Fundus flavimaculatus group 2の姉弟例の十数年後の眼所見について

Fundus flavimaculatus in a pair of siblings over a period of more than ten years

　進行して検眼鏡的にはinversc retinitis pigmentosa様の所見を呈し，機能検査の結果からはprogessive cone-rod dcgeneration （dystrophy）と診断される例の十数年前の眼底写真から，fundus Havimaculatus with atrophic macular dcgenerationの進行例である事が明らかとなった一家系2症例について眼底所見を中心に報告した。初診時はFishman分類のstage 2の状態で，再診時はstage 3〜4の移行期まで進行した状態と判断される。本症のstage 3以降は他の黄斑変性症の進行期との鑑別が困難だが，中間周辺部から赤道部近くまで散在する灰色斑が診断の際に有用な所見である。

We observed a 30-year-old male and his 35-year-old sister, both with fundus flavimaculatus. We could check clinical records of both patients taken11 and 14 years before respectively.

While the atrophic macular degeneration with numerous grayish flecks were the chief findings during the previous examinations more than 10 years before, the flecks had now almost disappeared except in the midperipheral retina and the pigmen-tation had increased over the posterior fundus area. These findings simulated those of inverse retinitis pigmentosa. Functional examination showed the presence of cone-rod dysfunction.