida, without any notable pathological history, with evolution marked by a gestational diabetes balanced under hygienic-dietary measures, the diagnosis of exencephaly was raised following a morphological ultrasound carried out at 16SA, the delivery took place by vaginal route, and the diagnosis of exencephaly was retained on the basis of a post natal MRI.

Keywords: Exencéphalie, défect, encéphalocèle, congénital, périconceptionnelle, fosse cérébrale, alphafoetoproteine.