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Rieger氏综合征是一种以眼前节中胚叶组织发育异常或伴有面部牙齿、肌肉、骨骼的畸形。眼部主要异常为突起的Schwalbes环(后胚胎环)、虹膜周边广泛前粘、虹膜前基质营养不良,出现瞳孔移位、变形和多瞳,继发角膜异常如大角膜、小角膜。笔者于1983年3月发现一家系,现报告如下。 (一)调查结果该家系4代共27人,除已故的Ⅰ代2人采用询问方式了解病情外,共检查了25人。有血缘关系的19人。具有典型Rieger氏症者
Rieger’s syndrome is an anterior segment mesodermal tissue dysplasia or facial deformity associated with teeth, muscles and bones. The major ocular anomalies are raised Schwalbes ring (posterior embryo annulus), widespread anterior iris periphery, malrotory anterior iris stroma, pupil displacement, deformation and multiple pupils, secondary corneal anomalies such as the cornea and cornea. I found a family in March 1983, the report is as follows. (A) Findings The family of 4 generations a total of 27 people, except for the deceased II generation using the inquiry method to understand the disease, a total of 25 people were examined. 19 people have a blood relationship. With typical Rieger’s disease