Bilateral Keratoconus in a Patient with Isolated Foveal Hypoplasia

We present a 29-year-old woman complaining of low visual acuity since childhood being deteriorated in the past six months. Ophthalmic examination revealed lowamplitude, jerky horizontal nystagmus in both eyes that worsened in end-gaze. The manifest refraction was –3.00 –9.5 × 20° and –2.5 –9.00 × 150° in her right and left eyes, respectively. The best spectacle-corrected visual acuity (BSCVA) was 20/70 in both eyes. In both eyes, BCVA with rigid gas permeable lenses was 20/50. Direct and consensual pupillary light reflexes were within normal limits and no relative afferent pupillary defect was detected. Color vision tested by Ishihara’s color plates revealed no deficits. Slit lamp examination showed mild paracentral corneal thinning and bulging, Fleischer’s ring, and Vogt’s striae in both eyes. Iris examination revealed no sign of transillumination. Intraocular pressure measured by Goldmann applanation tonometry was 15 mmHg in both eyes. Dilated fundus examination revealed generalized chorioretinal atrophy and no foveal reflex and no macular yellow hue. Retinal capillaries at the macular area showed mild irregularity. Both optic discs were pink and had sharp margins.