Malformações cefálicas congênitas

As malformações cefálicas congênitas (português brasileiro) ou malformações cefálicas congénitas (português europeu) são causadas por danos ou desenvolvimento anormal do sistema nervoso central. Cefálico é um termo que quer dizer "cabeça" ou "extremo superior do corpo". Congênito(pt-BR) ou Congénito(pt-PT?) significa que o transtorno se apresenta usualmente antes do nascimento. Observe-se que as malformações do formato do crânio não estão necessariamente associadas à malformações do sistema nervoso e vice versa.

Cranio de infante apresentando hipertelorismo

As malformações cefálicas congênitas e/ou do sistema nervoso não são causadas necessariamente por um só fator, podendo ser provocados por condições hereditárias ou genéticas ou por exposições ambientais durante a gravidez, tais como medicamentos que a mãe tenha tomado, infecção materna ou exposição a radiações. Algumas malformações cefálicas ocorrem quando as suturas cranianas (as juntas fibrosas que conectam os ossos do crânio) se unem prematuramente a exemplo da síndrome de Crouzon.

A maioria das malformações são causadas por um problema que ocorre nas primeiras etapas do desenvolvimento do sistema nervoso fetal. O sistema nervoso humano desenvolve-se a partir de uma capa pequena e especializada de células localizadas na superfície do embrião. Nas primeiras etapas do desenvolvimento, esta capa de células forma o tubo neural, uma envoltura estreita que se fecha entre a terceira e quarta semana do gravidez para formar o cérebro e a medula espinhal do embrião. Quatro processos principais são responsáveis do desenvolvimento do sistema nervoso: a proliferação celular, um processo no qual as células nervosas se dividem para formar novas gerações de células; a migração celular, um processo no qual as células nervosas se movem de seu lugar de origem até o lugar onde estarão o resto da vida; a diferenciação celular, um processo durante a qual as células adquirem características individuais; e a morte celular, um processo natural no qual as células morrem.

Os danos ao sistema nervoso em desenvolvimento são uma causa importante de transtornos crônicos e incapacitações e, às vezes, da morte de bebês, crianças e até adultos. Os danos sofridos pelo sistema nervoso em desenvolvimento podem afetar a mente e o corpo em graus que variam muito. Muitas incapacidades são suficientemente leves e permitem que os pacientes afetados possam viver normalmente.

Os índices mesocefálicos estabelecidos pela craniometria vão de 76° a 80° os extremos são a braquicefalia e dolicocefalia

Como malformações específicas da cabeça enquanto, deformidade do aparelho osteomuscular, temos destacada no CID 10ª Rev. : Assimetria facial (Q67.0); Deformidade facial por compressão (Q67.1); Dolicocefalia (Q67.2); Q67.3 Plagiocefalia. Deve-se distinguir a dolicocefalia e/ou braquicefalia enquanto variedades anatômicas do formato do crânio estabelecidas pela craniometria, disciplina da antropologia física. O tipo dolicocéfalo, por exemplo, corresponde ao tipo humano cuja largura do crânio tem quatro quintos (4/5) do comprimento.

Lista de malformações do sistema nervoso, da face ou cefálicas congênitas

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Bibliografia

  • Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson. Robbins & Cotran Patologia Humana (7ª ed). Madrid, ES, El Sevier. Google livros Dez. 2011
  • Smith, David W. . Síndromes de Malformações Congênitas. SP, Manole, 1985
  • Graham, John M.; Smith, David W. Smith's recognizable patterns of human deformation. USA, Sauders - Elsevier, 2007 Google Books Dez. 2011

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