Holoprosencephaly: Morphological aspects of twelve cases of autopsy

Lúcia De Noronha, Ramon Coral Ghanem, Fabíola Medeiros, José Knopfholz, Tiago Augusto Magalhães, Gilberto Antunes Sampaio, Maria José Serapião, Luiz Fernando Bleggi Torres

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Abstract

The term holoprosencephaly (HPC) is used to indicate the group of hemispheric deformities caused by a failure in the development of the prosencephalic vesicle. The purpose of this study is to explain the morphologic specter of twelve cases of HPC, qualify them, and compare them to the literature. It was evaluated 5837 pediatrics necropsies, and there were 12 cases of HPC. Data like gender, age and the presence of another associated malformations were evaluated and compared to another malformations and to the total number of necropsies. The majority of the cases was male (66.66%), and stillborns (75%). The most frequently type of HPC found was the lobar type (58.3%), and the most frequently type of facial alteration was the ciclopy (25%). There is a statistic tendency to HPC affect the male sex, in comparison with other neurologic malformations. Besides, the stillborns are more frequently observed in HPC than in other neurologic malformations.
Original languageAmerican English
Pages (from-to)913-919
Number of pages821
JournalArquivos de Neuro-Psiquiatria
StatePublished - 1 Dec 2001
Externally publishedYes

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De Noronha, L., Ghanem, R. C., Medeiros, F., Knopfholz, J., Magalhães, T. A., Sampaio, G. A., Serapião, M. J., & Torres, L. F. B. (2001). Holoprosencephaly: Morphological aspects of twelve cases of autopsy. Arquivos de Neuro-Psiquiatria, 913-919.