黄斑部疾患に関する考察錐体（-杆体）ジストロフィ

Observations on maculopathies. Cone-rod dystrophy

　我々はび漫性錐体機能不全を示した錐体（-杆体）ジストロフィ52名，およびその疑い2名計54名の初診時の後極部所見をIA,IB,II群，その他に分類し，経過観察を行えた症例および家族発症例を中心に，各病型の進行様式について検討を加え，以下のように推論した．

　IA群は輪状の萎縮病巣（＝標的黄斑病巣）が内方に向って進行する病型で，やがて黄斑部には均一な色素上皮の萎縮病巣が形成され，さらに進行すると病巣内には脈絡膜毛細管萎縮が出現する．

　IB群は標的黄斑病巣が，外方に向かって進行する病型で，進行期には色素上皮の萎縮病巣は黄斑部耳側から乳頭を越えた範囲までひろがり，病巣内には脈絡膜毛細管板萎縮が出現する．

　II群は初期に中心窩を含んで色素上皮の萎縮病巣のみられるもので，進行期には多量の色素沈着を伴う色素上皮の萎縮病巣は黄斑部耳側から乳頭鼻側までひろがり，病巣内には脈絡膜毛細管板萎縮が出現する．

We classified 54 cases of cone-rod dystrophy with diffuse cone dysfunction based on fundus and fluor-escein angiographic findings. The progression of fundus pathologies was evaluated in view of this classification.

The cases were classified into 3 types : Group IA : bull's eye macular lesion progressing toward inside Group IB : bull's eye macular lesion progress-ing peripherally. Group II : diffuse atrophy of the retinal pigment epithelium (RPE) originating in and around the fovea,

In eyes belonging to Group IA, RPE atrophy appearing in an almost normal macula turned intobull's eye macular lesion. In its advanced stage bull's eye macular lesion progressed centrally, form-ing an oval, uniform atrophic RPE lesion in the macular area. Atrophy of the choriocapillaris occa-sionally appeared associated with pigmentations. In eyes belonging to Group In, bull's eye macular lesion advaced peripherally, resulting in atrophy of the RPE spreading from the macula in the nasal and temporal directions involving the peripapillary area. In its advanced stage, atrophy of the chor-iocapillaris developed with pigmentations. In cases belonging to Group II, fine granular atrophy of RPE in the macular area spread in all directions involving the peripapillary area. It later turned into diffuse atrophy of the RPE associated with atrophy of the choriocapillaris and with pigmentation.

Rinsho Ganka(Jpn J Clin Ophthalmol) 41(5) : 461-468, 1987