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Instituto Nacional de Neurología y Neurocirugía

2013, Número 2

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Arch Neurocien 2013; 18 (2)


Síndrome de Dandy-Walker

Figueroa-Ángel V, Reyes-Moreno I, García-López R, Rodríguez-Aceves C, Martínez-Muñiz I

Idioma: Español
Referencias bibliográficas: 73
Paginas: 92-98
Archivo PDF: 200.31 Kb.


RESUMEN

La asociación de dilatación quística del cuarto ventrículo, diversos grados de alteración del vermix cerebeloso e hidrocefalia supratentorial, fue descrita por primera vez en 1887 por Sutton y confirmada en 1914 por Dandy y Blackfan. La definición como síndrome, criterios diagnósticos y nomenclatura siguen siendo un tema de permanente debate entre los autores. La etiopatogenia parece basarse principalmente en un desarrollo anormal de las vías de comunicación del líquido cefaloraquídeo. Se ha sugerido predisposición genética; asimismo, se le ha asociado a diversas malformaciones y alteraciones cromosómicas. Las manifestaciones clínicas son muy variadas y están en relación directa con las alteraciones anatómicas. Al nacimiento la mayoría de los afectados se presentan asintomáticos o con ligeras manifestaciones, siendo la macrocefalia y evidencia de presión intracraneana elevada lo que domina el cuadro clínico. El diagnóstico se puede llevar a cabo durante la etapa prenatal mediante un estudio ultrasonográfico, o posnatal, de forma clínica aunada a estudios de imagen tales como tomografía computada y resonancia magnética. El tratamiento definitivo tiene amplia variabilidad, se realiza cuando los pacientes presentan manifestaciones de hidrocefalia sintomática.


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