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Purpose: To report a case of autosomal recessive bestrophinopathy (ARB) that presented with macular hole retinal detachment (MHRD). Methods: A case report. Results: A 31-year-old male patient presented with rapid deterioration of vision in the left eye. On fundus examination, bilateral retinal deposits in both eyes, which were brightly hyperautofluorescent, and an MHRD in the left eye could be detected. An electrooculogram demonstrated absent light rise with abnormal Arden’s ratio in both eyes. The patient was offered surgery for the MHRD but refused due to the guarded visual prognosis. Follow up of the patient after one year revealed progression of the retinal detachment. Genetic testing revealed a novel, homozygous missense mutation in the BEST1 gene, confirming the diagnosis of ARB. Conclusion: ARB can present with an MHRD. Counseling patients with inherited retinal dystrophies about the visual prognosis following surgical intervention is important.

Background: Visual field defects (VFD) usually do not show improvement beyond 12 weeks from their onset. It has been shown that repetitive presentation of a stimulus to areas of residual vision in cases of visual field defect can improve vision. The counterpart of these areas in the brain are the partially damaged brain regions at the perilesional areas where plasticity can be enhanced. Objective: We aimed to study the effect of navigated repetitive transcranial magnetic stimulation (rTMS) applied to perilesional areas on the recovery of patients with cortical VFD. Methods: Thirty-two patients with cortical VFD secondary to stroke of more than 3 months duration received 16 sessions of either active or sham high frequency navigated perilesional rTMS. Automated perimetry and visual functioning questionnaire (VFQ-25) were performed at baseline and after completion of the sessions. Results: The active group showed significant improvement after intervention, compared to the sham group, in both mean deviation (MD), visual field index (VFI) and in the VFQ-25 scores. Conclusions: Navigated rTMS is a new treatment option for post-stroke VFD as it can selectively stimulate areas of residual vision around the infarcted tissue, improving the threshold of visual stimulus detection which could be used alone or in combination with existing therapies.

Purpose To report the anatomic and functional outcomes of autologous retinal transplantation (ART). Design Multicenter, retrospective, interventional, consecutive case series. Participants One hundred thirty eyes of 130 patients undergoing ART for the repair of primary and refractory macular holes (MHs), as well as combined MH-rhegmatogenous retinal detachment (MH-RRD), between January 2017 and December 2019. Methods All patients underwent pars plana vitrectomy and ART, with surgeon modification of intraoperative variables. A large array of preoperative, intraoperative, and postoperative data was collected. Two masked reviewers graded OCT images. Multivariate statistical analysis and subgroup analysis were performed. Main Outcome Measures Macular hole closure rate, visual acuity (VA), external limiting membrane and ellipsoid zone (EZ) band integrity, and alignment of neurosensory layers (ANL) on OCT. Results One hundred thirty ART surgeries were performed by 33 vitreoretinal surgeons worldwide. Patient demographics were: mean age of 63 ± 6.3 years, 58% female, 41% White, 23% Black, 19% Asian, and 17% Latino. Preoperative VA was 1.37 ± 0.12 logarithm of the minimum angle of resolution (logMAR; Snellen equivalent, approximately 20/500), which improved significantly to 1.05 ± 0.09 logMAR (Snellen equivalent, approximately 20/225; P Conclusions In this global experience, patients undergoing ART for large primary and refractory MHs and MH-RRDs achieved good anatomic and functional outcomes, with low complication rates despite complex surgical pathologic features.

Mohamed Nowara,1,2 Yousef A Fouad,1,3 Ihab Abdel Aziz,1,4 Ahmed M Habib,1,3 Mariam Al-feky,1,3 Hisham Hassan1 1Al Mashreq Eye Center, Cairo, Egypt; 2Department of Ophthalmology, Electricity Hospital, Cairo, Egypt; 3Department of Ophthalmology, Ain Shams University Hospitals, Cairo, Egypt; 4Memorial Institute for Ophthalmic Research, Giza, EgyptCorrespondence: Yousef A FouadDepartment of Ophthalmology, Ain Shams University Hospitals, Ramses Street, 11517 Abbasiya, Cairo, EgyptTel +201063781237Email yousef.a.fouad@gmail.comBackground: Coats’ disease is a rare entity with retinal vascular telangiectasia that can progress to exudative retinal detachment, neovascular glaucoma, and a blind painful eye requiring enucleation. Despite recent therapeutic advances decreasing the need for enucleation, no consensus exists about the optimum management of exudative Coats’ disease. The use of intravitreal anti-vascular endothelial growth factor agents as an adjunct to ablation therapy has been shown to achieve favorable outcomes, but some reports suggest an increased incidence of vitreoretinal (VR) fibrosis and tractional retinal detachment (TRD).Methods: We retrospectively reviewed records of patients presenting with exudative Coats’ disease (stages 2 and 3) from April 2016 till November 2020. Extracted data included clinical and radiological assessment, stage (Shields’ classification), interventions, and follow-up.Results: Sixteen eyes were included in the final analysis, of which 4 (25%) were stage 2 and 12 (75%) were stage 3. All eyes underwent intravitreal ranibizumab injection combined with ablation therapy, 14 (87.5%) underwent cryotherapy, 4 (25%) underwent laser ablation, 3 (18.75%) underwent external subretinal fluid drainage, and 3 (18.75%) underwent buckle or vitrectomy surgery. After a median follow-up of 16 months, 11 eyes (68.75%) had complete resolution, 4 (25%) had incomplete resolution, and only one (6.25%) progressed but did not require enucleation. Three eyes (18.75%) developed VR fibrosis, but none progressed to TRD.Conclusion: Combining intravitreal ranibizumab injection with ablation therapy is effective in managing exudative Coats’ disease. External drainage should be preserved for when ablation therapy is not feasible. Future prospective trials with pre-defined outcomes are required.Keywords: Coats’ disease, Leber’s miliary aneurysms, exudative retinal detachment, vascular endothelial growth factor, cryotherapy

To report the anatomic and functional outcomes of autologous retinal transplantation (ART).Multicenter, retrospective, interventional, consecutive case series.One hundred thirty eyes of 130 patients undergoing ART for the repair of primary and refractory macular holes (MHs), as well as combined MH-rhegmatogenous retinal detachment (MH-RRD), between January 2017 and December 2019.All patients underwent pars plana vitrectomy and ART, with surgeon modification of intraoperative variables. A large array of preoperative, intraoperative, and postoperative data was collected. Two masked reviewers graded OCT images. Multivariate statistical analysis and subgroup analysis were performed.Macular hole closure rate, visual acuity (VA), external limiting membrane and ellipsoid zone (EZ) band integrity, and alignment of neurosensory layers (ANL) on OCT.One hundred thirty ART surgeries were performed by 33 vitreoretinal surgeons worldwide. Patient demographics were: mean age of 63 ± 6.3 years, 58% female, 41% White, 23% Black, 19% Asian, and 17% Latino. Preoperative VA was 1.37 ± 0.12 logarithm of the minimum angle of resolution (logMAR; Snellen equivalent, approximately 20/500), which improved significantly to 1.05 ± 0.09 logMAR (Snellen equivalent, approximately 20/225; P0.001) after surgery (mean follow-up, 8.6 ± 0.8 months). Autologous retinal transplantation was performed for primary MH repair in 27% of patients (n = 35), for refractory MH in 58% of patients (n = 76; mean number of previous surgeries, 1.6 ± 0.2), and for MH-RRD in 15% of patients (n = 19). Mean maximum MH diameter was 1470 ± 160 μm, mean minimum diameter was 840 ± 94 μm, and mean axial length was 24.6 ± 3.2 mm. Overall, 89% of MHs closed (78.5% complete; 10% small eccentric defect), with a 95% closure rate in MH-RRD (68.4% complete; 26.3% small eccentric defect). Visual acuity improved by at least 3 lines in 43% of eyes and by at least 5 lines in 29% of eyes. Reconstitution of the EZ (P = 0.02) and ANL (P = 0.01) on OCT were associated with better final VA. Five cases of ART graft dislocation (3.8%), 5 cases of postoperative retinal detachment (3.8%), and 1 case of endophthalmitis (0.77%) occurred.In this global experience, patients undergoing ART for large primary and refractory MHs and MH-RRDs achieved good anatomic and functional outcomes, with low complication rates despite complex surgical pathologic features.