Baqueiro-Canto R, Padrón-Arredondo G

Language: Spanish
References: 16
Page: 18-21
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ABSTRACT

Introduction. Several types of encephalo-dysplasias associated with cystic formations have been described. In 1904 Thurnbull described a case under the term cystic aplasia, where both parietal lobes were involved. In 1950 Hamby, Krauss and Beswick reported seven children with dysplasia using the term hydranencephaly, as it is known today.
Clinical case 1. Term male newborn patient obtained by caesarean section with Apgar, Silverman and normal somatometry, from a mother of 23 years of age, HIV carrier without prenatal control who was identified by obstetric ultrasound as hydranencephaly vs severe obstructive ventriculomegaly. Ultrasound and CT scan prove the diagnosis. The patient dies seven days after birth.
Clinical case 2. Three-month-old male, product of the second pregnancy of 35 weeks gestation by vaginal delivery that does not breathe at birth and is intubated; obstetric ultrasound identified hydrocephaly. The new transfontanellar ultrasound showed hydroencephaly pending nuclear magnetic resonance for diagnostic reconfirmation. The definitive diagnosis by transfontanellar ultrasound was hydroencephaly.
Discussion. The aetiology of hydranencephaly is controversial but since the meninges and skull are intact, it is presumed that from the rudiments of neopallium the defect is already present during the period of embryological formation of these layers. Fetal and postnatal neuroimaging data and histopathological findings point to early occlusion of the internal carotid arteries, which occurs mainly between the eighth and twelfth week of gestation, as the main pathogenic mechanism.

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