关于Marfan综合征,在国内外已有很多报导,为常染色体显性遗传的先天性疾患,其特点有骨骼、心血管及眼部异常等多种病变。Marfan综合征的眼改变有50%～80%发生晶状体异位,多数是双侧对称,有时还伴有白内障。虹膜缺损、瞳孔残膜、兰巩膜、斜视、眼球震颤,高度近视、继发青光眼、视网膜色素变性等眼改变,但伴有视网膜脱离者并不多见。最近我院收治两例Marfan综合征伴有严重视网膜脱离的患者,兹报告如下。 About Marfan syndrome, there are many reports at home and abroad, autosomal dominant congenital disorders, which are characterized by skeletal, cardiovascular and ocular abnormalities and other diseases. Marfan syndrome changes in the eyes of 50% to 80% of the lens ectopic, most bilateral symmetry, sometimes accompanied by cataracts. Iris defect, pupil residual film, blue sclera, strabismus, nystagmus, high myopia, secondary glaucoma, retinal pigment degeneration and other eye changes, but associated with retinal detachment are rare. Recently admitted to our hospital two cases of Marfan syndrome associated with severe retinal detachment of patients, it is reported as follows.