先天性晶体脱位（35例）临床观察

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。

A clinical study and analysis of congenital lenticular dislocation (35 cases)]

Thirty-five cases of congenital lenticular dislocation seen in our Center since 1985 have been studied and analyzed clinically. By the survey of pedigrees and examination of these patients, including ocular, systemic, skeletal X-ray, psychocardiogram, and urinary sodium-nitroprusside test, 21 cases were diagnosed as Marfan's syndrome, 6 cases as simple ectopia lentis, 3 cases as Weill-Marchesani's syndrome, 4 cases as aniridia and 1 case as homecys tinuria. We found that the most significant ocular manifestation of congenital lenticular dislocation was reduction in visual acuity. The severity of visual disturbance varied with the types of dislocation and the visual deficiency was closely related to the intermediate-grade (II) dislocation of the lens. Examination of ERG showed normal function in most of the patients. From this, we believe that the major cause of visual reduction in congenital lenticular dislocation is lenticular myopia and astigmatism. There fore, early diagnosis and effective correction of vision should be emphasized to prevent the occurrence of amblyopia.