中心性輪紋状脈絡膜ジストロフィーの1例

A central areolar choroidal dystrophy

　中心性輪紋状脈絡膜ジストロフィーと思われる38歳の女性の1例を14カ月間にわたって観察し病態の進行を確認した．主要な病変は黄斑部の地図状の網膜色素上皮および脈絡膜毛細管板の萎縮と，それを取り囲む点状の網膜色素上皮萎縮であった．中心窩はこれらの病変から免れていたが，螢光眼底所見では黄斑周囲毛細血管系蹄が不明瞭であった．14カ月の経過で，視力，眼底所見は悪化したが視野，色覚，ERG,EOGの各所見に著変はなかった．網膜色素上皮の萎縮巣は初め点状であったものが次第に癒合し，拡大して地図状となった．この拡大は部位により相違があり，前駆病変たる点状病巣の分布の広範囲な所で必ずしも著しくなく，むしろ点状病変の分布は狭い．しかし点状，小斑状，小地図状など多様の病変が混在した場所で，地図状病巣の拡大は大きかった．

We diagnosed a 38-year-old female as bilateral central areolar dystrophy in its early stage. As characteristic features of macular involvement, a well-defined geographic atrophy was located at the level of retinal pigment epithelium (RPE) surround-ed by numerous atrophic dots in the same level. The perifoveal capillary arcade failed to fill by fluores-cein angiography.The visual acuity was 0.7 and 0.9 for the right and left eye.

The pathological fundus findings became more pronounced during the follow-up period of 14 months, associated with decrease in visual acuity bytwo lines in either eye. No remarkable changes were observed concerning other visual functions including visual field, color vision, electrooculo-gram and photopic electroretinogram. The enlarge-ment of geographic macular area occurred as conse-quence of confluence of dot lesions. The extension of geographic lesion was more manifest in sectors with irregular borders. The enlargement of the geographic lesion was associated with an increase in pigment clumps and in the visibility of choroidal vessels.

Rinsho Ganka (Jpn J Clin Ophthalmol) 41(4) : 383-386, 1987