完全型先天停在性夜盲の経過中にdome-shaped maculaを合併した13歳男子

Dome-shaped macula in a 13-year-old boy with complete type of congenital stationary night blindness

要約　背景：Dome-shaped macula（DSM）は強度近視眼の後部ぶどう腫で黄斑部が前方凸に隆起する形態異常である。目的：DSMを生じた完全型先天停在性夜盲の症例の報告。症例：13歳男子が1歳時から内斜視，強度近視，左眼の弱視で加療中であった。7歳時に星が見えないことを自覚して受診した。矯正視力は右1.2，左0.15であり，それぞれ－8Dと－10Dの近視があった。眼軸長はそれぞれ26.18mmと26.37mmであった。光干渉断層計で両眼黄斑部が前方凸に隆起し，左眼中心窩下に漿液性網膜剝離があった。網膜電図での所見とから，DSMが併発した完全型先天停在性夜盲と診断した。結論：強度近視を伴う完全型先天停在性夜盲ではDSMが併発することがある。

Abstract.　 Background：Dome-shaped macula is defined as anterior protrusion of the macula in high myopia with posterior staphyloma. Purpose：To report a case of congenital stationary night blindness with dome-shaped macula. Case：A 13-year-old boy had been diagnosed with esotropia,high myopia and amblyopia in the left eye at the age of one year. He was seen by us at the age of 7 years with night blindness as the chief complaint. Visual acuity was 1.2 right and 0.15 left when corrected by －8 and －10 diopters respectively. Axial length was 26.18 mm right and 26.37 mm left. Optical coherence tomography showed anterior protrusion of the macula,or dome-shaped macula,in both eyes. The left eye showed serous macular detachment. Electroretinography showed findings compatible with congenital stationary night blindness. Conclusion：This case illustrates that dome-shaped macula may develop in eyes with congenital stationary night blindness and high myopia.