Vitelliform maculopathy: Diverse etiologies originating from one common pathway

Vitelliform lesions (VLs) are associated with a wide array of macular disorders but are the result of one common pathway: retinal pigment epithelium (RPE) impairment and phagocytic dysfunction. VLs are defined by the accumulation of yellowish subretinal material. In the era of multimodal advanced re...

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Bibliographic Details
Published in:Survey of ophthalmology 2023-05, Vol.68 (3), p.361-379
Main Authors: Iovino, Claudio, Ramtohul, Prithvi, Au, Adrian, Romero-Morales, Veronica, Sadda, SriniVas, Freund, K Bailey, Sarraf, David
Format: Article
Language:English
Subjects:
Adult
Aged
Fluorescein Angiography - methods
Fundus Oculi
Humans
Hyperreflective subretinal material
Macular atrophy
Macular neovascularization
Retinal Pigment Epithelium - pathology
Tomography, Optical Coherence - methods
Vitelliform lesion
Vitelliform Macular Dystrophy - etiology
Vitelliform Macular Dystrophy - genetics
Vitelliform maculopathy
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Summary:Vitelliform lesions (VLs) are associated with a wide array of macular disorders but are the result of one common pathway: retinal pigment epithelium (RPE) impairment and phagocytic dysfunction. VLs are defined by the accumulation of yellowish subretinal material. In the era of multimodal advanced retinal imaging, VLs can be further characterized by subretinal hyperreflectivity with optical coherence tomography and hyperautofluorescence with fundus autofluorescence. VLs can be the result of genetic or acquired retinal diseases. In younger patients, VLs usually occur in the setting of Best disease. Additional genetic causes of VL include pattern dystrophy or adult-onset vitelliform macular dystrophy. In older patients, acquired VLs can be associated with a broad spectrum of etiologies, including tractional, paraneoplastic, toxic, and degenerative disorders. The main cause of visual morbidity in eyes with VLs is the onset of macular atrophy and macular neovascularization. Histopathological studies have provided new insights into the location, nature, and lifecycle of the vitelliform material comprised of melanosomes, lipofuscin, melanolipofuscin, and outer segment debris located between the RPE and photoreceptor layer. Impaired phagocytosis by the RPE cells is the unifying pathway leading to VL development. We discuss and summarize the nature, pathogenesis, multimodal imaging characteristics, etiologies, and natural course of vitelliform maculopathies.
ISSN:0039-6257
1879-3304
DOI:10.1016/j.survophthal.2023.01.009