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(收稿:2007—08—12修回:2008—04—02)
(本文编辑:王璐璐)
图1右眼外观
双眼先天性无虹膜一例
刘利莉李学军崔晓冬
患儿,男,1O岁.
自幼双眼视力差,畏
光.于2008年3月
15日就诊.患儿为足
月顺产,父母非近亲
结婚,家族中无遗传
病史.全身检查:一
般情况好,智力及发
育无异常.眼部检
查:视力右眼0.3,矫正0.8(一3.75DS/+2.50DC×170.);左
眼0.2,矫正0.7(一4.25DS/+2.75DC×10.);双眼眼位正
常,各方向运动自如.右眼角膜透明,前房深度适中,全周虹膜
阙如,可见晶状体悬韧带.晶状体透明,余前节未见异常(图
1).左眼前节检查同右眼.眼底检查:双眼底未见明显异常.
诊断:双眼先天性无虹膜,屈光不正,弱视.
作者单位:075000河北省张家IZl市第四医院眼科 通讯作者:刘利莉(Email:ililuil@sohu.corn)
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病例
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讨论:先天性无虹膜是一种少见的,与常染色体显性遗传 有关的先天性虹膜发育不良.群体患病率为1/55000.该病 发病原因不明,可能与胚胎发育时期神经外胚层或中胚层发育 异常有关,亦可与基因突变有关.先天性无虹膜多双侧发病, 患者多伴明显畏光,视力减退等症状.根据临床表现的轻重, 该病可分为4种类型…:I型可同时伴白内障,青光眼,眼球震 颤,黄斑发育不良等眼部异常,且视功能较差;II型视功能较 好,可能与基因突变有关;?型可伴智力低下;IV型可伴肾胚胎 瘤等全身异常.本例患者由于无明显的家族遗传史且视功能 损害较轻,符合?型表现.该病一般无特殊治疗,可配戴变色 镜,墨镜或周边带色的虹膜型角膜接触镜以减轻患者的不适症 状.合并有白内障,青光眼等眼部异常者可考虑手术治疗. 参考文献
1刘英奇,赵亮,主编.现代眼科学[M].北京:北京科学技术出版社, l996:697
(收稿:2008—03-21)
(本文编辑:王璐璐)