Enfermedad de Stargardt

También llamada degeneración macular juvenil.

Enfermedad hereditaria en la que predomina la pérdida de los conos y por tanto de la visión diurna. Entra dentro de un grupo de distrofias retinianas llamadas maculopatía en ojo de buey.

Clínicamente se caracteriza por una pérdida progresiva de agudeza visual, con mala visión de los colores. A veces asocia fotofobia.

En la exploración, la mácula tiene un aspecto característico como de metal golpeado cuando la enfermedad esta evolucionada, puede ser normal al principio.

  • Se hereda autosómica recesiva.
  • Suele comenzar entre la primera y segunda década.

El estudio patológico muestra una pérdida completa de epitelio pigmentario y fotorreceptores en la mácula.

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