Rieger 氏综合征又名Axenfeld 综合征,伴有青年性青光眼和角膜、虹膜中胚层发育障碍,为一种眼前节中胚叶发育异常,并伴有外胚叶组织发育不全的一组综合征。现将我科所见1家系报告如下。例1 男7岁生后8—9月出现畏光,家长发现其右眼“黑眼球起雾”,以后随年龄增长,逐渐加重。视力进行性下降,眼酸胀、流泪、怕光,双眼阵发性疼痛。于1992年到我科就诊。患儿为第二胎、 Rieger’s syndrome, also known as Axenfeld syndrome, associated with young glaucoma and cornea, developmental disorders of the iris mesoderm, an anterior segment mesodermal dysplasia, accompanied by a group of syndromes of ectodermal hypoplasia. Now I see a family of families reported as follows. Example 1 Male 7-year-old after 8-9 months appear photophobia, parents found that the right eye “black eyeball fog”, with age, then gradually increased. Progressive decline in visual acuity, soreness, tearing, fear of light, his eyes paroxysmal pain. In 1992 to our department. Children for the second child,