Surgical outcomes of tractional maculopathy associated with familial exudative vitreoretinopathy in children

Purpose: To evaluate the surgical outcomes of pediatric familial exudative vitreoretinopathy (FEVR) complicated by tractional maculopathy. Methods: Retrospective case series. Chart review of 14 children (15 eyes) diagnosed with tractional maculopathy-complicated FEVR who received vitrectomy. Results: The mean age at surgery was 7.2 years. The mean follow-up duration was 14.1 months. The logarithm of the minimum angle of resolution (logMAR) of best-corrected visual acuity (BCVA) improved from 1.0±0.6 (20/200 Snellen) to 0.6±0.6 (20/80 Snellen) post-operation (t=4.293, p=0.001). The peripapillary temporal inner angle (PTIA, 63.9(15.7) ° vs. 71.1(31.2) °, z=-2.726, p=0.006) and peripapillary temporal outer angle (PTOA, 63.4±25.2° vs. 69.6±23.5°, t=-2.820, p=0.014) widened post-operation. Postoperative BCVA was superior in eyes with a shorter time between symptom onset and surgery (r=0.688, p=0.019), better preoperative logMAR BCVA (r=0.830, p<0.001), and preoperative widening of the outer nuclear layer (ONL, r-pb=0.730, p=0.007) and foveal avascular zone (FAZ, r-pb=0.794, p=0.002), and in eyes with postoperative ellipsoid (r-pb=0.641, p=0.018) and interdigitation zones integrity (r-pb=0.614, p=0.026), widening of the ONL(r-pb=0.816, p=0.001) and FAZ (r-pb=0.940, p<0.001), and absence of the inner retinal layer at the fovea (r-pb=0.672, p=0.012). Conclusion: Vitrectomy is effective for pediatric FEVR complicated by tractional maculopathy. Patient selection is crucial and iatrogenic complications should be avoided.