Brittle cornea syndromeと思われた1症例

A case of presumed brittle cornea syndrome

（P2-1-16）　38歳男性が右眼の眼圧上昇で紹介され受診した。矯正視力は右眼は指数弁，左眼は0であった。Goldmann眼圧計による眼圧は右29mmHg，左32mmHg。角膜径は左右とも11.5mmで，角膜厚は中央部で03mmであり，球状角膜の所見を呈していた。眼軸長は左右とも40mm。青色強膜はなかった。全身的に関節弛緩症，歯牙が抜けやすいこと，高音領域の聴力の軽度低下があった。Brittle corneasyndromeと診断した。右眼に線維柱帯切除術を行った。炭酸脱水酵素阻害薬の内服を中止すると，左眼の眼圧は変化しなかったが，眼圧が関与すると推定される左眼の疼痛が出現した。角膜が薄い場合には，みかけの眼圧値と実際の眼圧が解離していることが推定された。

A 38-year-old male presented with raised intraocular pressure (I0P) in his right eye. His corrected visual acuity was hand motion right and no light perception left. By Goldmann tonometry, his IOP was 29 mmHg right and 32 mmHg left. The corneal diameter was 11.5 mm in either eye. The central corneal thickness was 0.3 mm. Spherocornea was present in both eyes. Blue sclera was absent. The axial length was 40 mm in either eye. Systemically, he had hyperextensive joints, tendency of the teeth to fall off, and bilateral hearing loss in the high frequency range. We diagnosed him as brittle cornea syndrome. The right eye was treated by trabeculectomy. After discontinuation of oral acetazolamide, the left eye developed severe pain. It appeared that applanation tonometry gave artifically lower TOP value than the actual value.