A 52-year-old woman presented to the emergency department with sudden painless loss of vision in her left eye.
She had no personal or family history of interest.
In the exploration, its best corrected visual acuity (BCVA) in the right eye (OD) was 0.9 and 0.6 in the left eye.
Intraocular pressure (IOP) and biomicroscopy (BMC) of the anterior pole showed no significant changes.
When viewing the eye fundus (FO), a brownish motto was observed, more prominent temporal to the fovea, which corresponded to the typical image of «fondo en skin» in the matrix.
Angioid striae (AS) and colloid bodies in both eyes (AO) were also observed.
In his OI, there were striking features of optic nerve drapes and a subfoveal greyish lesion in the sinus of an AS, elevated and bordered by hemorrhages, compatible with CNV.
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Therefore, a series of complementary tests were performed, appearing in the background autofluorescence (FA) of AO a diffuse hyperautofluorescent (hyper-FA) mottled hyperautofluorescent (hyper-FA) and irregular linear lesions.
In the OI, emphasis was placed on optic nerve and colloid bodies with intense hyper-FA.
Fluorescence angiography (FFA) revealed a hyper- and hypofluorescent layer due to alteration of the pigmentary epithelium (CNP) compatible with a hyperfluorescent lesion with late diffusion in the O.
Optical coherence tomography (OCT) showed a small rounded edge structure (ENVPR-coriocautery in BE, and in OI a cupulepiiform neuroprene lesion).
After the diagnosis was made, the patient was referred to the elastic pseudoxantoma unit of our hospital, where the disease was confirmed.
Treatment was initiated with monthly intravitreal injections of 0.05ml of ranibizumab (Lucentis) for 3 months in the OI, with measurement of the MAVC and OCT at one month after each injection.
Periodic control OCT was performed during a one-year follow-up period.
At 3 months there was a clear improvement of the clinical picture, as the AVM improved to 0.7, disappearing subretinal hemorrhages and decreasing the macular thickness on OCT.
