Abstract
The holoproscencephaly is one of the most serious and common among the facial and brain malformations; it consists in the incomplete or null diverticulation of the embrionary prosencefalus through the brain hemispheres and lateral ventricles; is accompanied by defects of the middle line structures, as mouth and nose, it has and early showing in the development of the central nervous system, and it occurs as a consequence of a possible failure over a set of molecules, that include the Sonic Hedgehog protein, and the superfamily genes BPM, indispensable to the normal formation of the encephalon, moreover it has been associated with anomalies over the 13, 3, 7 and 18 chromosomes. Till now, the diagnostic and classification is anatomic-pathologic. It describes two cases of the semilobar diagnostic by ecography, and later by necropsy. The objective of this article is to expose thy cases and done a brief description of the physiopathology involve mechanism.
Keywords: Holoproscencephaly. Chromosome Aberrations. Cleft Palate.
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