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+A 58-year-old man presented with sudden loss of vision in his left eye. The patient had been under follow-up by Internal Medicine and Dermatology for 12 years due to PXE.
+His MAVC in the OD was 0.6 and in the OI 0.4. No alterations were found in IOP or anterior pole CMB.
+The FO examination revealed bilateral AEs. In the OD, there was a raised yellowish-grey gliotic lesion and, in the OI, a pigmented triradiate image bordered by orange areas in the macular area, together with a small juxtafoveal lesion, somewhat greyish and slightly raised, surrounded by abundant subretinal haemorrhages, mainly nasal. Suspicion of CNV membrane within a "reticular pattern" retinal dystrophy led us to perform an AFG, where a gliotic eschar was observed in the OD and, in the OI, a hypofluorescent image in the form of a reticulum, in the upper area of which there was a hyperfluorescent lesion with lacy borders that increased in intensity and size in the late stages, thus confirming the previous suspicion. OCT corroborated the diagnosis, showing a discrete elevation of the EPR-choriocapillary band.
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+The FA of the LA revealed a curious image, as the dystrophy in pattern appeared as the negative of the angiographic image; in other words, the reticulum, which angiographically appeared hypofluorescent, was intensely hyper-FA in FA due to the alteration of the EPR at that level.
+In this case, the same treatment as in the previous patient was applied according to the protocol described above. A higher VA was achieved, reaching 0.7. Progressive OCT scans showed a progressive decrease in macular thickness and the control AFG showed an improvement in the condition.
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