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Purpose: To evaluate the outcome for vitreoretinal surgery in children with familial exudative vitreoretinopathy (FEVR) and to evaluate the risk factors associated with failure., Methods: This is a retrospective interventional case series of 43 consecutive eyes (34 patients) with vitreoretinal surgery for FEVR. Ocular status prior to intervention and at last follow-up and all surgical steps were recorded. Follow-up time was at least 6 months. Main outcome measure was surgical failure (defined as one of the following: (1) deterioration of visual acuity and stage, (2) persistence or development of total retinal detachment, (3) phthisis)., Results: After a mean follow-up of 3.3 ± 3.4 years (median 2.3; 0.5-15.7 years), surgery was successful in 30 eyes (70%) and failed in 13 eyes (30%). Twenty-one eyes (49%) improved, 13 (30%) remained stable, and 9 (21%) deteriorated. Postoperatively, stages and VA improved significantly (p = 0.001; p = 0.04, respectively). Surgical failure was only observed on patients with stages 4 and 5. Mean macular thickness decreased significantly in eyes (stages 2 and 3) with tractional epiretinal membrane., Conclusion: Eyes with tractional epiretinal membrane in stages 2 and 3 seem to benefit from vitrectomy and membrane peeling with a positive risk-benefit profile. Advanced stages have a low success rate and limited functional improvement, but in selected cases, surgery seems beneficial.

Purpose: To determine clear-cut distinctions between tractional and exudative intraretinal cystoid spaces subtypes., Design: Retrospective, multicenter, observational case series., Methods: A cohort of patients diagnosed with intraretinal cystoid spaces and imaged with optical coherence tomography (OCT), fluorescein angiography (FA), blue fundus autofluorescence (BFAF), en face OCT, and OCT angiography (OCT-A) was included in the study. All images were qualitatively and quantitatively evaluated., Results: In this study were included 72 eyes of 69 patients. Exudative intraretinal cystoid spaces (36/72 eyes, 50%) displayed a "petaloid" morphology as seen with en face OCT, FA, and BFAF. Tractional intraretinal cystoid spaces (24/72 eyes, 33.3%), displayed a radial "spoke-wheel" en face OCT pattern. There was no leakage with FA and BFAF did not reveal specific patterns. Eyes with full-thickness macular hole (FTMH, 12/72 eyes, 16.7%) displayed a "sunflower" en face OCT appearance. FTMH showed OCT, OCT-A, and BFAF features of both exudative and tractional cystoid spaces, but without any FA leakage. Inner nuclear layer (INL) thickness was significantly lower in tractional cystoid spaces (P < .001). There were a greater number of INL cystoid spaces in both the exudative and FTMH subgroups (P = .001). The surface area of INL cystoid spaces was significantly lower in the tractional subgroup (P < .001). There was a significant reduction of the microvascular density in eyes with exudative vs tractional (P = .002) and FTMH (P < .001) subgroups., Conclusions: Exudative and tractional intraretinal cystoid spaces displayed characteristic multimodal imaging features and they may represent 2 different pathologic conditions with equally different clinical implications., (Copyright © 2019 Elsevier Inc. All rights reserved.)