ObjectiveTo analyze the color Doppler flow imaging (CDFI) features of familial exudative vitreoretinopathy (FEVR) at different stages. MethodsA retrospective study. A total of 104 patients with 201 eyes from Department of Ophthalmology of Beijing Tongren Hospital who were hospitalized for fundus examination and diagnosed with FEVR from 2018 to 2022 were included. There were 69 male cases with 133 eyes and 35 female cases with 68 eyes. The age was ranged from 2 months to 11 years, with a mean age of 2.9 years. Fundus and CDFI examination were performed in both eyes. Fluorescein fundus angiography was performed in 72 cases (144 eyes). FEVR staging was conducted according to literature standards. The presence of avascular areas in the peripheral retina or abnormal retina neovascularization was stage 1; the presence of retinal neovascularization at the vitreoretinal interface in the avascular area was stage 2; partial retinal detachment without macula involvement was stage 3; partial retinal detachment involving the macula was stage 4; complete retinal detachment was stage 5. The CDFI ultrasound features of FEVR at different stages were analyzed. The CDFI image features of FEVR patients in different stages were observed. ResultsAmong the 104 patients, 97 (93.3%, 97/104) cases were binocular and 7 (6.7%, 7/104) cases were monocular. In 201 eyes, stages 1 to 5 of FEVR were 49 (24.4%, 49/201), 23 (11.4%, 23/201), 39 (19.4%, 39/201), 71 (35.3%, 71/201), and 19 (9.5%, 19/201) eyes, respectively. CDFI examination showed no abnormality or mild vitreous opacity in 49 eyes vitreous body at stage 1. Vitreous opacities were observed in all 23 eyes in stage 2, and the echo of the temporal ballwall was not smooth. In 39 eyes at stage 3, the anterior globular cluster echo in temporal peripheral eyes was observed in 17 eyes and partial retinal detachment was observed in 13 eyes. In 71 eyes at stage 4, 51 eyes had temporal or infratemporal retinal folds, and 20 eyes had temporal retinal detachment. All the 19 eyes in stage 5 had total retinal detachment, of which 15 eyes had closed "funnel-shaped" retinal detachment. Retinal folds were found in 51 eyes, 26 eyes in 13 cases. There were vitreous opacities in 25 eyes of 25 monocular cases, and the contralateral eye had different degrees or banded echoes in front of the temporal eye. Blood flow signals could be detected on the retinal folds with Doppler imaging. ConclusionsThe CDFI manifestations of FEVR patients at different stages have different characteristics. The possibility of FEVR should be considered when the temporal or infratemporal retinal folds of both eyes are present, as well as the retinal folds of one eye, the contralateral vitreous body opacity, or the anterior temporal peribulbar cluster echoes are present.