黄斑部にみられた先天性網膜色素上皮肥大

A case of congenital hypertrophy of the retinal pigment epithelium in the macula

要約　目的：黄斑部にみられた先天性網膜色素上皮肥大の報告。症例：15歳女性が左眼黄斑部の異常を指摘され受診した。所見：矯正視力は右1.2，左0.5で，左眼底黄斑部に脱色素斑に囲まれた扁平な円形の色素斑がみられた。光干渉断層計で網膜色素上皮の肥厚，視細胞層の障害がみられた。眼底自発蛍光では，色素斑部は均一な低蛍光を呈し，脱色素斑は過蛍光を示した。黄斑部の先天性網膜色素上皮肥大と診断し，経過観察している。結論：先天性網膜色素上皮肥大が黄斑部に生じ，視力が比較的保たれていることがある。

Abstract.　Purpose：To report a case of congenital hypertrophy of the retinal pigment epithelium（CHRPE）in the macula. Case：A 15-year-old female presented with macular pigmented lesion in the left eye. Findings：Corrected visual acuity was 1.2 right and 0.5 left. A flat round pigmented lesion with a non-pigmented marginal halo was observed in the macular area in the left eye. Optical coherence tomograph（OCT）showed thickening of retinal pigment epithelium and damage of photoreceptor layer. Pigmented lesion showed homogenous hypoautofluorescence with hyperautofluorescence at the non-pigmented surrounding area. The diagnosis of macular CHRPE was made. Conclusion：CHRPE may be present in the macular area and vision may not be severely impaired.