Dome-shaped maculaに伴う漿液性網膜剝離を合併した完全型先天停在性夜盲の1例

A case of complete-type congenital stationary night blindness associated with serous retinal detachment due to dome-shaped macula

要約　目的：完全型先天停在性夜盲にdome-shaped maculaと漿液性網膜剝離が併発した症例の報告。

症例：39歳女性が右眼の視力低下と変視症で受診した。幼少時から夜盲と視力低下があり，20歳のときに網膜色素変性症と診断された。父親の親族に夜盲と色覚異常があるという。

所見：矯正視力は右0.5，左0.8で，それぞれ−15Dと−14.5Dの近視があった。光干渉断層計で，両眼の黄斑部が前方凸に隆起し，右眼の中心窩下に漿液性網膜剝離があった。

結論：完全型先天停在性夜盲には強度近視が併発することがあり，経過中にdome-shaped maculaや，それに伴う漿液性網膜剝離などの合併症が起こったものと解釈される。

Abstract　Purpose: To report a case of complete-type congenital night blindness associated with dome-shaped macula and serous retinal detachment.

Case: A 39-year-old woman presented with metamorphopsia and impaired vision in the right eye. She had had night blindness and impaired vision since childhood. She had been diagnosed with retinitis pigmentosa at the age of 20. A paternal relative had reportedly night blindness and color anomaly.

Findings: Best-corrected visual acuity was 0.5 right and 0.8 lef when corrected by −15 and −14.5 diopters respectively. Optical coherence tomography showed anterior protrusion of the macula in both eyes. Serous retinal detachment was present in the right macula. Electroretinogram was of negative type. She was diagnosed with complete-type congenital night blindness.

Conclusion: This case illustrates that complete-type congenital night blindness may be associated with high myopia. Dome-shaped macula and serous retinal detachment are interpreted as complications of high myopia.