Anlise do seu espectro morfolgico em doze casos de autpsia

ABSTRACT - The term holoprosencephaly (HPC) is used to indicate the group of hemispheric deformitiescaused by a failure in the development of the prosencephalic vesicle. The purpose of this study is to explainthe morphologic specter of twelve cases of HPC, qualify them, and compare them to the literature. It wasevaluated 5837 pediatrics necropsies, and there were 12 cases of HPC. Data like gender, age and the presenceof another associated malformations were evaluated and compared to another malformations and to thetotal number of necropsies. The majority of the cases was male (66.66%), and stillborns (75%). The mostfrequently type of HPC found was the lobar type (58.3%), and the most frequently type of facial alterationwas the ciclopy (25%). There is a statistic tendency to HPC affect the male sex, in comparison with otherneurologic malformations. Besides, the stillborns are more frequently observed in HPC than in other neurologicmalformations.KEY WORDS: holoprosencephaly, autopsy, nervous system malformations.