Clinical assessment, optic disk measurements, and visual-evoked potential in optic nerve hypoplasia.

Purpose: Because there is a broad spectrum of visual dysfunction in eyes with optic nerve hypoplasia, we determined whether any clinical observations, including optic disk measurements, could predict visual outcome in eyes with hypoplastic optic nerves.

Methods: We prospectively studied the eyes of 19 children (eight consecutive and 11 randomly selected), ages 7 to 92 months, in whom a diagnosis of optic nerve hypoplasia was made by an ophthalmologist. Clinical assessment included visual acuity, pupillary responses, and nystagmus. The ratio of the horizontal disk diameter to the disk-macula distance was calculated from fundus photographs. The amplitude and latency of the flash visual-evoked potential as well as the threshold response to pattern reversal checks were recorded.

Results: Visual acuity was associated with pupil reactivity, nystagmus, the ratio of the horizontal disk diameter to the disk-macula distance, pattern visual-evoked potential threshold, and flash visual-evoked potential amplitude (P < .001 for all parameters). All eyes with a ratio of the horizontal disk diameter to the disk-macula distance greater than .30 had good visual acuity, and all eyes with a ratio of the horizontal disk diameter to the disk-macula distance less than .30 had poor visual acuity. No eye with a ratio of the horizontal disk diameter to the disk-macula distance less than or equal to .15 had visual acuity better than light perception.

Conclusions: Visual-evoked potential and photogrammetric measurement of relative disk size can be used to approximate visual acuity in eyes with optic nerve hypoplasia. This finding could lead to a diagnostic criterion or a severity classification for optic nerve hypoplasia.