Enfermedad de Norrie

De herencia ligada al sexo, se caracteriza por una malformación retiniana con ceguera total debida a desprendimiento de retina en los primeros meses de vida.

Se produce una hipoplasia de las capas retinianas internas asociada a proliferación vascular que causa el desprendimiento de retina. Puede tener un aspecto de masa pseudotumoral grisácea detrás del cristalino (llamada pseudotumor o pseudo glioma. Hay una proliferación excesiva del epitelio pigmentario. Con el tiempo se desarrollan cataratas, opacidad de cornea y ptisis bulbi hacia el final de la primera década de vida.

Las manifestaciones extraoculares no son siempre constantes e incluyen retraso mental, sordera neurosensorial progresiva, microcefalia, criptorquidia, estatura corta y cara característica.

  • Distrofias maculares
  • Enfermedad de Norrie
  • Degeneración macular
  • PSEUDOGLIOMA SYNDROMES

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