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PurposePatients with non-proliferative macular telangiectasia type 2 (MacTel) have ganglion cell layer (GCL) and nerve fibre layer (NFL) loss, but it is unclear whether the thinning is progressive. We quantified the change in retinal layer thickness over time in MacTel with and without diabetes.MethodsIn this retrospective, multicentre, comparative case series, subjects with MacTel with at least two optical coherence tomographic (OCT) scans separated by >9 months OCTs were segmented using the Iowa Reference Algorithms. Mean NFL and GCL thickness was computed across the total area of the early treatment diabetic retinopathy study grid and for the inner temporal region to determine the rate of thinning over time. Mixed effects models were fit to each layer and region to determine retinal thinning for each sublayer over time.Results115 patients with MacTel were included; 57 patients (50%) had diabetes and 21 (18%) had a history of carbonic anhydrase inhibitor (CAI) treatment. MacTel patients with and without diabetes had similar rates of thinning. In patients without diabetes and untreated with CAIs, the temporal parafoveal NFL thinned at a rate of −0.25±0.09 µm/year (95% CI [−0.42 to –0.09]; p=0.003). The GCL in subfield 4 thinned faster in the eyes treated with CAI (−1.23±0.21 µm/year; 95% CI [−1.64 to –0.82]) than in untreated eyes (−0.19±0.16; 95% CI [−0.50, 0.11]; p<0.001), an effect also seen for the inner nuclear layer. Progressive outer retinal thinning was observed.ConclusionsPatients with MacTel sustain progressive inner retinal neurodegeneration similar to those with diabetes without diabetic retinopathy. Further research is needed to understand the consequences of retinal thinning in MacTel.

This case report describes a vasoproliferative tumor in a 16-year-old male patient with Coats' disease, treated with laser photocoagulation, cryoablation, and anti-vascular endothelial growth factor injections therapies, that developed an epiretinal membrane as a complication. The surgical treatment in association with the retinal laser treatment greatly improved visual acuity. At 8 months follow-up from surgery, visual acuity had significantly improved, and macular anatomy recovered. Vasoproliferative tumors may occur in patients with Coats' disease, and in such a clinical scenario, a multiple therapeutical approach should be considered, in order to reach the best anatomical and functional outcome for the patient.

A six-year-old boy with history of Coats' disease and cystoid macular edema (CME) was treated with a combination of peripheral retinal laser, anti-vascular endothelial grown factor injections, and short-acting injectable steroids to control his disease process. Given the excellent response to shorter acting steroids and the need for general anesthesia for each procedure, the patient underwent placement of the injectable fluocinolone acetonide implant 0.18 mg (YUTIQ) with control of his CME at 30 months of follow-up. The authors conclude that a long-acting steroid implant may be beneficial in select patients with Coats' disease. [Ophthalmic Surg Lasers Imaging Retina 2024;55:679–681.]

This study investigated the surgical outcomes of Coats disease and the role of external drainage (XD) of subretinal fluid (SRF). The study is a multicenter retrospective interventional case series of 26 consecutive eyes of 26 patients who underwent surgeries for advanced Coats disease with retinal detachment. Main outcomes measured were: 1) comparison of complete SRF resolution with or without XD, and 2) variables that were associated with functional postoperative best-corrected visual acuity (BCVA) defined as BCVA of 0.1 or better, 3) intraocular vascular endothelial growth factor (VEGF) levels. Complete SRF resolution was achieved in all 14 eyes in which XD had been performed and in 75% of 12 eyes in which XD had not been performed ( P  = .03). Multivariable logistic regression analysis revealed that initial BCVA was the only variable associated with functional postoperative BCVA (odds ratio 3.24, 95% CI 0.93–11.33; P  = .04). Markedly elevated VEGF levels were noted in the SRF compared with those in the vitreous humor (49,760 ± 52,990 vs. 707 ± 611 pg/mL, P  = .03). XD seems to provide better anatomical success than without XD in the treatment of advanced Coats disease as XD could effectively eliminate substantial amount of VEGF in the SRF.

Background This report aims to present the causes, clinical features, and surgical outcomes of persistent exudative retinal detachment (ERD) in a single tertiary referral center experience. Methods We retrospectively analyzed 48 patients who underwent vitreoretinal intervention for persistent ERD between 2017 and 2024. The study assessed patient demographics, underlying causes of ERD, the success rate of surgical interventions, and postoperative visual outcomes. Results Of the 2040 eyes with retinal detachment treated at our center, 48 (2.35%) were diagnosed with ERD. Coats disease was the most frequent underlying cause, identified in 18 (37.5%) of ERD cases. Choroidal hemangioma ( n  = 6, 12.5%) and nanophthalmos ( n  = 5, 10.4%) were the next most common etiologies. The study population comprised 48 patients (30 males and 18 females) with a mean age of 37.48 years (range, 4–88 years). The mean best-corrected visual acuity remained stable from baseline to follow-up. The recurrence rate following primary surgery was 29.2%. Patients underwent a mean of 1.75 ± 1.00 surgeries. Globe preservation was achieved in 44 eyes (91.7%), while phthisis bulbi developed in 4 eyes (8.3%). Conclusion Vitreoretinal surgery may be considered as a salvage procedure in cases where persistent ERD threatens the posterior pole. In our study, Coats’ disease, choroidal hemangioma, and nanophthalmos were identified as the most common etiologies associated with persistent ERD. Surgical intervention has the potential to preserve vision, but the high recurrence rate necessitates a cautious approach and the possibility of multiple surgeries.

Idiopathic macular telangiectasia (MacTel II) is a degenerative disorder of the Müller cells, characterised by pigment proliferation and retinal degeneration. We present a case of an elderly woman with bilateral asymmetric loss of vision. Fundus examination in the right eye revealed profuse pigment proliferation at the macula and pigment plaques temporal to the macula in the left eye, with bilateral retinal greying and right-angled venules. Optical coherence tomography and optical coherence tomography angiography helped identify this condition as bilateral MacTel II with outer retinal neovascularisation and subretinal neovascularisation. Over 1 year of follow-up, progressive vision loss and neovascular changes were noted in the left eye compared with the stable vision and minimal neovascular changes in the right eye. We explore the possible role of pigment proliferation in preventing the progression of neovascularisation in eyes with MacTel II.

Purpose: Coats' disease is associated with poor outcomes, and there are limited studies on long-term outcomes of Coats' disease. The purpose of our study is to identify various predictive factors to help in prognosticating the treatment outcomes in advanced Coats' disease in children. Methods: This is a retrospective case series from a single tertiary eye care center of children (<18 years) diagnosed with coat's disease. Sixty-seven patients with Coat's disease were identified from the medical records from 2009 to 2020. Patients' demographic data, clinical presentation, stage, extent of involvement, detailed treatment history, clinical sequelae post-treatment (including complications and anatomical and functional outcomes) were noted. Binary logistic regression was performed to correlate the predictive factors for anatomical and functional improvement. Results: Of the 67 patients, 51 eyes of 51 patients were included in the study. The male to female proportion was 2.2. Mean age at presentation was 4.98 ± 3.55 years (range: 2 months-15 years). Mean duration of follow-up was 31.53 ± 26.38 months. Overall, our globe salvage rate was 92.2%. We found that vitreoretinal fibrosis (P < 0.001), subretinal gliosis (P < 0.001), vitreous hemorrhage (P = 0.02), tractional or combined retinal detachment (P < 0.001), foveal scar (P < 0.006), and cataract (P < 0.001) to be important factors to affect the outcome. Conclusion: Advanced stage of presentation (stage 3B and above), diffuse involvement, cataract, vitreoretinal fibrosis (preretinal and subretinal), vitreous hemorrhage, tractional or combined retinal detachment, and anterior hyaloid proliferation are poor prognostic factors for globe salvage in advanced disease. Subretinal gliotic nodule or scar and lack of visual rehabilitation suggest poor functional outcomes.

Coats-like response refers to a condition where abnormal telangiectatic retinal vessels and aneurysms associated with subretinal exudation are seen in the setting of other ocular or systemic diseases. So far, it has been described with various ocular disorders like retinitis pigmentosa, chronic ischemic branch retinal vein obstruction and pars planitis. A man in his 30s presented with a 1-month history of diminution of vision in the left eye. On examination, the left eye fundus showed extensive subretinal exudation along with evidence of old and recent periphlebitis. Fluorescein angiography revealed multiple telangiectatic vessels and peripheral aneurysms along with perivenular leak. Optical coherence tomography of the macula demonstrated cystoid oedema with subretinal fluid. The case was diagnosed as Coats-like response with occlusive periphlebitis and was successfully managed with oral steroids alone.

Objectives: To evaluate the long-term progression of macular telangiectasia type 2 (MacTel) using a standardized classification system and to assess the incidence, progression, and management strategies of complications such as macular neovascularization (MNV) and macular hole (MH). Materials and Methods: This retrospective study analyzed the medical records of patients diagnosed with MacTel at a tertiary referral center in Turkiye from January 2004 to February 2025. Patients with a minimum follow-up of 3 years and no confounding macular pathologies were included. Data collection included best corrected visual acuity (BCVA), multimodal imaging (optical coherence tomography [OCT], fundus autofluorescence, fluorescein angiography), and demographic variables. Disease severity was classified using the MacTel Classification System developed by Chew et al. Longitudinal changes in BCVA and OCT parameters were statistically analyzed. Results: A total of 184 eyes from 94 patients (mean age: 63.89[+ or -]9.98 years; mean follow-up: 79.27[+ or -]50.69 months) were included. A significant decline in BCVA was observed (p<0.001). MNV was present in 29 eyes (15.8%), with 18 receiving intravitreal anti-vascular endothelial growth factor (anti-VEGF) therapy (mean injections: 5.89[+ or -]3.72). While post-treatment BCVA showed improvement (p<0.001), long-term visual outcomes were not significantly different from baseline (p = 0.213). MH formation occurred in 8 eyes (4.3%), with 6 undergoing successful surgical closure. Structural retinal changes, including ellipsoid zone disruption and pigmentation, significantly progressed over time (p<0.001). Conclusion: MacTel demonstrates a progressive decline in visual and structural integrity over extended follow-up. While anti-VEGF therapy offers short-term benefits for MNV, its long-term efficacy remains limited. MH development, though rare, poses a significant challenge, with variable surgical outcomes. Keywords: Macular telangiectasia type 2, macular neovascularization, macular hole