視力障害を伴った結節性硬化症の2症例

Two cases of tuberous sclerosis with visual impairment

　結節性硬化症73例（146眼）の臨床像を検討し，2例（2.7％）の片眼に視力予後の不良な症例を認めた。第1例には，瞳孔反応の異常を伴う視神経乳頭過誤腫がみられた。第2例では巨大な網膜過誤腫と陳旧性網膜剥離・後極白内障・視神経萎縮がみられ，虹彩および水晶体の非定型的欠損を伴った。

Two cases with unilateral visual impairment were detected out of a consecutive series of 73 cases with tuberous sclerosis. The first case was a 8-year-old female with alternating hyperphoria, hamar-toma of the optic disc in the left eye and ipsilatcral afferent pupillary defect. The second case was a 12-year-old female with a large retinal tumor sur-rounded by atrophic retinal pigment epithelium, small white tumors, diffuse retinal atrophy, persis-tent retinal detachment, vitreorctinal proliferating strands, posterior polar cataract, optic atrophy and coloboma of the iris and the lens in her left eye.