Publicado May 6, 2022



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Luis Eduardo Nieto Ramírez https://orcid.org/0000-0002-7848-0636

Sarita Mejia Jaramillo https://orcid.org/0000-0002-8049-4375

Tatiana Camayo Vásquez https://orcid.org/0000-0002-6085-8290

Carolina Téllez Hernández https://orcid.org/0000-0002-7689-494X

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Resumen

El síndrome de Möbius (SM) hace parte de un grupo de desórdenes genéticos conocidos como desordenes congénitos de denervación craneal (CCDD). Se trata de una parálisis oculofacial congénita infrecuente con pocos casos reportados desde su primera descripción por Von-Graefe en 1880 y Moebius en 1888.  La prevalencia del síndrome de Möbius se estima que es entre 1-2 por cada 10.000 nacimientos, afectando por igual a ambos sexos. Se caracteriza por compromiso de múltiples nervios craneales, más frecuentemente el VI y VII par. Entre los signos y síntomas de mayor relevancia están la inhabilidad para realizar expresiones faciales, y compromiso de la mirada conjugada. Múltiples posibles causales han sido estudiadas, pero no se ha determinado una etiología específica, entre estas causas están los trastornos vasculares secundarios a medicamentos, tóxicos, fenómenos de la actividad uterina, así como la teoría familiar genética de herencia autosómica dominante. Es frecuente que este síndrome curse con malformaciones extracraneales en particular de los miembros superiores. El diagnóstico se hace generalmente en la infancia, es principalmente clínico, sin embargo, la confirmación imagenológica es necesaria. Aunque la sintomatología no es progresiva es indispensable que el abordaje sea multidisciplinario incluyendo valoración inicial por Neurología, Genética, manejo quirúrgico por Cirugía Plástica, Terapia Física, y Rehabilitación neurológica. 






Keywords

mobius syndrome, moebius syndrome, congenital facial nerve palsy, congenital facial paralysis, congenital cranial dysinnervation disorderssíndrome de Möbius, síndrome de Moebius, parálisis nervio facial congénita, parálisis facial congénita, desordenes de denervación craneal congénita

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Cómo citar
Nieto Ramírez, L. E., Mejia Jaramillo, S., Camayo Vásquez, T., & Téllez Hernández, C. (2022). Manifestaciones clínicas del Síndrome de Möbius: scoping review. Universitas Medica, 63(2). https://doi.org/10.11144/Javeriana.umed63-2.mobi
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