Resumo

DIAZ, Mardorys et al. Dysraphia-exencephaly-anencephaly sequence associated with amniotic band, Klinefelter syndrome and polyhydramnios. Salus [online]. 2016, vol.20, n.2, pp.44-48. ISSN 1316-7138.

Neural tube defects are a complex of central nervous system abnormalities that are among the most common human congenital abnormalities, second only to cardiovascular defects, excluding chromosomal abnormalities and heterogeneous group. It is characterized as the end result of a progressive anomaly that starts with a dysraphism the rostral neuropore by a mesenchymal defect, followed by a second phase exhibit distinct cerebral hemispheres developed beyond the embryonic skull concluding with a phase of disintegration. It can occur as a single event or associated with other malformations. We report three cases of anencephaly -exencephaly-dysraphism sequence, one of them in the presence of polyhydramnios, other associated amniotic band syndrome and other chromosomal study reports Klinefelter syndrome, all with a common result, poor perinatal outcome.

Palavras-chave : anencephaly; exencephaly; amniotic band; Klinefelter syndrome; polyhydramnios; dysraphism.

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