MALFORMA˙'ES DO SISTEMA NERVOSO CENTRAL ANLISE DE 157 NECR"PSIAS PEDITRICAS

RESUMO - As malformacoes do sistema nervoso central (SNC) tem sido relatadas como de alta prevalencia,acometendo 5 a 10 criancas para 1000 nascidos vivos. Estudamos a frequencia das malformacoes do SNC em5837 necropsias pediatricas realizadas no Servico de Anatomia Patologica do Hospital de Clinicas-UFPR, entre1960 e 1995. Encontraram-se 157 malformacoes do SNC, correspondendo a 2,69% das necropsias. Asmalformacoes mais comuns foram os defeitos do tubo neural (61%), incluindo 47 anencefalias e 45 casosclassificados no grupo das mieloencefaloceles. As anomalias da vesicula prosencefalica foram responsaveis por8% das malformacoes encefalicas, havendo sete casos de holoprosencefalia. Em 3%, as alteracoes ocorreram nafossa posterior, com tres malformacoes de Arnold-Chiari. No presente estudo, os indices de mortalidade pormalformacoes do SNC foram mais altos no periodo neonatal.PALAVRAS-CHAVE: malformacao, sistema nervoso central, necropsia.Malformations of the central nervous system: analysis of 157 pediatric autopsiesABSTRACT - The malformations of the central nervous system affect about 5 to 10 children per 1000 births. Westudied the central nervous system malformations in 5837 pediatric autopsies performed in the Sector of AnatomicPathology, Hospital de Clinicas-UFPR, between 1960 and 1995. There were 157 central nervous systemmalformations (2.69%), the commonest were neural tube defects (61%): 47 cases of anencephaly and 45 casesclassified in the group of myeloencephaloceles. The anomalies of the prosencephalic evagination correspondedto 8% of all central nervous system malformations, with seven cases of holoprosencephaly. Posterior fossamalformations occurred in 3%, with three cases of Arnold-Chiari. In the present study, the mortality rate due tocentral nervous system malformations was higher in the neonatal period.KEY WORDS: malformation, central nervous system, autopsy.