Unilateral best vitelliform macular dystrophy- a case series.

PURPOSE: To report 2 cases with unilateral Best vitelliform macular dystrophy (VMD) and describe their multimodal investigations findings.

METHODS

Two patients in their fifties who were misdiagnosed as central serous chorioretinopathy were carefully evaluated using multimodal imaging and electrooculography (EOG) investigations.

RESULTS

Both patients showed neurosensory elevation at the macula in one eye only leading to reduced vision. The optical coherence tomography showed subretinal hyperreflective material lining the undersurface of the elevated retina with thinning of the overlying photoreceptor layer. There was no choroidal thickening or pachy vessels. There was no leakage seen on fluorescein angiography nor any choroidal hyperpermeability on indocyanine green angiography. There was no choroidal neovascular membrane noted. The left eye was clinically unaffected for both. The EOG showed a reduced light peak to dark trough (LP:DT) ratio in both the eyes of these patients confirming the diagnosis of Best VMD. No change was seen in the eye condition over 2 years.

CONCLUSIONS

Best VMD can present in unilateral fashion in rare instances. EOG can be confirmatory along with genetic testing. It can be misdiagnosed as CSCR where multimodal imaging and EOG can help differentiate it as Best VMD.