[Vitelliform degeneration of the macula].
Macular vitelliform degeneration is an hereditary affection, with autosomal dominan transmission. It is probably congenital. Three phases in the evolution of lesions: I The "fried eggs" aspect. II Various ophthalmologic aspects: mixed egg, pseudo-hypopion, retractile form. During these first two phases, the yellowish vitelline substance only may extend in macular region, especially downards. III The atrophic phase. Visula acuity remains strangely good, especially during the first two phases. Nevertheless, the visual function study, especially colour vision and profile perimetry, elecites a precocious suffereance of macular cones, associated to fluorographic alteration of pigmentary epithelium in phases II and III. Actually, these lesions are mostly situated in perifoveolar area, respecting the foveola, which explains conservation of good vision. The electro-oculogram is deeply modified in most cases. The pathogenesis of this affection remains unexplained, but the evolution of lesions both in pigmentary epithelium and neuro-epithelium enables to think that vitellin substance lays between these two layers.
D000328:Adult; D001284:Atrophy; D002675:Child, Preschool; D003118:Color Perception; D005071:Evoked Potentials; D005260:Female; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008266:Macula Lutea / Q000473:pathology; D008268:Macular Degeneration / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D012162:Retinal Degeneration / Q000473:pathology*; D014792:Visual Acuity; D014794:Visual Fields
La dégénérescence vitelliforme de la macula