Bietti Crystalline Retinopathy: Report of Retinal Crystal Deposition in Male Adolescent Siblings

Figure 1. Color fundus photographs of patient 1 (A; the proband) and patient 2 (B) showing multiple crystalline deposits that are more numerous at the posterior pole and peripapillary region than at the retinal periphery, which is relatively spared. The corresponding fundus autofluorescence images of patient 2 (C) are also shown.

Figure 2. Spectral-domain optical coherence tomographic (SD-OCT) and infrared images of patient 1 (A; the right [upper] and left [lower] eyes) and patient 2 (B; the right [upper] and left [lower] eyes). The SD-OCT images show hyperreflective lesions scattered throughout the retina that do not always appear to coincide with the crystals seen on infrared imaging.

Figure 3. Normal full-field electroretinograms (ERGs) and pattern ERGs (PERGs) from the right and left eyes of the proband and representative normal traces (bottom row). The ERGs were recorded under dark-adapted (DA) and light-adapted (LA) conditions according to the published standards of the International Society for Clinical Electrophysiology of Vision. The DA ERGs are shown for white flash strengths of 0.01 and 11.0 candela (cd) s m⁻². Standard LA full-field ERGs are shown for a flash intensity of 3.0 cd s m⁻² (mean [SD], 30 [2] Hz). The PERGs are recorded to an alternating checkerboard. The broken lines replace eye movement artifacts.