Multimodality imaging in macular telangiectasia 2: A clue to its pathogenesis.

Macular telangiectasia type 2 also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A is an acquired bilateral neurodegenerative macular disease that manifests itself during the fifth or sixth decades of life. It is characterized by minimal dilatation of the parafoveal capillaries with graying of the retinal area involved, a lack of lipid exudation, right‑angled retinal venules, refractile deposits in the superficial retina, hyperplasia of the retinal pigment epithelium, foveal atrophy, and subretinal neovascularization (SRNV). Our understanding of the disease has paralleled advances in multimodality imaging of the fundus. Optical coherence tomography (OCT) images typically demonstrate the presence of intraretinal hyporeflective spaces that are usually not related to retinal thickening or fluorescein leakage. The typical fluorescein angiographic (FA) finding is a deep intraretinal hyperfluorescent staining in the temporal parafoveal area. With time, the staining may involve the whole parafoveal area but does not extend to the center of the fovea. Long‑term prognosis for central vision is poor, because of the development of SRNV or macular atrophy. Its pathogenesis remains unclear but multimodality imaging with FA, spectral domain OCT, adaptive optics, confocal blue reflectance and short wave fundus autofluorescence implicate Müller cells and macular pigment. Currently, there is no known treatment for this condition.

A Case of Bilateral Macular Hole in a Patient with Bilateral Macular Telangiectasia

PURPOSE: To report a case of bilateral macular holes in a patient with bilateral macular telangiectasia (Mac Tel). CASE SUMMARY: A 61-year-old male presented with decreased central vision in both eyes. His best corrected visual acuity (BCVA) was 0.8 in the right eye and 0.6 in the left eye. On fundoscopy, fluorescein angiography (FAG), and optical coherence tomography (OCT), he was diagnosed with Mac Tel type 2 combined with stage 1A of the right eye and stage 1B impending macular hole in the left eye. Two years and 8 months later, the BCVA of both eyes was unchanged. On fundoscopy, FAG and OCT, there were no definitive changes in both eyes. The patient was observed without treatment. CONCLUSIONS: Patients with Mac Tel type 2 may be predisposed to the development of a macular hole.

A Case of Combination Therapy for Subretinal Neovascularization in Bilateral Acquired Parafoveal Telangiectasis

PURPOSE: To evaluate the clinical results of a case of subretinal neovascularization (SRN) in bilateral acquired parafoveal telangiectasis, performed combination therapy of intravitreal bevacizumab (1.25 mg/0.05ml) and photodynamic therapy (PDT). CASE SUMMARY: A 46-year-old female presented with a decrease invisual acuity. Her best-corrected visual acuity (BCVA) was 1.0 in the right eye and 0.1 in the left eye. On ophthalmic examination, she was diagnosed with bilateral acquired parafoveal telangiectasis combined with SRN in the left eye. Intravitreal bevacizumab was injected, and then photodynamic therapy was performed 4 days later. Three months after the combination therapy, her BCVA improved to 0.5, a regression of SRN was observed and vascular leakage was markedly decreased. However, her BCVA decreased to 0.1 and SRN recurred 9 months later. CONCLUSIONS: The combined treatment of intravitreal bevacizumab and photodynamic therapy may be used as a selective alternative treatment modality for SRN in bilateral acquired parafoveal telangiectasis, although its effect is short-term.

The Clinical Manifestations and Treatments of Parafoveal Telangiectasis

PURPOSE: To investigate the clinical manifestations and treatments of parafoveal telangiectasis. METHODS: Twenty-two eyes of fifteen patients diagnosed as parafoveal telangiectasis were selected who had visited Kong Eye Center from September 1999 to August 2003 complaining of decreasing visual acuity. Visual acuity was checked and fluorescein angiography and optical coherence tomography were done. Authors treated six eyes of six patients experienced decreasing visual acuity due to macular edema with argon laser photocoagulation and one eye of one patient accompanying with choroidal neovascularization with photodynamic therapy. RESULTS: Among fifteen patients, group 1 cases were eight (1B eight) and group 2 cases were seven by classification of Gass and Blodi. Visual acuity improved in two eyes of six eyes and maintained in four eyes of six eyes and optical coherence tomography showed decrease of macular edema in six eyes of six patients all after argon laser photocoagulation. Visual acuity improved and optical coherence tomography showed decrease of macular edema in one eye of one patient after photodynamic therapy. CONCLUSIONS: Group 1 cases were more than group 2 cases. Argon laser photocoagulation can be effectively used to treat decreasing visual acuity due to macular edema in parafoveal telangiectasis patients. Photodynamic therapy seems to be effective in parafoveal telangiectasis accompanied with choroidal neovascularization but we think that additional studies with more cases are needed.