網膜および乳頭上新生血管を伴う錐体ジストロフィが疑われた1例

A case of presumed cone dystrophy with retinal and disc neovascularizations

　網膜および乳頭上新生血管を伴った錐体ジストロフィと考えられる症例を報告した。症例は14歳女性で，高度の視力障害および色覚異常を伴い，両眼底には後極部，特に黄斑部に色素異常がみられ，乳頭およびその周囲に網膜新生血管を伴っていた。螢光眼底造影では明らかな無血管野は存在せず，新生血管からの旺盛な螢光色素漏出が認められた。

　網膜電位図は低振幅で錐体系の網膜電位図は消失型であり，暗順応検査では一次，二次とも閾値の上昇がみられた。これらの結果より網膜新生血管を伴った錐体ジストロフィの特異な1例と考えた。

A 14-year-old girl presented with severe visual disturbance, photophobia, dyschromatopsia and day blindness as chief complaints. Pigmented lesions were present throughout the posterior fun-dus, particularly in the macula, in both eyes. Neovascularizations were present from the disc and along the retinal vascular arcades in both eyes. Preretinal and vitreous hemorrhages were present in the right eye. Fluorescein angiography showed dye leakage from the newly formed vessels. Capil-lary nonperfusion was absent. Perimetry showed generalized constriction and depression by internal isopters. Color discrimination tests showed total color blindness. Bright flash electroretinogram showed low amplitudes.in a and b waves. This case was diagnosed as an unusual type of advanced cone dystrophy with retinal and disc neovasculariza-tions.