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朱××男13岁右眼胀痛、视力下降半年。半月前,家人发现患儿右眼瞳孔变形,于1989年4月21日来我院眼科门诊。视力:右0.6(矫正不应),左1.2。双眼角膜透明,KP(一),前房清,前房深浅正常。双眼虹膜呈广泛性萎缩,色素大量脱失,基质稀疏,虹膜菲薄。右眼瞳孔上方色素外翻,瞳孔变形,瞳孔移至7点处虹膜根部,大小约6×4mm,光反应(一)。近角膜缘1～5点处有1.5×1.0mm和3.0×4.0mm的孔洞。右眼晶体和玻璃体透明。右眼视神经乳头呈瓷白色,边界清,视乳头血管偏向鼻侧,C/D=1.0。视网膜动脉正
Zhu × × male 13-year-old right eye pain, decreased vision for six months. Half a month ago, the family found that children with right pupil deformity, in April 21, 1989 to our hospital ophthalmology clinic. Eyesight: Right 0.6 (should not be corrected), left 1.2. Cornea transparent, KP (a), anterior chamber clear, anterior chamber normal depth. Eyes iris showed atrophy, a large number of pigment loss, matrix sparse, iris meager. Right pupil pupil valgus eversion, pupil deformation, the pupil moved to the iris at 7 o’clock root, the size of about 6 × 4mm, light response (a). Near the limbus 1 to 5 points at 1.5 × 1.0mm and 3.0 × 4.0mm holes. Right eye crystal and vitreous transparent. Right optic nerve head was porcelain white, clear border, visual nipple blood vessels to the nasal side, C / D = 1.0. Retinal artery is positive