马方综合征孪生兄弟眼部体征及生物学参数比较  

Comparison of biometric and structural ocular manifestations of Marfan syndrome in twins

作　　者：何晴 魏瑞华[1] He Qing;Wei Ruihua(Tianjin Medical University Eye Hospital,Eye Institute and School of Optometry,Tianjin Branch of National Clinical Research Center for Ocular Disease,Tianjin Key Laboratory of Retinal Functions and Diseases,Tianjin 300384,China)

机构地区：[1]天津医科大学眼科医院天津医科大学眼视光学院天津医科大学眼科研究所国家眼耳鼻喉疾病临床医学研究中心天津市分中心天津市视网膜功能与疾病重点实验室,300384

出　　处：《中华眼科杂志》2021年第7期540-543,共4页Chinese Journal of Ophthalmology

摘　　要：16岁孪生兄弟,弟弟左眼视力下降6个月,哥哥双眼视力下降6个月。2例患者身高均为2.05 m,瘦高体型,蜘蛛状指(趾);均双眼晶状体脱位,双眼豹纹状眼底;双眼生物学参数,包括角膜前后表面曲率和角膜曲率、角膜前后表面散光度数和角膜总散光度数、角膜厚度、眼轴长度等均较为一致。孪生兄弟临床诊断均为马方综合征和双眼晶状体半脱位,临床较为少见。弟弟因左眼视网膜劈裂行后巩膜加固术,术后视网膜基本复位。哥哥予以验光配镜治疗。A pair of 16-year-old twin brothers presented to our hospital.The younger brother had been losing vision in his left eye for 6 months,and the older brother had been losing vision in both eyes for 6 months.Both patients were 2.05 meters tall and lean with spider-like fingers(toes).They had lens subluxation and a leopard-shaped fundus in both eyes.Their ocular manifestations,including anterior and posterior corneal curvature,total corneal refractive power,anterior and posterior and total corneal astigmatism,corneal thickness,and axial length,were relatively consistent.The twin brothers were both diagnosed with Marfan syndrome and bilateral lens subluxation,which is a relatively rare clinical condition.The younger brother underwent posterior scleral reinforcement for retinal foveoschisis in the left eye,and the retina was basically attached after surgery.The older brother was treated with optometry and frame glasses.

关 键 词：马凡综合征 双生 晶体半脱位 视网膜劈裂症 角膜地形图 视敏度 

分 类 号：R779.6[医药卫生—眼科]