A variant of central areolar choroidal dystrophy.
This article describes the variable ophthalmoscopic features of a macular disorder in five generations of one family. This disease shares similarities with central areolar choroidal dystrophy and other progressive dominant macular dystrophies but demonstrates significant differences that required further consideration. The milder affected individuals had confluent hyperfluorescence around the macular area while the more severe lesions consisted of marked chorioretinal atrophy of the macula. The visual fields revealed a central scotoma. The disorder was transmitted as an autosomal dominant trait.
Department of Ophthalmology, East Surrey Hospital, UK.
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Chopdar
D000328:Adult; D017668:Age of Onset; D000368:Aged; D015862:Choroid Diseases / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D003118:Color Perception; D003241:Consanguinity; D004594:Electrophysiology; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D005787:Gene Frequency; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D006113:United Kingdom; D014792:Visual Acuity; D014794:Visual Fields