Bilateral Type 1 Idiopathic Macular Telangiectasia in a Female Patient: Multimodal Imaging of a Rare Presentation

We report a bilateral case of type 1 idiopathic macular telangiectasia (IMT) in a female patient. A 40-year-old otherwise healthy female with gradual vision loss since 2 years ago with best-corrected visual acuity of 20/400 in both eyes was referred. Her past medical history was negative for any systemic disease including diabetes and systemic hypertension. Color fundus photography showed bilateral loss of normal foveal reflex with macular edema. Fluorescein angiography demonstrated symmetric perifoveal telangiectasia mainly in the superior and nasal macula in both eyes with late staining and leakage. Spectral-domain optical coherence tomography revealed significant intraretinal fluid bilaterally and subretinal fluid in the left eye. Optical coherence tomography angiography (OCTA) revealed obvious saccular parafoveal capillary telangiectasia and capillary dropout as well as decreased vascular density in both superficial and deep capillary plexus. Deep capillary plexus involvement in OCTA was more evident than superficial plexus. Based on the patient’s medical history and multimodal imaging, the diagnosis of bilateral IMT type 1 was made. The patient underwent 5 intravitreal monthly injection of bevacizumab in both eyes, which resulted in macular edema resolution. However, after 3 months of discontinuation of intravitreal bevacizumab, macular edema relapsed. In conclusion, type 1 IMT can occur bilaterally in an otherwise healthy female patient as a very rare presentation. To the best of our knowledge, this case is the 4th case of bilateral type 1 IMT reported in a female.

Multimodal Imaging Findings and Treatment with Dexamethasone Implant in Three Cases of Idiopathic Macular Telangiectasia Type 1

This is a report of 3 patients diagnosed with idiopathic macular telangiectasia type 1 (MacTel 1) at Medical Retina Center of the University of Eye Clinic of Genova and then prospectically followed for a mean time of 26 months between 2016 and 2019. It is the first report of patients affected by MacTel 1 treated with dexamethasone (DEX) implant as a first choice of treatment. Aim of our study is to better characterize the disease using a multimodal wide-field imaging and to determine efficacy of DEX implant on MacTel 1 in terms of central macular thickness (CMT) and best-corrected visual acuity (BCVA). MacTel 1 is a rare unilateral disease, characterized by telangiectatic retinal capillaries, cystoid macular edema, and lipid deposition occurring temporal to the fovea. Patients underwent a comprehensive ophthalmic examination, BCVA, swept-source optical coherence tomography (SS-OCT), SS-OCT angiography (SS-OCTA), ultra-widefield (UWF) color, and fluorescein angiography (FA) fundus photograph. All the patients presented monolateral reduced BCVA and macular edema with increased CMT evaluated by SS-OCT. With SS-OCTA, we showed that the telangiectasia-associated vascular changes originate in the deep retinal vascular plexus and as a consequence macular edema and exudation develop causing vision loss. Furthermore, UWF imaging helped us to highlight vascular changes typical of Coats Disease at the far retinal periphery. All the patients were treated with DEX intravitreal implant, showing a decrease in CMT and a stabilization of visual acuity. Due to the recurrent nature of macular edema, patients underwent a mean of 4 DEX implants during the follow-up period. In order to address the clinical features of this uncommon disease avoiding diagnostic errors, it might be important to use a multimodal imaging approach. The anatomical and functional beneficial effects of DEX implant were well evident although transient.

Ultra-Widefield Imaging of Peripheral Vascular Abnormalities in Idiopathic Macular Telangiectasia Type 1

Purpose: This work reports unusual peripheral vascular anomalies on ultra-widefield angiography in a case of idiopathic macular telangiectasia (IMT) type 1. Methods: A case report is discussed. Results: A 51-year-old woman with a 6-year history of IMT type 1 presented with ongoing vision symptoms from persistent macular edema despite extended antivascular endothelial growth factor therapy. Examination and macular imaging findings were consistent with a diagnosis of IMT type 1, and ultra-widefield angiography additionally demonstrated nonperfusion, terminal vascular loops, and arteriovenous anastomosis without exudation or telangiectasis. The patient’s condition remained stable after she elected observation. Conclusion: While extrafoveal vascular anomalies are a known feature of IMT type 1, they commonly consist of peripheral telangiectasias and exudation. Describing new peripheral vascular abnormalities in IMT adds to our knowledge of this condition and could be of value for diagnosis, management, and further study.

Efficacy of Laser Photocoagulation for Type 1 Idiopathic Macular Telangiectasia: A Retrospective Study

Aims: The aim of this study was to evaluate the efficacy of laser photocoagulation for type 1 idiopathic macular telangiectasia (MacTel). Study Design: Retrospective case series Place and Duration of Study: Department of Ophthalmology, Oita University Hospital during the period from 2004 to 2013. Patients and Methods: Six eyes of 6 patients with type 1 MacTel diagnosed consecutively in the Oita University Hospital during the period from 2004 to 2013 were studied. They showed macular edema and their visual acuity were deteriorated due to leakage from the aneurysms. Four patients were male and two were female with an average age of 64 years (range: 41 to 74 years). Informed consent was obtained from each patient and the laser photocoagulation was applied to leaky aneurysms in all eyes. The central macular thickness (CMT) was measured by optical coherence tomography (OCT). Mean follow up period was 44 months (range: 24 - 63 months). Results: Mean visual acuity significantly increased from logMAR 0.25±0.27 at baseline to logMAR 0.11±0.14 (P<0.05) at the last visit. Visual acuity improved more than 0.2 logMAR in five eyes and was stable (gain or loss of < 0.3 logMAR) in one eye. Mean central macular thickness significantly decreased from 454±181 μm at baseline to 231±67 μm (P<0.05) at the last visit. Macular edema was not detected by OCT inany patients at the last visit. Conclusion: In type I MacTel, laser photocoagulation may achieve a visual improvement and normalize the central macular thickness.

Reduced foveal cone density in early idiopathic macular telangiectasia

ObjectiveIdiopathic macular telangiectasia (MacTel) is considered primarily a vascular disease affecting juxtafoveal retinal capillaries. However, recent evidence suggests that neuronal changes may occur early in disease development. We used high-resolution adaptive optics retinal imaging to elucidate the foveal cone photoreceptor changes at a cellular level in patients with MacTel.Methods and analysisWe used adaptive optics scanning light ophthalmoscopy (AOSLO) to evaluate the foveal cone photoreceptors in the less-affected eye of patients with asymmetric MacTel. AOSLO images of cone photoreceptors were obtained in a 4°×4° area centred on the foveola. Individual cone positions were identified within a 2°×2° area centred on the fovea, using semiautomatic cone marking software with manual correction, permitting calculation of a map of cone density.ResultsIn all participants, one eye was affected with MacTel, the fellow eye was clinically normal or near normal, with visual acuity of 20/25 or better and subtle angiographic leakage. The foveal cone mosaics were continuous with tight packing and cones exhibited normal reflectivity. However, cone density was significantly lower for all participants (mean=80 733 cones/mm2) within 0.5° than the cone density previously reported for normal eyes.ConclusionsFoveal cone density is lower than normal in the clinically less-affected eyes of patients with asymmetric MacTel. This suggests that cone photoreceptor loss may precede classic obvious vascular changes in idiopathic MacTel.