| Stage Number | Stage Name | Fundus Appearance | OCT Findings | FAF Findings | Visual Acuity | Clinical Description |
|---|---|---|---|---|---|---|
| 1 | Previtelliform / Subclinical | Fundus may appear normal or show only subtle RPE irregularity; early macular change without classic yellow lesion (pqac-00000015, pqac-00000016, pqac-00000018, pqac-00000019) | Subtle OCT abnormalities; early disturbance at the photoreceptor–RPE interdigitation zone; heightened RPE–outer segment reflectance may be present (pqac-00000016, pqac-00000018) | Near-infrared autofluorescence may help identify early disease; FAF can be minimal or only subtly abnormal before obvious vitelliform deposition (pqac-00000016, pqac-00000018) | Usually normal or near-normal vision (pqac-00000019) | Earliest clinically recognized stage; RPE-photoreceptor interaction is already abnormal, but overt lesion formation may not yet be visible on ophthalmoscopy (pqac-00000016, pqac-00000018) |
| 2 | Vitelliform | Classic yellow, dome-shaped “egg-yolk” lesion in the macula; central vitelliform material beneath the neurosensory retina (pqac-00000015, pqac-00000017, pqac-00000018, pqac-00000019) | Dome-shaped subretinal hyperreflective material/elevation; raised ellipsoid/interdigitation zones; subretinal deposit corresponding to vitelliform lesion (pqac-00000018, pqac-00000020) | Marked hyperautofluorescence corresponding to vitelliform material (pqac-00000016, pqac-00000020) | Mild visual loss, though many patients still retain good central acuity; symptoms may include photophobia, metamorphopsia, or nyctalopia (pqac-00000019) | Hallmark stage of BVMD with lipofuscin-rich/unphagocytosed outer segment material accumulation in the macula (pqac-00000015, pqac-00000016) |
| 3 | Pseudohypopyon | Yellow material gravitates inferiorly within the lesion, creating a fluid level / layered appearance (pqac-00000016, pqac-00000018, pqac-00000019) | Layering of vitelliform material within subretinal space; inferior pooling of hyperreflective material (pqac-00000018) | Persistent hyperautofluorescence, often redistributed according to layered material (pqac-00000018, pqac-00000019) | Variable; often still relatively preserved but may begin to decline (pqac-00000016, pqac-00000019) | Transitional stage in which accumulated material separates and settles inferiorly, producing the “pseudohypopyon” appearance (pqac-00000016, pqac-00000018) |
| 4 | Vitelliruptive / “Scrambled-egg” | Fragmented, irregular, clumped yellow deposits with “scrambled-egg” appearance; lesion disintegration (pqac-00000016, pqac-00000017, pqac-00000019, pqac-00000021) | Clumped/disrupted hyperreflective material; increasing outer retinal disorganization, ONL thinning, and ellipsoid zone disruption (pqac-00000015, pqac-00000018) | Mixed or irregular autofluorescence as material fragments and redistributes (pqac-00000018) | Visual acuity often declines substantially compared with earlier stages (pqac-00000019) | Represents breakdown/resorption of the vitelliform lesion with increasing photoreceptor dysfunction and structural damage (pqac-00000015, pqac-00000019) |
| 5 | Atrophic / Fibroatrophic (Atrophic/Fibrotic) | Macular atrophy, fibrosis, or scar-like end-stage lesion; may be complicated by choroidal neovascularization (pqac-00000015, pqac-00000016, pqac-00000018, pqac-00000019) | Loss of outer retinal layers with photoreceptor loss, RPE atrophy, and advanced structural collapse; possible fibroatrophic change (pqac-00000016, pqac-00000018) | Reduced/heterogeneous autofluorescence in atrophic areas; prior hyperautofluorescent material may be lost as atrophy advances (pqac-00000015, pqac-00000018) | Severe, often irreversible visual loss in advanced disease (pqac-00000019) | End stage characterized by RPE death, photoreceptor loss, and possible CNV or fibrosis; central vision impairment becomes most pronounced (pqac-00000016, pqac-00000019) |


*Table: This table summarizes the five classic clinical stages of Best vitelliform macular dystrophy, integrating fundus, OCT, FAF, and functional features. It is useful for disease characterization, differential diagnosis, and structuring natural history or trial-readiness annotations.*