Visual outcome following subretinal hemorrhage in Best disease

To review cases of Best disease associated with subretinal hemorrhage to better understand their long-term visual prognosis.

Patients were identified through the photographic file database at the University of Iowa. Seventy-eight files of patients with clinical evidence of Best disease were reviewed and 12 patients (14 eyes) were identified with subretinal hemorrhage. The visual acuity and clinical course were reviewed in all of these patients when possible. Three patients demonstrated subretinal hemorrhage on their last follow-up visit. Nine patients (11 eyes) were followed through to resolution of subretinal hemorrhage. Eight patients were screened on the VMD2 gene and all were found to have disease-causing sequence variations.

All patients noted visual loss at presentation with subretinal hemorrhage (median 20/100; range 20/30-20/400). The median final visual acuity in the 11 eyes with follow-up was 20/50 (20/16-20/400 range). Ten of 11 eyes demonstrated improvement of vision with 9/11 having a final visual acuity of 20/50 or better.

The natural history of patients with Best disease with subretinal hemorrhage and moderate visual loss is relatively good. The presence of subretinal hemorrhage in Best disease may be related to mild, incidental trauma.