The authors reported a case of Bietti's crystalline retinopathy in 1981. In the present paper they report three additional cases studying the stages of this disease and the clinical findings at each stage. Conclusions obtained are as follows: This disease is a type of total RPE-choriocapillaris dystrophy, the process of which starts at the posterior pole. This disease can be divided into three stages, based on the clinical findings: Stage 1: RPE atrophy with uniform fine white crystalline deposits is observed at the macular area. Stage 2: RPE atrophy extends beyond the posterior pole. Choriocapillaris atrophy in addition to the RPE atrophy appears markedly at the posterior pole. Crystalline deposits in the lesion vary in shape and size and have a tendency to conflue. The number of crystalline deposits is less in the advanced atrophic areas of the RPE-choriocapillaris complex. Stage 3: RPE-choriocapillaris complex atrophy is observed throughout the fundus. The total number of crystalline deposits decreases because the crystalline deposits that disappear number more than those that appear in Stage 3. Crystalline deposits which are observed at the corneal limbus are possibly prominent in the advanced stage of atrophy of the RPE-choriocapillaris complex.

Suggested is the strong possibility that cigarette smoking enhances the neovascular form of AMD risk in late middle aged males, though the magnitude of risk by smoking variables might be overestimated, in part, because of health oriented controls.