Fig. 1

Images from before treatment for ocular ischemic syndrome with retinal vascular abnormalities due to unilateral ocular artery and internal carotid artery stenosis in a pediatric NF1 patient. Color fundus photographs showed poor retinal color and severe retinal vascular abnormalities in the right eye. Vascular abnormalities of the retina formed a complex vascular plexus; some of the abnormalities had corkscrew-like shapes. The foveal reflex had disappeared and optic disc pallor was visible (a). Ultra-widefield fluorescein angiography revealed multiple sites of neovascularization, from the posterior pole to the equator, and a large non-perfusion area was observed in the peripheral retina in the right eye (b). Optical coherence tomography angiography (OCTA) en-face images showed retinal vascular abnormalities in the superficial and deep retinal layers in the right eye (c: superficial retinal layer, d; deep retinal layer). In OCTA B-scan images, thickening of the inner retina of the right eye was apparent, and there was a slight difference in the retinal surface and deep blood flow signals between the left and right eyes (e: right eye, k: left eye). Some vascular abnormalities on the temporal side of the macula were also observed as having traffic in the superficial and deep retinal layers (c–e). Furthermore, deformation of the foveal avascular zone (FAZ) was observed in the right eye and the area of the FAZ was 63.2% larger in the right eye than in the left eye (c: right eye, i: left eye). Laser speckle flowgraphy (LSFG) imaging showed a marked decrease in chorioretinal blood flow compared with the left eye (f: right eye, l: left eye). In the left eye, no abnormal findings were observed throughout the course of observation (g–l)