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自1985年以来我们共收治了先天性晶体脱位35例,通过家系调查,眼部和全身的详细检查,以及骨骼X线照片。超声心动图和亚硝基铁氰化钠试验等检查,诊断为马凡氏综合征21例,单纯性晶体异位6例,马奇山尼氏综合征3例,先天性无虹膜4例,同型胱氨酸尿症1例。我们发现先天性晶体脱位最明显的眼部表现是视力减退。视力损害的程度与晶体脱位的程度有密切的关系。以中等度(Ⅱ度)的晶体脱位对视力影响最大。视网膜电流图(ERG)检查表明大部分病例的视网膜功能是正常,提示先天性晶体脱位造成视力下降的主要原因是晶体性近视及散光。因此必须强调早期诊断,及时进行视力矫正,防止弱视的发生。
We have treated 35 cases of congenital dislocation of the crystal since 1985, through pedigree investigation, detailed eye and body examination, and bone radiographs. Echocardiography and nitrosylated sodium cyanide test and other tests, the diagnosis of Marfan syndrome in 21 cases, 6 cases of simple crystal ectopic, March Hill Nyhee’s syndrome in 3 cases, 4 cases of congenital absence of the iris, the same type of cyst 1 case of aciduria. We found that the most obvious ocular manifestation of congenital dislocation of the eyes is vision loss. The degree of visual impairment and crystal dislocation are closely related. With moderate (Ⅱ degree) of the dislocation of the greatest impact on vision. Retinal electrogram (ERG) examination showed that most cases of retinal function is normal, suggesting that congenital dislocation of the lens caused by decreased vision was mainly due to myopia and astigmatism. Therefore, we must emphasize the early diagnosis, timely vision correction, to prevent the occurrence of amblyopia.