To describe the clinical characteristics, multimodal imaging findings, and treatment response in patients with idiopathic macular telangiectasia type 1 (MactTel Type 1). This retrospective cohort study involved patients affected by idiopathic MactTel Type 1. Multimodal imaging with fundus photography, optical coherence tomography (OCT), and optical coherence tomography angiography (OCT-A) and fluorescein angiography were reviewed. Treatment response to intravitreal anti-vascular endothelial growth factor (anti-VEGF) and dexamethasone (DEX) implants was evaluated. A retrospective, observational study including 10 eyes from 10 patients diagnosed with MactTel Type 1. All eyes exhibited telangiectasias and microaneurysms in the foveal region. OCT showed intraretinal cysts and macular thickening. OCT-A demonstrated a mild reduction in superficial capillary density and numerous telangiectasias in the deep capillary plexus, correlating with macular edema. Fluorescein angiography revealed prominent parafoveal capillaries with late-phase leakage. Moreover, the presence of equatorial leakage was identified in 20% of cases and vascular hyperfluorescence in 60% of cases. Complete response to treatment was observed in 40% patients. MactTel type 1 predominantly affects the temporal retinal quadrants. While anti-VEGF therapy is minimally effective, intravitreal DEX appears to be a promising alternative.

Idiopathic macular telangiectasia type 1 (MacTel 1) almost always occurs unilaterally, bilateral MacTel 1 in female is unusual and has only been described rarely in literature. This study reviews the literature on clinical and imaging presentations of bilateral MacTel 1 and treatment options. This report aims to raise awareness among clinicians regarding such rare clinical scenarios. A 76-year-old female patient presented with blurred vision in both eyes for over 5 years. She had a history of well-controlled hypertension for more than 6 years, her best-corrected visual acuity was 0.06 in the right eye and 0.25 in the left eye. Fundus examination, ultra-widefield fluorescein angiography and optical coherence tomography/optical coherence tomography angiography images supported the diagnosis of bilateral MacTel 1. She received intravitreal anti-vascular endothelial growth factor therapy in oculus uterque, and the left eye was treated with a dexamethasone intravitreal implant. The patient's condition was characterized by cystoid macular edema and hard exudate, without response to anti-vascular endothelial growth factor drug. However, a dexamethasone implant showed improvement of the cystoid macular edema, but her best-corrected visual acuity remained unchanged. This case of bilateral MacTel 1 in a female provides valuable insights into multimodal imaging studies and interventions for this uncommon condition.

We aimed to evaluate the anatomical and functional outcome of selective photocoagulation of idiopathic macular telangiectasia type 1 by navigated focal laser (Navilas, OD-OS GmBH). Consecutive patients with idiopathic macular telangiectasia type 1 were included in the analysis. All patients were treated with navigated focal laser, planned on multimodal imaging. Seven eyes of seven patients were retrospectively analyzed. Navigated laser photocoagulation of idiopathic macular telangiectasia type 1 successfully occluded the microaneurysms, inducing regression of macular edema and exudation, significative improvement in best-corrected visual acuity at 3 (P = 0.035) and 6 months (P = 0.034) and a decrease in central macular thickness at 3 (P = 0.01) and 6 months (P = 0.01). Patients with idiopathic macular telangiectasia type 1 are ideal candidates for navigated laser treatment. Navigated focal treatment has been shown to be effective and safe in occluding aneurysmal dilations without any side effects. [Ophthalmic Surg Lasers Imaging Retina 2024;55:545-551.].

To report the case of a patient with cystoid macular edema secondary to idiopathic macular telangiectasia (MacTel) Type 1, which was successfully treated by cystotomy and en bloc removal of the fibrinogen-rich component of the cystoid lesion. An 80-year-old man was referred to our department because of a visual defect in his right eye. His best-corrected decimal visual acuity was 0.7 (Snellen equivalent, 20/30). A fundus examination revealed clustered temporal juxafoveal microaneurysms and foveal cystoid macular edema. The patient refused to undergo conventional treatments, including direct retinal photocoagulation for microaneurysms, intravitreal anti-vascular endothelial growth factor injection, and intravitreal triamcinolone injection. However, he provided consent to undergo cystotomy and en bloc removal of the fibrinogen-rich component of the cystoid lesion. His best-corrected decimal visual acuity was 0.2 (Snellen equivalent, 20/100) just before the surgery. A 27-gauge vitrectomy with internal limiting membrane peeling was performed. Cystotomy was performed during the surgery, and the fibrinogen clot visible in the cystoid cavity was also removed. Cystoid macular edema rapidly disappeared after the surgery. Three years postoperatively, the patient had best-corrected decimal visual acuity of 0.5 (Snellen equivalent, 20/40) at the last medical examination, and the cystoid macular edema had not recurred. Cystotomy and en bloc removal of the fibrinogen-rich component of the cystoid lesion could be valid treatment options for cystoid macular edema secondary to MacTel Type 1.

Idiopathic macular telangiectasia type 1 (MacTel 1) almost always occurs unilaterally. In this article, we report the case of a 73-year-old man with no significant history diagnosed with MacTel 1 but with atypical bilateral presentation, something very rare in this disease. The usefulness of multimodal imaging studies, including optical coherence tomography angiography (OCTA), is highlighted to differentiate it from macular telangectasia type 2 (MacTel 2). The patient's condition was characterized by cystoid macular edema (CME) with discreet results with treatment with a dexamethasone implant. However, aflibercept therapy showed favorable results, but with recurrences when extending the doses.