目的探讨先天性脉络膜缺损合并视网膜脱离的临床特点，寻找较好的手术方法，以提高疗效。方法对８例（８只眼，其中７只眼视盘在缺损区内）脉络膜缺损合并视网膜脱离患者，施行玻璃体视网膜手术，术中在高倍显微镜下观察视网膜裂孔、玻璃体后界膜、缺损区的情况，行相应处理，硅油填充。结果术后视网膜均获得解剖复位，视力均有提高。结论此类视网膜脱离可因位于缺损区内的视网膜裂孔造成，其裂孔位于缺损区内带状视网膜脱离的边缘处，无玻璃体后脱离、缺损区巩膜外膨出等特点。充分的玻璃体切除合并封闭缺损区、硅油填充，可望成功 Objective To investigate the clinical characteristics of congenital choroidal defects with retinal detachment and to find a better surgical method to improve the curative effect. Methods Eight patients (8 eyes, including 7 eyes) with choroid defect and retinal detachment were treated with vitreoretinal surgery. The retinal breaks, the posterior vitreous membrane and the defect were observed under high magnification microscope Corresponding treatment, silicone oil filling. Results Postoperative retinal anatomical reduction were obtained, visual acuity increased. Conclusion Such retinal detachment may be caused by retinal breaks located in the defect area. The fissures are located at the edge of the retinal detachment in the defect area without posterior vitreous detachment and bulging of the sclera outside the defect area. Full vitrectomy with closed defect area, filled with silicone oil, is expected to succeed