gms | German Medical Science

102. Jahrestagung der DOG

Deutsche Ophthalmologische Gesellschaft e. V.

23. bis 26.09.2004, Berlin

Adult onset vitelliform macular dystrophy (AVMD): case presentation

Meeting Abstract

  • corresponding author A. Klosowska-Zawadka - Katedra i Klinika Okulistyki Akademii Medycznej w Poznaniu, Poznan, Poland
  • J. Bernardczyk-Meller - Katedra i Klinika Okulistyki Akademii Medycznej w Poznaniu, Poznan, Poland

Evidenzbasierte Medizin - Anspruch und Wirklichkeit. 102. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft. Berlin, 23.-26.09.2004. Düsseldorf, Köln: German Medical Science; 2004. Doc04dogP 170

Die elektronische Version dieses Artikels ist vollständig und ist verfügbar unter: http://www.egms.de/de/meetings/dog2004/04dog661.shtml

Veröffentlicht: 22. September 2004

© 2004 Klosowska-Zawadka et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielf&aauml;ltigt, verbreitet und &oauml;ffentlich zug&aauml;nglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

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.