J Korean Ophthalmol Soc.  2021 Mar;62(3):337-344. 10.3341/jkos.2021.62.3.337.

The Characteristics and the Clinical Courses of Best Disease in Korean Patients

Affiliations
  • 1Kim’s Eye Hospital, Seoul, Korea

Abstract

Purpose
To evaluate the clinical characteristics and courses of Best disease in Korean patients.
Methods
We retrospectively reviewed the medical records of patients with Best disease followed-up for more than 12 months after diagnosis. The disease stage and best-corrected visual acuity (BCVA) at diagnosis, and stage progression during follow-up, were evaluated. The BCVA at diagnosis was compared to that at the final follow-up. The BCVA changes were compared between those evidencing stage progression and not.
Results
A total of 16 patients (32 eyes) were enrolled. The mean follow-up period was 52.1 ± 31.8 months. At diagnosis, three eyes had stage 1, nine stage 2, seven stage 3, 11 stage 4, and two stage 5 disease. During follow-up, stage progression was observed in nine eyes (28.1%). The mean logarithm of the minimal angle of resolution (logMAR) BCVA significantly worsened from 0.19 ± 0.19 at baseline to 0.30 ± 0.27 at the final follow-up (p = 0.004). In six eyes (18.8%), a deterioration of logMAR 0.2 or greater was noted. The extent of deterioration was greater in the nine eyes (0.25 ± 0.26) exhibiting stage progression than in the 24 eyes (0.05 ± 0.08) lacking such progression (p = 0.001).
Conclusions
The visual acuity of Best disease patients was relatively good at diagnosis. However, stage progression and visual deterioration were noted in some patients. Stage progression was associated with a poor visual prognosis.

Keyword

Best disease, Characteristics, Outcome, Stage progression, Vitelliform macular dystrophy
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