Clinical characteristics and surgical management of familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment.

PURPOSE

The purpose of this study was to report the clinical characteristics and surgical outcome of familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment.

METHODS

Retrospective interventional case series of patients with familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment. All cases had preoperative and postoperative clinical evaluations. Eyes were divided into three groups: no, moderate, and severe foveal dragging according to the status of fovea. Scleral buckling procedures or vitrectomy was performed to attach the retina.

RESULTS

Twenty-four eyes in 22 patients were included in this study. The male to female ratio was 18:4. The average age was 16.42 ± 5.48 years. There were 14, 5, and 5 eyes in the no, moderate, and severe foveal dragging groups, respectively. Proliferative vitreoretinopathy was present in only one eye in the severe foveal dragging group. In the subgroup of eyes without foveal dragging, neither proliferative vitreoretinopathy, posterior located breaks, nor giant tears were noted. All eyes needed only scleral buckle to reattach the retina. In the subgroup with moderate foveal dragging, posterior located break was noted in one eye, which was the only eye in this group that needed vitrectomy besides scleral buckle to attach the retina. In eyes with severe foveal dragging, Stage D proliferative vitreoretinopathy was noted in one eye and posterior located break in one eye. Encircling buckle and vitrectomy were necessary in all eyes. The average number of operation is 1.1, 1.2, and 2.4 for each of the three groups, respectively. Final visual acuity improved in 23 of 24 eyes. Final retinal attachment was obtained in 95.8% of eyes (23 of 24).

CONCLUSION

Male predominance and juvenile onset are the main characteristics in familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment. Surgical success rates are comparable to rhegmatogenous retinal detachment unassociated with familial exudative vitreoretinopathy in cases without severe foveal dragging and are worse in the group with severe foveal dragging.