成人発症型卵黄様黄斑変性症の4例

Four cases of adult-onset vitelliform foveomacular dystrophy

成人発症型卵黄様黄斑変性症の4例を経験した。1例は47歳女性，他は47歳，53歳，70歳の男性で，すべて両眼に発症していた。矯正視力は3眼が0.1～0.4，2眼が0.5～0.9，3眼が1.0以上であった。インドシアニングリーン（ICG）による赤外蛍光造影では，病変部は低蛍光，その周囲は淡い過蛍光を呈し，それぞれ網膜色素上皮細胞の凝集によるブロックとICG色素の貯留が推定された。光干渉断層計（OCT）では病変部に色素上皮層の肥厚とその内方に高反射層があり，過去の報告と一致し，網膜色素上皮細胞の凝集と網膜下の物質貯留が推測された。過去に報告がない合併症として，囊胞様黄斑浮腫が53歳男性の両眼にあった。

We observed four bilateral cases of adult-onset vitelliform foveomacular dystrophy. One was a 47-year-old female. The other three were males aged 47,53 and 70 years. Corrected visual acuity was 0.1 to 0.4 in 3 eyes,0.5 to 0.9 in 2 eyes,and 1.0 or over in 3 eyes. Indocyanine green（ICG）angiography showed hypofluorescence in the area of vitelliform lesion,suggesting blockage by accumulated retinal pigment epithelial（RPE）cells. It showed moderate hyperfluorescence around the vitelliform lesion,suggesting staining by ICG dye. Optical coherence tomography（OCT）showed thickening of RPE layer and presence of high-reflective layer inner to it. These findings were consistent with past reports and were interpreted as accumulation of RPE cells and deposit in the subretinal layer. Cystoid macular edema（CME）was present in both eyes of 53-year-old male. This complication has not been reported in past literature.