角膜移植により重度の羞明が改善した金柑型膠様滴状角膜ジストロフィの1例

Improvement of severe photophobia following keratoplasty in a case of kumquat-type of gelatinous drop-like corneal dystrophy

要約　目的：重度の羞明が角膜移植により改善した金柑型膠様滴状角膜ジストロフィ（GDLD）の症例の報告。

症例：34歳，女性。幼少時より両眼の視力低下と羞明があり，GDLDを指摘されていた。

所見と経過：初診時の視力は右手動弁，左光覚弁であった。両眼とも角膜全体に血管侵入を伴う黄色の膠様隆起物を認めた。右眼の全層角膜移植を行い，切除した角膜にアミロイドを認め，金柑型GDLDと診断した。術後3か月より羞明が改善し，開瞼が可能となった。術後6か月で右眼矯正視力は（0.4）となり，QOL（quality of life）が向上した。

結論：GDLDに対する角膜移植で羞明が劇的に改善しQOLが向上した。

Abstract　Purpose: To report a case of kumquat-type of gelatinous drop-like corneal dystrophy（GDLD）in whom severe photophobia improved after keratoplasty.

Case: A 34-year-old female presented with impaired vision and severe photophobia in both eyes. She had been diagnosed with GDLD since infancy.

Findings and Clinical Course: Best-corrected visual acuity was hand motion right and light perception left. The cornea in both eyes showed yellow gelatinous elevation with neovascularization. The right eye was treated by penetrating keratoplasty. The removed corneal specimen showed amyloid deposit, leading to the diagnosis of kumquat-type GDLD. Photophobia alleviated 3 months after surgery, enabling opening of eyelid. Corrected visual acuity improved to 0.4 six months after surgery with better quality of life（QOL）.

Conclusion: Keratoplasty resulted in dramatic improvement of severe photophobia with better QOL.