先天性黑蒙自从1869年Leber介绍以来,国外不断有病例报告,以荷兰多见。据1959年Schappent—Kimmijser的统计,已报导227例,其中成人114例,儿童113例。但国内未查到本病的病例报告。我院在1982年4月遇到一例,特报告如下: 王×,女性,4个月。于1982年4月1日初诊。主诉:患儿生后至今不能注视任何物体。首胎,足月 Congenital amaurosis Since the introduction of Leber in 1869, there have been cases of foreign reports, more common in the Netherlands. According to Schappent-Kimmijser in 1959, 227 cases have been reported, including 114 in adults and 113 in children. However, the domestic case did not find the report of the disease. In our hospital in April 1982 encountered a case, special report is as follows: Wang ×, female, 4 months. On April 1, 1982 newly diagnosed. Chief complaint: children can not watch any object after birth. The first child, full-term