[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.