The research progress of macular telangiectasia type 2 / 中华实验眼科杂志

Macular telangiectasia type 2 (MacTel type 2) is a kind of disease that characterized by the dilation of capillary in temporal macula lutea and the degeneration of neurosensory retina.It usually affects both eyes in patient aged 50 to 60.The pathophysiology of MacTel type 2 was not understood very well.With the rapid development of imaging technic and the genomic research,we have illuminated some secrets of this disease.In this article,the development of classification,fundus fluorescein angiography (FFA) and histology feature,and the characteristic of optical coherence tomography (OCT),optical coherence tomographic angiography (OCTA),macular pigment,genome about MacTel type 2 were reviewed.

Telangiectasia macular eruptiva perstans: reporte de caso y revisión de la literatura / Telangiectasia macularis eruptiva perstans: case report and literature review

La Telangiectasia Macular Eruptiva Perstans (TMEP), forma infrecuente de mastocitosis cutánea consiste en la proliferación anormal de mastocitos en dermis papilar sin participación de otros órganos. Afecta mayormente a adultos, con algunos informes en niños. Típicamente, las lesiones de TMEP son máculas eritematosas con finas telangiectasias, distribuidas principalmente en tronco y extremidades superiores con un patrón simétrico, puede presentar afectación sistémica o asociarse con neoplasias linfoproliferativas. Se presentan dos casos de TMEP en una mujer de 32 años cuyo inicio coincidió con su primera gestación y el de una mujer de 55 años, ambas sin síntomas sistémicos, se discute la presentación clínica, histopatología y tratamiento.

Macular telangiectasia eruptiva perstans (TMEP), an uncommon form of cutaneous mastocytosis, consists in the abnormal proliferation of mast cells in the papillary dermis without the participation of other organs. It affects almost exclusively adults, with some reports in children. Typically, TMEP lesions are erythematous macules with fine telangiectasias, distributed mainly in the trunk and upper limbs with a symmetrical pattern, may present systemic involvement or be associated with lymphoproliferative neoplasms. We present two cases of TMEP in a 32-year-old woman whose onset coincided with her first pregnancy and that of a 55-yearold woman, both without systemic symptoms, whose clinical presentation, histopathology and treatment are discussed.

Combination Therapy with Photodynamic Therapy and Intravitreal Bevacizumab in Idiopathic Macular Telangiectasia Type I

PURPOSE: We investigated the clinical manifestations of combination therapy with photodynamic therapy (PDT) and intravitreal bevacizumab in idiopathic macular telangiectasia type I patients. METHODS: The present study included 7 patients who visited our clinic from May 2008 to February 2011 complaining of decreasing visual acuity and diagnosed as idiopathic macular telangiectasia type I including visible aneurysms at juxtafoveal area and telangiectatic vessels leakage based on fluorescein angiography. Additionally, all patients were treated with combination therapy with PDT and intravitreal bevacizumab. RESULTS: Visual acuity improved from 0.48 ± 0.14 (log MAR) to 0.18 ± 0.17 (log MAR) after the combination therapy, however, there was no change in intraocular pressure between before (17.9 ± 3.1) and after (16.8 ± 2.3) therapy. After combination therapy, fluorescein angiography showed decreased leakage of telangiectatic vessels and optical coherence tomography showed only minimal intraretinal edema. Central subfield macular thickness decreased from 301.9 ± 50.7 μm to 193.6 ± 58.8 μm after the combination therapy. CONCLUSIONS: Combination therapy with intravitreal bevacizumab injection and PDT in patients with idiopathic macular telangiectasia type I can result in a rapid decrease of macular edema, which can lead to rapid visual recovery.

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.

Demographic Features of Idiopathic Macular Telangiectasia in Korean Patients

PURPOSE: To investigate the clinical and demographic features of idiopathic macular telangiectasia (MacTel) in Korean patients since the introduction of spectral domain optical coherence tomography (SD-OCT). METHODS: We reviewed medical records of patients who were diagnosed with MacTel from 2009 to 2013. All patients underwent fluorescein angiography and SD-OCT and were classified as type 1 or type 2 according to the classification system proposed by Yannuzzi. RESULTS: Over a period of 5 years, 4 (18.2%) patients were diagnosed with type 1 MacTel and 18 (81.8%) patients were diagnosed with type 2 MacTel. All patients with type1 MacTel were male, and their mean age was 51 +/- 8.6 years. Among patients with type 2 MacTel, 3 (16.7%) were male, 15 (83.3%) were female, and the mean age was 60 +/- 13.6 years. Whereas all type 1 MacTel patients had either metamorphopsia or mild scotoma, of the 18 patients with type 2 MacTel, only 4 (22.2%) had those symptoms, 10 (55.6%) complained of only mild visual impairment, and the other 4 (22.2%) had no symptoms. Intraretinal cystoid spaces were observed in 26 (72.2%) of 36 eyes with type 2 MacTel by SD-OCT. These cystoid spaces had irregular boundaries and did not correspond to angiographic leakages. CONCLUSIONS: Type 2 MacTel was most common in the present study. The wider availability of SD-OCT may have contributed to the diagnosis of type 2 MacTel. Type 2 MacTel may be more prevalent than type 1 in Koreans, which corresponds to the results of Western countries.

Demographic Features of Idiopathic Macular Telangiectasia in Korean Patients

PURPOSE: To investigate the clinical and demographic features of idiopathic macular telangiectasia (MacTel) in Korean patients since the introduction of spectral domain optical coherence tomography (SD-OCT). METHODS: We reviewed medical records of patients who were diagnosed with MacTel from 2009 to 2013. All patients underwent fluorescein angiography and SD-OCT and were classified as type 1 or type 2 according to the classification system proposed by Yannuzzi. RESULTS: Over a period of 5 years, 4 (18.2%) patients were diagnosed with type 1 MacTel and 18 (81.8%) patients were diagnosed with type 2 MacTel. All patients with type1 MacTel were male, and their mean age was 51 +/- 8.6 years. Among patients with type 2 MacTel, 3 (16.7%) were male, 15 (83.3%) were female, and the mean age was 60 +/- 13.6 years. Whereas all type 1 MacTel patients had either metamorphopsia or mild scotoma, of the 18 patients with type 2 MacTel, only 4 (22.2%) had those symptoms, 10 (55.6%) complained of only mild visual impairment, and the other 4 (22.2%) had no symptoms. Intraretinal cystoid spaces were observed in 26 (72.2%) of 36 eyes with type 2 MacTel by SD-OCT. These cystoid spaces had irregular boundaries and did not correspond to angiographic leakages. CONCLUSIONS: Type 2 MacTel was most common in the present study. The wider availability of SD-OCT may have contributed to the diagnosis of type 2 MacTel. Type 2 MacTel may be more prevalent than type 1 in Koreans, which corresponds to the results of Western countries.

Different treatment modalities for choroidal neovascularization in two eyes of one patient with bilateral type 2A parafoveal telangiectasia.

A 60-year-old diabetic man presented with a history of decrease in vision in both eyes since 2 weeks. At presentation, best corrected visual acuity (BCVA) in the right eye (RE) was 20/30 and that in the left eye (LE) was 20/80. The right fundus revealed a grayish reflex, yellowish crystalline deposits and retinal pigment epithelial hyperplasia at the macula. The left fundus showed subretinal fluid and temporal subretinal hemorrhage near a grayish reflex at the macula. A diagnosis in both eyes of idiopathic macular telangiectasia (IMT) type 2A with RE stage 4 and LE stage 5, choroidal neovascularization (CNVM) was made. The patient was treated with photodynamic therapy (PDT) in LE. The visual acuity improved to 20/40 over the next 6 months. At a 4-year follow-up, he developed decreased vision in RE diagnosed as IMT with CNV and was treated with intravitreal ranibizumab. At 6-month follow-up post injection, the vision was 20/40p.

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.