網膜色素変性症の眼底所見と病期—無色素変性症と脈絡膜毛細血管板萎縮について

Angiographic classification of primary pigmentary retinal dystrophy

　63例の定型的および無色素性網膜色素変性症（以下PRDと略）を主に螢光眼底造影上の脈絡膜毛細血管板萎縮像（以下CCAと略）に基づいて4群に分類し他の臨床所見との相関を調べた．第1群は無色素性PRD，第2群はCCAを認めない定型的PRD，第3群は視神経乳頭径大以下の小型のCCAを認める定型的PRD，第4群は視神経乳頭径大以上の大型のCCAを散在性あるいは融合性に認める定型的PRDである．以上の分類は，遺伝型式および発症時期とは明らかな相関を有せず発症後の期間，視力，視野，色素異常と相関しており，本症の病期を反映すると考えられた．

A series of 63 cases with primary pigmentary retinal dystrophy were classified into four groups based on funduscopic and fluorescein angiographic findings. Group 1 : pigmentary retinal dystrophy without pigment patches. Group 2 : typical pigmen-tary retinal dystrophy without hypofluorescent areas suggestive of atrophy of the choriocapillaris. Group 3 : typical pigmentary retinal dystrophy with hypo-fluorescent patches smaller than one disc diameter. Group 4 : typical pigmentary retinal dystrophy with disseminated or confluent hypofluorescent patches larger than one disc diameter.

The different modes of inheritance were dis-tributed evenly among the four groups. The age of onset was not correlated with the classification. The duration of subjective symptoms and the degree of visual field impairment were positively correla-ted with Group 1, 2, 3 and 4 in the ascending order.

From these findings, we concluded that the pig-mentary retinal dystrophy without pigment is an early form and that the atrophy of choriocapillaris is more prevalent in the advanced stage of the disease.