Fig. 4

A Top left. The color fundus photo and FFA of the right eye of a 40-year-old female patient with angioid streaks and PXE. Note the linear streaks with RPE hyperpigmentation radiating centrifugally from the ONH margins (arrows). One of those streaks has bisected the fovea. Note the characteristic peau d’orange appearance of the fundus. FFA revealed transmission fluorescence of the streak bisecting the fovea. Note the subfoveal well-circumscribed mild but progressive leakage adjacent to the angioid streak that indicated a subfoveal CNV. Top right. SS-OCT of the macular area in a radial scan mode shows a subfoveal hyperreflective amorphous lesion with underlying conspicuous dehiscence of the RPE-Bruch’s complex (arrow) and choroidal excavation. Bottom. SS-OCTA image of the outer retina in a 3 × 3 mm field and the corresponding flow density map. Note the hyperintense signal of an active neovascular network with characteristic vascular looping and anastomosis and high flow density in the corresponding flow density map. B Top right. SS-OCT of the macular area in a radial scan mode 7 months after the patient received a single IVA injection. Note the reduction in the size of the previously noted subfoveal lesion. Bottom. SS-OCTA image of the outer retina in a 3 × 3 mm field and the corresponding flow density map. Note signs of inactivity in the form of the marked reduction of the previously noted hyperintense signal and disappearance of anastomosis and looping with the development of linear vascular streaks (dead-tree) and the low vascular flow in the flow density map