The importance of fluorescein angiography in diagnosing pattern dystrophies of the retinal pigment epithelium.
We describe three different types of pattern dystrophies in the retinal pigment epithelium: annular dystrophy, butterfly-shaped dystrophy with features of cone dystrophy, and butterfly dystrophy. In all the cases, fluorescein angiography was a good method to evaluate the morphology and range of macular changes. The butterfly-shaped lesions were not always easily visible with the ophthalmoscope, therefore, the fluorescein angiography was diagnostic. We also examined 17 relatives of these patients to discover the mode of inheritance in these diseases. The annular dystrophy seemed to be recessively inherited or a possible sporadic case, as was the unusual pattern dystrophy with features of cone dystrophy. The dominant heredity was found in the typical cases of butterfly-pattern dystrophy.
Department of Ophthalmology, University Hospital of Kuopio, Finland.
K
K
Tuppurainen
M
M
Mäntyjärvi
D000293:Adolescent; D005260:Female; D005451:Fluorescein Angiography / Q000379:methods*; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D012162:Retinal Degeneration / Q000175:diagnosis* / Q000235:genetics