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Macular telangiectasia

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131: 56: 2032: 2062: 2072: 248:(IVTA) which have proved to be beneficial in the treatment of macular edema by their anti-inflammatory effect, their downregulation of VEGF production, and stabilization of the blood retinal barrier, were reported anecdotally in the management of Macular telangiectasia type 1. In two case reports, IVTA of 4 mg allowed a transitory reduction of retinal edema, with variable or no increase in 422:, particularly in the early stages of Type 2 disease. Formerly, FA was essential in making a definitive diagnosis. However, the diagnosis can be established with less invasive imaging techniques such as Ocular Coherence Tomography (OCT) and fundus autofluorescence. Some clinicians argue that FA testing may be unnecessary when a diagnosis is apparent via less invasive means. 2052: 2042: 2098: 178: 233:(VEGF) agents. Photocoagulation was recommended by Gass and remains to date the mainstay of treatment. It seems to be successful in causing resolution of exudation and VA improvement or stabilization in selected patients. Photocoagulation should be used sparingly to reduce the chance of producing a symptomatic paracentral 241:. Small burns (100–200 μm) of moderate intensity in a grid-pattern and on multiple occasions, if necessary, are recommended. It is unnecessary to destroy every dilated capillary, and, particularly during the initial session of photocoagulation, those on the edge of the capillary-free zone should be avoided. 510:
that secrete ciliary neurotrophic factor". During Phase 2 testing, the implant device was "shown to slow the progression of retinal degeneration compared with participants who received the sham treatment. Based on the positive Phase 2 results, two parallel Phase 3 studies were initiated... enrolling
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Today, laser photocoagulation remains mostly effective, but the optimal treatment of Macular telangiectasia type 1 is questioned, and larger series comparing different treatment modalities seem warranted. The rarity of the disease, however, makes it difficult to assess in a controlled randomized
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originally identified four types of idiopathic juxtafoveolar retinal telangiectasis in 1982, contemporary researchers describe three types collectively known as idiopathic juxtafoveal telangiectasia: macular telangiectasia type 1, macular telangiectasia type 2, and macular telangiectasia type 3.
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The most crucial aspect of treating macular telangiectasia is recognition of the clinical signs. This condition is relatively uncommon: hence, many practitioners may not be familiar with or experienced in diagnosing the disorder. MacTel must be part of the differential in any case of idiopathic
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isoforms, have shown improved visual outcome and reduced leakage in macular edema from diabetes and retinal venous occlusions. In one reported patient with Macular telangiectasia type 1, a single intravitreal bevacizumab injection resulted in a marked increase in VA from 20/50 to 20/20, with
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The natural history of macular telangiectasia suggests a slowly progressive disorder. A retrospective series of 20 patients over 10 to 21 years showed deterioration of vision in more than 84% of eyes, either due to intra-retinal edema and serous retinal detachment (Type 1) or retinal pigment
433:. Recognition of this condition can save an affected patient from unnecessarily undergoing extensive medical testing and/or treatment. MacTel should be considered in cases of mild paramacular dot and blot hemorrhages and in cases of macular and paramacular retinal pigment epithelium (RPE) 735:
Copyright © Middle East African Journal of Ophthalmology. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
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of triamcinolone acetonide has also been reported in a limited number of patients with Macular telangiectasia type 1 with improvement or stabilization of vision after a mean follow-up of 10 months. Further studies are needed to assess the efficacy of this treatment modality.
295:(OCT) up to 12 months. It is likely that patients with Macular telangiectasia type 1 with pronounced macular edema from leaky telangiectasis may benefit functionally and morphologically from intravitreal anti-VEGF injections, but this warrants further studies. 252:(VA). As expected with all IVTA injections, the edema recurred within 3–6 months, and no permanent improvement could be shown. In general, the effect of IVTA is short-lived and complications, mainly increased intraocular pressure and cataract, limit its use. 32: 1037:
Charbel Issa, Peter; Finger, Robert P.; Kruse, Kathrin; Baumüller, Sönke; Scholl, Hendrik P.N.; Holz, Frank G. (2011). "Monthly Ranibizumab for Nonproliferative Macular Telangiectasia Type 2: A 12-Month Prospective Study".
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Typical patients present with a mean age of 55–59 years, and a wide range of symptoms from totally asymptomatic to substantially impaired; in most cases however, patients retain functional acuity of 20/200 or better.
527:"Idiopathic macular telangiectasia type 3 is a rare, acquired, eye disease characterized by progressive visual loss, due to bilateral juxtafoveolar capillary occlusions, capillary telangiectasia, and minimal 977:
Gantner, Marin L.; Eade, Kevin; Wallace, Martina; Handzlik, Michal K.; Fallon, Regis; Trombley, Jennifer; Bonelli, Roberto; Giles, Sarah; Harkins-Perry, Sarah; Heeren, Tjebo F.C.; Sauer, Lydia (2019-10-10).
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Although MacTel has been previously regarded as a rare disease, it is in fact probably much more common than previously thought. The subtleness of early symptoms of MacTel may cause mis-diagnosis by
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Areas of focal RPE hyperplasia (pigment plaques) often develop in the paramacular region as a response to these abnormal vessels. Other signs of macular telangiectasia type 2 include right angle
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MacTel may also be referred to by various names, including (idiopathic) juxtafoveolar telangiectasia, or parafoveal telangiectasis, depending on the source. All refer to the same disease.
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manner. A 2014 case study of a single type 1 macular telangiectasia patient concluded Bevacizumab was not effective, but Aflibercept produced beneficial results.
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Type 3 is an extremely rare, poorly understood neurological disease of the retina. It is characterized by occlusion and telangiectasia of the capillaries of the
72:, the light-sensing tissue at the back of the eye that causes gradual deterioration of central vision, interfering with tasks such as reading and driving. 412:, representing an unusual alteration of the vasculature in the paramacular area, with vessels taking an abrupt turn toward the macula as if being dragged. 2128: 1561: 1902: 2123: 807:
Charbel Issa, Peter; Gillies, Mark C.; Chew, Emily Y.; Bird, Alan C.; Heeren, Tjebo F.C.; Peto, Tunde; Holz, Frank G.; Scholl, Hendrik P.N. (2013).
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patients in the United States, Australia, and Europe to determine the safety and efficacy... for the treatment of MacTel". In February 2019, the
502:(CNTF) is believed to have neuroprotective properties and could thus be able to slow down the progression of MacTel type 2. According to the 1801: 856:
Heeren, Tjebo F. C.; Holz, Frank G.; Issa, Peter Charbel (2014). "First Symptoms and Their Age of Onset in Macular Telangiectasia Type 2".
1937: 1279: 477: 230: 1099:"Neurotech Pharmaceuticals, Inc. Granted Fast Track Designation from the U.S. FDA for the Treatment of Macular Telangiectasia type 2" 503: 445:
In September 2019, researchers announced, "a combination of genetics, metabolism and biochemistry drives a disease such as MacTel".
2055: 1927: 1361: 953: 255: 2088: 1907: 512: 395: 292: 1551: 499: 488:(Avastin) may reduce vascular leakage, but neural atrophy may still occur in treated eyes. In proliferative stages ( 1531: 1251: 1128: 1960: 1177:"Idiopathic Macular Telangiectasia Type 3 disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials" 1752: 1674: 1669: 1589: 1349: 1074: 1732: 1706: 415: 380: 288: 245: 106: 1152: 556: 130: 1324: 1272: 1176: 750:"Macular Oedema in Idiopathic Macular Telangiectasia Type 1 Responsive to Aflibercept but Not Bevacizumab" 539:
Type 3 macular telangiectasia is also called "occlusive idiopathic juxtafoveolar retinal telangiectasis".
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Bonelli R (2019): Integration of multi-'omics data identifies genetic & metabolic causes of MacTel
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and loss of central vision. The two diseases may be distinguished by symptoms, clinical features, and
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Macular telangiectasia type 2 is commonly under-diagnosed. The findings may appear very similar to
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Type 1 idiopathic macular telangiectasia patients are typically males, 40 years old or older.
631:"Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study" 2035: 1917: 1887: 1866: 1829: 1812: 1727: 1691: 1681: 1576: 1496: 1440: 1403: 1388: 1329: 1296: 1265: 1227: 1055: 1019: 1001: 934: 873: 838: 789: 771: 726: 708: 658: 650: 611: 603: 308: 258:-guided laser photocoagulation directed at the leaky microaneurysms and vessels combined with 210: 139: 95: 80: 79:
in the retina, typically affects a single eye in male patients, and it may be associated with
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Diagnosis of MacTel type 2 may be aided by the use of advanced imaging techniques such as
354:(visual distortions) may be a subjective complaint. Due to the development of paracentral 113: 1897: 1851: 1664: 1594: 1413: 1014: 979: 929: 904: 833: 808: 784: 749: 721: 686: 373: 369: 351: 238: 91: 76: 646: 398:. These can help to identify the abnormal vessels, pigment plaques, retinal crystals, 2112: 1980: 1769: 1764: 1626: 1566: 1469: 824: 459: 366:
Type 2 MacTel commonly occurs in both eyes, and occurs equally in males and females.
249: 49: 1098: 885: 2045: 1985: 1970: 1796: 1521: 1371: 1356: 1302: 900: 326: 315: 158: 519:" designation for approval of the device to treat macular telangiectasia type 2. 1222: 1205: 920: 869: 598: 358:(blind spots), impaired reading ability may be the first symptom of the disease. 1844: 1789: 1686: 1541: 1516: 1334: 485: 481: 463: 462:
or focal pigment hypertrophy, especially in those patients without a history of
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inhibitors have not proven to be effective in non-proliferative MacTel type 2.
1817: 1784: 1556: 1506: 1491: 1486: 1479: 1464: 1454: 1245: 1051: 507: 455: 311: 275: 1005: 905:"Perifoveal Müller Cell Depletion in a Case of Macular Telangiectasia Type 2" 775: 712: 654: 607: 2005: 1747: 1742: 1606: 1584: 1459: 1423: 703: 493: 384: 198: 31: 1231: 1059: 1023: 938: 877: 842: 793: 730: 630: 615: 429:, and many cases have been incorrectly ascribed to diabetic retinopathy or 996: 980:"Serine and Lipid Metabolism in Macular Disease and Peripheral Neuropathy" 766: 662: 531:. It is associated with systemic or cerebral vascular occlusive disease." 1511: 1288: 469:
Treatment options are limited. Laser therapy may actually enhance vessel
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region, leading to vascular incompetence. Telangiectatic vessels develop
109:. It generally affects both eyes and usually affects both sexes equally. 102: 1990: 1450: 1418: 1311: 1129:"Novel implant for macular telangiectasia gains fast-track designation" 899:
Powner, Michael B.; Gillies, Mark C.; Tretiach, Marina; Scott, Andrew;
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Christakis, Panos G.; Fine, Howard F.; Wiley, Henry E. (2019-03-22).
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Nowilaty, Sawsan R.; Al-Shamsi, Hanan N.; Al-Khars, Wajeeha (2010).
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Shibeeb, O'Sam; Vaze, Anagha; Gillies, Mark; Gray, Timothy (2014).
687:"Idiopathic Juxtafoveolar Retinal Telangiectasis: A Current Review" 206: 176: 209:. Macular telangiectasia type 1 has been called as a variant of 1261: 282:, a FDA-approved monoclonal antibody fragment that targets all 266:
Recently, intravitreal injections of anti-VEGF agents, namely
94:. It is categorized as "macular perifoveal telangiectasia", a 1257: 405:
and intraretinal cavities associated with this disorder.
1206:"The Diagnosis and Management of Macular Telangiectasia" 582:"The Diagnosis and Management of Macular Telangiectasia" 473:
and promote intraretinal fibrosis in these individuals.
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Contemporary research has shown that MacTel is likely a
197:(a focal expansion or outpouching) and dilation in the 287:
significant and sustained decrease in both leakage on
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Cystoid Macular Oedema in patient with MacTel type 1
1948: 1886: 1647: 1575: 1530: 1439: 1310: 1295: 43: 24: 903:; Hageman, Gregory S.; Fruttiger, Marcus (2010). 134:Diagram of the human eye showing macula and fovea 1204:Christakis PG, Fine HF, Wiley HE (March 2019). 193:Type 1 may be defined as an acquired capillary 16:Disease of the retina affecting central vision 1273: 586:Ophthalmic Surgery, Lasers and Imaging Retina 418:(FA) is helpful in identifying the anomalous 8: 691:Middle East African Journal of Ophthalmology 221:Treatment options for Type 1 include laser 1562:Reproductive endocrinology and infertility 1307: 1280: 1266: 1258: 629:Gass, J. D.; Blodi, B. A. (October 1993). 205:that leak fluid, blood, and occasionally, 54: 30: 21: 1903:Bachelor of Medicine, Bachelor of Surgery 1221: 1013: 995: 928: 832: 783: 765: 754:Case Reports in Ophthalmological Medicine 720: 702: 597: 437:where no other cause can be identified. 376:markers in the clinically altered area. 129: 2093: 548: 75:Type 1, a very rare disease involving 1210:Ophthalmic Surg Lasers Imaging Retina 952:Labios, Liezel (September 11, 2019). 116:in one or both eyes, as well as some 7: 2041: 1802:Physical medicine and rehabilitation 813:Progress in Retinal and Eye Research 680: 678: 676: 674: 672: 2051: 314:disease. MacTel is correlated with 165:epithelium (RPE) scar formation or 1938:Medical Scientist Training Program 466:or contributory systemic disease. 291:(FA) and cystoid macular edema on 231:vascular endothelial growth factor 14: 2129:Visual disturbances and blindness 1133:American Academy of Ophthalmology 1040:American Journal of Ophthalmology 513:U.S. Food and Drug Administration 504:American Academy of Ophthalmology 2096: 2070: 2060: 2050: 2040: 2031: 2030: 825:10.1016/j.preteyeres.2012.11.002 431:age-related macular degeneration 383:(which may identify an abnormal 148:age-related macular degeneration 146:Both macular telangiectasia and 2124:Disorders of choroid and retina 2071: 984:New England Journal of Medicine 809:"Macular telangiectasia type 2" 1928:Doctor of Osteopathic Medicine 1362:Oral and maxillofacial surgery 1: 647:10.1016/S0161-6420(93)31447-8 372:studies have shown a loss of 260:sub-Tenon’s capsule injection 256:Indocyanine green angiography 90:) is the most common macular 1908:Bachelor of Medical Sciences 1675:Neurosurgical anesthesiology 1223:10.3928/23258160-20190301-02 1127:Lee, Keng Jin (2019-02-15). 921:10.1016/j.ophtha.2010.04.001 870:10.1097/IAE.0000000000000082 599:10.3928/23258160-20190301-02 293:optical coherence tomography 500:Ciliary neurotrophic factor 244:Intravitreal injections of 2145: 2026: 1052:10.1016/j.ajo.2010.11.019 38: 29: 1862:Transplantation medicine 1753:Clinical neurophysiology 1670:Obstetric anesthesiology 1590:Interventional radiology 1350:Digestive system surgery 454:paramacular hemorrhage, 278:, circulatory VEGF; and 1733:Intensive care medicine 1707:Mass gathering medicine 1552:Maternal–fetal medicine 704:10.4103/0974-9233.65501 416:Fluorescein angiography 381:fluorescein angiography 289:fluorescein angiography 246:triamcinolone acetonide 227:intravitreal injections 107:coronary artery disease 86:Type 2 (referred to as 1325:Cardiothoracic surgery 1254:(video, 58:08 minutes) 476:Monthly injections of 182: 135: 68:is a condition of the 66:Macular telangiectasia 39:Macular telangiectasia 25:Macular telangiectasia 1976:Personalized medicine 1835:Reproductive medicine 1760:Occupational medicine 1714:Evolutionary medicine 997:10.1056/NEJMoa1815111 274:targeted against pro- 229:of steroids, or anti- 180: 133: 1996:Traditional medicine 1956:Alternative medicine 1823:Addiction psychiatry 1637:Transfusion medicine 1632:Medical microbiology 1547:Gynecologic oncology 1399:Reproductive surgery 427:diabetic retinopathy 2018:History of medicine 2001:Veterinary medicine 1808:Preventive medicine 1660:Adolescent medicine 1502:Infectious diseases 1108:. 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Index


Specialty
Ophthalmology
Edit this on Wikidata
retina
microaneurysms
Coats' disease
telangiectasia
neurodegenerative
metabolic disorder
diabetes
coronary artery disease
fovea
exudation

J. D. Gass
age-related macular degeneration
photoreceptor
atrophy
pathogenesis
neovascularization

ectasia
parafoveal
micro-aneurysms
lipid
Coats' disease
photocoagulation
intravitreal injections
vascular endothelial growth factor

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