Visual acuity in patients with best vitelliform macular dystrophy.

ABSTRACT

PURPOSE: Forty-seven patients with Best vitelliform macular dystrophy were evaluated in a cross-sectional fashion for visual acuity loss with age. METHODS: The authors assessed only patients who had at least one eye with a recognizable phenotype of Best vitelliform macular dystrophy. Patients with absent foveal changes or with only minimal foveal pigment mottling and hypopigmentation in each eye were excluded. RESULTS: A significant difference was noted between the visual acuities of the two eyes of the patients (2 lines or greater in the majority [64%] of patients). Nevertheless, for both eyes a significant correlation was noted between patient age and visual acuity, with older patients tending to have worse visual acuities. In the eyes with the best visual acuity, the majority of patients younger than 40 years of age (76%) had a visual acuity of 20/40 or better. In patients older than 30 years of age, a substantial percentage (74%) had a visual acuity of 20/100 or worse in at least one eye. CONCLUSION: The authors' findings indicate that although patients with Best vitelliform macular dystrophy who show characteristic macular lesions may retain good visual acuity in at least one eye, an appreciable number can lose substantial visual acuity, at least monocularly. In this population, no patient older than 50 years of age fulfilled the visual acuity criterion of 20/40 in at least one eye, the requirement in most states for an unrestricted driver's license, and only 20% of patients older than 40 years of age fulfilled this visual acuity criterion.