Fundus examination of the left eye of a healthy 19-year-old woman displayed continuous and segmental white arterioles including the macular branches (A, arrowheads). Visual acuity, perimetry (B), and fluorescein angiography including arteriovenous (C) and late venous (D) phases were normal and remained unchanged at 1 year. Fundus findings also remained identical. Lipemia retinalis (normal total cholesterol and triglycerides), inflammation (normal C-reactive protein and negative vasculitis serology), infectious diseases (negative borreliosis, Epstein Barr virus, varicella zoster virus, and herpes simplex virus 1 and 2 serology), arteriosclerosis, emboli, and hypercalcemia were ruled out.

A 12-year-old previously healthy girl presented with painless loss of vision of the left eye. Visual acuity was 20/20 in the right eye, and hand motion with an afferent pupillary defect in the left eye. Optic disc edema, macular edema, diffuse retinal hemorrhages, and retinal pallor (A) were noted. Fluorescein angiography demonstrated lack of both arterial and venous filling (B). Laboratory test results supported a diagnosis of systemic lupus erythematosus with positive antinuclear antibody, double-stranded DNA, antiribonuclear protein, and anti-Smith antibodies.

A 33-year-old otherwise healthy man presented with defective vision in his left eye with a best-corrected visual acuity of 20/32. The left optic disc had 2 pits, 1 at each edge, with maculopathy along with a retinochoroidal coloboma (color fundus photography; A, blue arrows indicate nasal and temporal optic disc pit, red arrow indicates serous retinal detachment, and yellow arrow indicates retinochoroidal coloboma; B, blue arrow indicates red-free image of the optic disc pit). OCT showed 2 pits with maculopathy (C, blue arrow indicates the temporal pit with maculopathy; D, blue arrow indicates the nasal pit).