Pseudoxanthoma elasticum is a rare autosomal recessive genetic disorder, which prevalence varies between 1/25,000 and 1/100,000 [4], characterized by calcification of elastic tissues, affecting various tissues, cutaneous, vascular, digestive and ophthalmological (angioid streaks). Angioid streaks are present in 85% of patients with elastic pseudoxanthoma [5]. On the fundus, angioid streaks; appearing as darksirregulars lines, starting from the optic disc, interconnected by a ring surrounding the optic disc, with a radial arrangement, and the‘’Peau d’orange’’ appearance in the temporal retina, are characteristics of pseudoxanthoma elasticum, such is the case of our patients.On fluorescein angiography, the angioid streaks appear non-homogeneous and hyperfluorescent throughout the entire sequence, without diffusion [6].

As ophthalmic lesions associated with pseudoxanthoma elasticum, angioid streaks develop in most patients, and the risk of neovascularization increases with age [7]. The frequency of non-exudative macular neovascularization is 33.3%[8]. Bilateralization is common (42-60%), asis the case in our two patients.

Preserving visual function and avoiding complications are the main goals of treatment.

Angioid streaks remain asymptomatic in the absence of neovascularization, and require no treatment other than regular ophthalmological monitoring [9]. However, prevention is recommended, in particular the avoidance of eye trauma [9,10].

Previously, many therapies have been proposed but without any visual benefit in the majority of cases. Argon laser photocoagulation has long been proposed for the treatment of extrafoveolar neovessels,with disappointing results and frequent recurrences (88% at 5 years) [9], and the retreatment was the norm [9,11]. Photodynamic therapy (PDT) has also been used to treat extra-, juxta- and retrofoveolar neovessels, but often without significant functional results [9,11,12].

Based on the results and efficacy of intravitreal injections of anti-VEGF, in the treatment of new vessels complicating age-related macular degeneration and high myopia [13,14], publications regarding the treatment of newvessels complicating angioid streaks by anti- VEGF IVTs, appeared in 2006 [9].

Recently, other studies have shown stabilization or improvement of visual acuity after intravitreen injection of anti-VEGF. Finger et al. reported in their prospective study of seven patients with PXE with CNV, who received IVT of anti-VEGF, an improvement in visual acuity, regression of CNV on AGF and a decrease in foveolar thickness on OCT, without recurrence with a follow-up of three months [3]. In their study of 35 eyes, Mimoun et al. found, an improvement in visual acuity in 85.7% of eyes, a stabilization or decrease in macular

thickness in 51.5% of eyes, and absence of activity on AGF in 65.7% of eyes in the six months following the last IVT [12]. Weigand et al. revealed, in their series, a regression of centromacular thickness averaging 67m after IVT, with complete resolution of subretinal fluid, with the exception of a single patient [15].Sawa et al. evaluated the efficacy of anti-VEGF IVTs in a retrospective study involving 15 eyes. Two-line VA improvement was noted in 33% of eyes, stabilization in 54% and worsening in 13% of cases. Angiographic follow-up showed the absence of neovascular reactivation in 67% of eyes [16].

The natural evolution of neovessels complicating the angioid streaks is extremely severe, and anti-VEGF IVTs have shown their effectiveness in our patient, which is consistent with the results of various studies.