Retinosis pigmentaria

Grupo de enfermedades hereditarias de la retina caracterizadas: Clínicamente por mala visión nocturna, constricción progresiva del campo visual, pérdida progresiva de agudeza visual. En la exploración el fondo de ojo muestra lesiones características llamadas en espículas óseas, hay atenuación de los vasos sanguíneos, palidez papilar, edema macular. La herencia puede ser autosómica dominante, recesiva o ligada al sexo. Hay casos esporádicos no hereditarios. Las formas dominantes tienen mejor pronóstico. Suele comenzar entre la primera a tercera década de la vida. Progresa lentamente, puede cursar en brotes de empeoramiento. La RP puede ser muy variable en su presentación tanto de los síntomas como del aspecto del fondo de ojo. Hay diferentes formas clínicas como la RP sine pigmento, La RP unilateral, La RP inversa, RP pericentral… Puede igualmente encontrarse asociada a otras patologías congénitas dentro de cuadros sindrómicos generales. El hallazgo patológico es la degeneración progresiva de fotorreceptores inicialmente bastones, posteriormente también conos y de las capas internas de la retina junto con el epitelio pigmentario. En estadios finales hay una proliferación glial en la retina y nervio óptico con hialinización de los vasos.

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