Article
Adult onset vitelliform macular dystrophy (AVMD): case presentation
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Published: | September 22, 2004 |
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Outline
Text
Objective
Adult onset vitelliform macular dystrophy (AVMD) was first reported by Gass in 1974 and was also called peculiar foveomacular dystrophy of Gass. Is characterized by a yellow, solitary, round or oval subretinal macular lesion that resembles juvenile onset vitelliform macular dystrophy and Best's disease. The authors present one case (two eyes) of this rare macular dystrophy.
Methods
Adult onset vitelliform macular dystrophy (AVMD) was first reported by Gass in 1974 and was also called peculiar foveomacular dystrophy of Gass. Is characterized by a yellow, solitary, round or oval subretinal macular lesion that resembles juvenile onset vitelliform macular dystrophy and Best's disease. The authors present one case (two eyes) of this rare macular dystrophy.
Results
Visual acuity of R.E. was 0,1, of L.E. was 1, 0 with metamorphopsia in Amsler-test. In ophthalmoscopy in LE typical a vitelliform-like macular lesion was seen, in RE faded vitelliform changes and the RPE atrophy around it. FA of L.E. showed typical hypofluorescent spot in the center of the macula with small hyperfluorescent spots around it, called corona sign. Foveal average thickness diagnosed by RTA was about 175 um in RA and 200 um in LA, what was over the normal value.The OCT, done in other Clinic, showed the normal fovea thickness in R.A. and changes of RPE in L.A.T. he ERG scotopic and photopic nad EOG results were normal. The patient had no family history of macular dystrophy. Also posterior uveitis investigations including toxoplasma and toxocara titers were within normal limits. The was also no history of smoking and toxic medications.
Conclusions
The ophthalmoscopic appearance of the AVMD is very similar to that in Best's disease. Additional ophthalmological investigations should be performed for diagnosis of AVMD.This dystrophy is probably inherited as an autosomal dominant trait. Genetic counseling and molecular genetic analysis should be performed in the future.