Table 2. [Bestrophinopathies: Frequency of Phenotypes]. - GeneReviews®

Phenotype	Ophthalmologic Findings	Comment
BVMD	"Egg yolk"-like vitelliform lesion	Multiple vitelliform lesions Atrophic scar	Choroidal neovascularization Unilateral vitelliform lesions	Vitelliform lesion progressing through stages of heterogeneous "scramble egg" to pseudohypopyon lesion to atrophic scar Visual acuity can progress from 20/20 to <20/200.
AVMD	Bilateral, small, circular vitelliform lesion	Atrophic scar	Choroidal neovascularization	Lesions ↑ & then ↓ in size, becoming an atrophic area. Visual acuity 20/40 to 20/200
ADVIRC	Peripheral chorioretinal atrophy w/white retinal opacities	Hyperopia Shallow anterior chamber/angle closure Microcornea Fibrillar vitreous condensation	Breakdown of blood-retinal barrier w/retinal neovascularization
ARB	White subretinal deposits Subretinal fluid Hyperopia	Subretinal fibrous scars Shallow anterior chamber/angle closure Glaucoma Cystoid macular edema	Choroidal neovascularization	Subretinal deposits w/subretinal fluid in early stages progressing to subretinal fibrosis Visual acuity can progress from 20/20 to <20/200.

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Phenotype	Ophthalmologic Findings			Comment
Phenotype	Most Common	Common	Infrequent	Comment
BVMD	"Egg yolk"-like vitelliform lesion	Multiple vitelliform lesions Atrophic scar	Choroidal neovascularization Unilateral vitelliform lesions	Vitelliform lesion progressing through stages of heterogeneous "scramble egg" to pseudohypopyon lesion to atrophic scar Visual acuity can progress from 20/20 to <20/200.
AVMD	Bilateral, small, circular vitelliform lesion	Atrophic scar	Choroidal neovascularization	Lesions ↑ & then ↓ in size, becoming an atrophic area. Visual acuity 20/40 to 20/200
ADVIRC	Peripheral chorioretinal atrophy w/white retinal opacities	Hyperopia Shallow anterior chamber/angle closure Microcornea Fibrillar vitreous condensation	Breakdown of blood-retinal barrier w/retinal neovascularization
ARB	White subretinal deposits Subretinal fluid Hyperopia	Subretinal fibrous scars Shallow anterior chamber/angle closure Glaucoma Cystoid macular edema	Choroidal neovascularization	Subretinal deposits w/subretinal fluid in early stages progressing to subretinal fibrosis Visual acuity can progress from 20/20 to <20/200.