特異な経過をたどった強膜化角膜の1例

A case of sclerocornea with unconventional clinical course

目的：緑内障と網膜剝離が併発した両眼の強膜化角膜の1症例の報告。症例と経過：在胎39週，3,110gで正常分娩をした男児。出生直後に両眼の眼球突出と角膜混濁があり，角膜への血管侵入と混濁が強く，球結膜との境界が不明瞭で，虹彩など眼内部が透見不能であり。触診で高眼圧であった。CT検査で前房が深かった。全身的に格別の異常はなかった。生後5日目の超音波検査で，両眼に網膜全剝離を認め，緑内障手術，角膜移植，硝子体手術などで視機能改善が期待できないと判断した。以後，眼圧変動に伴い，眼球は拡大と縮小を繰り返し，6か月後に左眼，1年後に右眼が眼球癆になった。結論：出生直後から強膜化角膜があり，これに併発した網膜剝離と高眼圧が病状を修飾した症例である。

Purpose：To report an infant of bilateral sclerocornea associated with ocular hypertension and retinal detachment. Case：A male infant was born after gestation of 39 weeks weighing 3,110 g. Both eyes showed proptosis and opaque cornea immediately after birth. The cornea was densely vascularized and was continuous with the conjunctiva. The inner ocular segment was invisible. Computerized tomography（CT）showed deep anterior chamber. Palpation showed elevated ocular pressure in both eyes. No systemic disorder was present. Ultrasonography showed total retinal detachment 5 days after birth. There was no indication for surgery for turbid cornea,glaucoma or retinal detachment. Thereafter,the eyes showed repeated enlargement and reduction in size. Both eyes became phthisic one year after birth. Conclusion：The observed unusual clinical course appeared to be due to retinal detachment and ocular hypertension in addition to sclerocornea.