In vivo microscopy of Best's Vitelliform Macular Dystrophy: optical coherence tomography study of combined stage III...

A 35-year-old man presented with decreased vision in both eyes. Fundus biomicroscopy of the right eye revealed a pseudohypopyon along with an area of hyperpigmentation and scarring at the macula (combined stage III and IVb Best's Vitelliform Macular Dystrophy [BVMD]). The left eye showed the presence of a pseudohypopyon along with macular atrophy (combined stage III and IVa). On optical coherence tomography both eyes had a neurosensory retinal detachment with an optically clear subretinal zone, an accumulation of material between neurosensory retina and the retinal pigment epithelium corresponding to the pseudohypopyon and subfoveal photoreceptor atrophy. We document on optical coherence tomography the transition of BVMD from stage III to IV (in the form of the combined stage). These new findings may be considered unique for this stage of BVMD, and would help in a further understanding of the disease as it passes through its various stages.