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张××,女,24岁。自幼两眼畏光、视力障碍。于1987年10月7日来我院就诊。家族中一胞姐有类似眼病。全身检查无异常所见。眼部检查:远视力双眼均为0.1~(+1),近视力0.3。角膜直径在正常范围。前房深,未见虹膜,晶体不均匀混浊。眼位正,未见眼球震颤。前房角镜检查:两眼前房角各种解剖标志清晰,睫状体前端有宽约1.0毫米环状虹膜残根。眼底:两眼视乳头略小,色红,境界清,生理凹陷不明显。血管正常,黄斑区色素紊乱,中心凹反射存在。周边部视网膜正常,玻璃体未见混浊。眼压:两眼均为2.73kPa。检影,右:+5.00DSC+1.00DCx90°
Zhang × ×, female, 24 years old. Eyes photophobia childhood, visual impairment. In October 7, 1987 came to our hospital. A sister in the family has a similar eye disease. No abnormal body check see. Eye examination: both eyes were far vision of 0.1 ~ (+1), near vision 0.3. Corneal diameter in the normal range. Anterior chamber deep, no iris, the lens is not uniform and turbid. Eye position is positive, no nystagmus. Gonioscopy: two anterior chamber angle various anatomical landmarks clear, the front of the ciliary body is about 1.0 mm wide annular iris root. Fundus: two eyes, slightly smaller nipple, color red, realm clear, not obvious physiological depression. Normal blood vessels, macular pigment disorders, foveal reflex exists. Peripheral retinal normal, no vitreous opacity. Intraocular pressure: both eyes are 2.73kPa. Retinoscopy, right: + 5.00DSC + 1.00DCx90 °