Bavbek T, Ogüt M S, Kazokoglu H
Marmara University, School of Medicine, Department of Ophthalmology, Istanbul, Turkey.
Doc Ophthalmol. 1993;83(4):313-22. doi: 10.1007/BF01204333.
Coloboma of the lens is characterized by notching of the equator of the lens. The cause of the condition is faulty development of the zonule. The lens deprived of its normal pull in the defective region is thicker and more spherical as if there were no loss of its substance. We report five cases of lens coloboma and localized zonular defect without any associated systemic anomalies. One case included retinal detachment; another included an iris coloboma; and a third included iris, choroid and optic disk colobomas and retinal detachment with lens coloboma. The remaining two cases were without associated ocular anomalies. The pathogenic mechanisms and relationship of this defect with the differentiation of the tertiary vitreous and development of the zonule is discussed. The defect most likely occurs in the third to fourth months of gestation during differentiation of the tertiary vitreous in response to toxic, inflammatory or genetic factors.
晶状体缺损的特征是晶状体赤道部有切迹。该病的病因是小带发育异常。在缺损区域失去正常牵拉的晶状体更厚且更呈球形，就好像其物质没有损失一样。我们报告了5例晶状体缺损和局限性小带缺损病例，均无任何相关的全身异常。1例伴有视网膜脱离；另1例伴有虹膜缺损；第3例伴有虹膜、脉络膜和视盘缺损以及视网膜脱离合并晶状体缺损。其余2例无相关眼部异常。本文讨论了这种缺损的致病机制以及与第三玻璃体分化和小带发育的关系。这种缺损很可能发生在妊娠第三至第四个月，在第三玻璃体分化期间，由毒性、炎症或遗传因素引起。
