Figure 2. Clinical evaluation of the female sibling from the Spanish family. Color fundus appearance photographs (A, B). The right eye shows a typical pseudohypopyon stage with a deposit of yellowish vitelliform material in the lower half of the macular lesion. The left eye shows a characteristic atrophic stage. Fluorescein angiography (AGF) shows a hyperfluorescence window defect due to atrophy of the retinal pigment epithelium (RPE; C, D), and hypofluorescence due to a deposit of lipofuscin (C). Vitelliform lesions appear as areas of hyperautofluorescence (E) and hypoautofluorescence (F) fundus autofluorescence (FAF). Optical coherence tomography (OCT) section of the pseudohypopyon lesion shows a hyperreflective material beneath the retina in the right eye (G) and a neurosensory retinal detachment in the left eye (H). OD: Right eye, OS: Left eye.