Figure 1. Patient V.5 (the first index case of the family). A: Mosaic multifield fundus photograph of the patient’s right eye, showing an avascular vitreous veil, approximately 2–3 disc diameters anterior to the inferior temporal retinal vein, with patches of chorioretinal atrophy, retinal pigmentary, and exudative lesions. B: Exudative yellow lesions with telangiectatic Coats-like neovascularization are seen in the retinal periphery. C: Fluorescein angiogram of the right eye of patient V.5 showing the superior pole and superior nasal and inferior temporal periphery. A mildly titled disc is seen. The retinal vasculature appears normal at the posterior pole and in the superior nasal periphery, and pigment clumping is present in the inferior temporal field. D: Fluorescein angiogram of the right eye of patient V.5 showing pigment clumping parallel to the ora in the inferior nasal periphery. Peripheral vascular leakage is seen as diffuse areas of hyperfluorescence. E: Slit-lamp photograph of the left eye of patient V.5 showing iris–lens synechiae from the 7:00 to the 10:00 position. F: OCT imaging of the right eye of patient V.5 showing a dense posterior hyaloid forming a bridge over the foveal pit.