類嚢胞黄斑浮腫とCoats病様眼底病変を合併した網膜色素変性症の1例

A case of retinitis pigmentosa associated with cystoid macular edema and exudative retinal lesions

　網膜色素変性症にCoats病様眼底病変と類嚢胞黄斑浮腫を合併した，10歳女性の1症例を報告した．本症例は黄斑浮腫による中心視力の低下により眼科受診したものである．眼底には網膜色素変性症の初期の所見として網膜にびまん性の軽い変性と，赤道部に小数の骨小体様色素沈着以外に，網膜にびまん性の軽度の浮腫と黄斑部に類嚢胞浮腫により層状円孔を生じ，さらに右眼耳下側赤道部に，血管異常を伴う黄白色滲出斑を認めた．螢光造影により，周辺部には毛細血管拡張症と無血管野が存在した．検査により，第二次暗順応の障害，ERGの消失，EOGの低下，視野の小さい指標での求心性狭窄を認めた．治療として，Indomethacinの点眼とPrednisoloneの内服および周辺部のCoats病様病変に対するアルゴンレーザーによる光凝固を行った．5カ月後眼底所見の改善および，視力の改善をみつつある．この症例は網膜色素変性症が基盤にあり，それに網膜血管異常を生じて周辺部にはCoats病様変化を生じ，黄斑部に類嚢胞黄斑浮腫を起こしたものと考えられた．このように網膜色素変性症は，網膜血管の障害を生じ，多彩な眼底像を示すことがあるので紹介した．

A 10-year-old female child sought medical advice for defective central vision in both eyes. The corrected visual acuity was 0.4 RE and 0.7 LE. There were no systemic abnormalities, hematological or serological disorders.

Bilaterally, the fundus manifested retinitis pig-mentosa in its early stage. Diffuse and low-grade retinal edema was present. Cystoid macular edema waspresent with a large lamellar hole in either eye. Addi-tionally, yellowish retinal exudates were present in the equator associated with pathologically dilated retinal vessels.Telangiectasis with avascular retina in the farthest periphery was seen in the right eye. Fluorescein angiography showed diffuse extravasation from retinal vessels. Electroretinogram was nonrecordable. The dark adaptation curve was markedly decreased at the second phase. We diagnosed this case as retinitis pig-mentosa in its early stage, with associated retninal vascular abnormalities resulting in cystoid macular edema and yellowish exudates simulating Coats' dis-ease.

Rinsho Ganka (Jpn J Clin Ophthalmol) 40(11) : 1251-1255, 1986