A SERIES OF FIVE PATIENTS WITH FOVEAL HYPOPLASIA DEMONSTRATING GOOD VISUAL ACUITY.

BACKGROUND/PURPOSE: Foveal hypoplasia is associated with low vision in a variety of conditions. We report and show imaging studies for a series of five patients with foveal hypoplasia who had visual acuity ranging from 20/25 to 20/60.

METHODS: Observational case series.

PATIENTS: This series includes five patients aged 5 to 18 years who presented to the Greater Baltimore Medical Center, Hoover Low Vision Clinic. All patients showed a partial or complete absence of the fovea in both eyes on examination.

RESULTS: Eight eyes exhibited absence of foveal depression on spectral domain optical coherence tomography (Cirrus high-definition OCT; Carl Zeiss Meditec). Four eyes exhibited Grade 3 foveal hypoplasia, two eyes exhibited Grade 4 foveal hypoplasia, and two eyes exhibited Grade 1 foveal hypoplasia. For two eyes (one patient), the optical coherence tomography was no longer available for grading, but was interpreted as foveal hypoplasia in both eyes in the chart. Visual acuities ranged from 20/25 to 20/60. Only two of five patients showed signs of albinism.

CONCLUSION: Despite the presence of foveal hypoplasia, patients can retain a high level of visual acuity, which suggests that a foveal depression is not essential for the development of good visual acuity.