Idiopathic macular telangiectasia type 2 (idiopathic juxtafoveolar retinal telangiectasis type 2A, Mac Tel 2)

Lihteh Wu, Teodoro Evans, J Fernando Arevalo

Research output: Contribution to journalArticle

Abstract

Macular telangiectasia type 2-also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A or Mac Tel 2-is an acquired bilateral neurodegenerative macular disease that usually manifests itself during the fourth to sixth decades of life and 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). Optical coherence tomography images typically demonstrate intraretinal hyporeflective spaces that are usually not related to retinal thickening or fluorescein leakage. The typical fluorescein angiographic finding is a deep intraretinal hyperfluorescent leakage in the temporal parafoveal area. With time the leakage may involve the whole parafovea, but does not extend to the center of the fovea. Long-term prognosis for central vision is variable and depends on the development of SRNV or macular atrophy. Pathogenesis remains unclear, but Müller cells and macular pigment appear to play a central role. Currently there is no known treatment for the underlying cause of this condition, but treatment of the SRNV may be beneficial.

Original languageEnglish (US)
Pages (from-to)536-559
Number of pages24
JournalSurvey of Ophthalmology
Volume58
Issue number6
DOIs
StatePublished - Nov 2013

Fingerprint

Retinal Telangiectasis
Telangiectasis
Fluorescein
Atrophy
Venules
Retinal Pigment Epithelium
Optical Coherence Tomography
Neurodegenerative Diseases
Hyperplasia
Retina
Dilatation
Lipids
Therapeutics

Keywords

  • Choroidal neovascularization
  • Idiopathic juxtafoveal telangiectasis
  • Juxtafoveal retinal telangiectasia
  • Lutein
  • Macular edema
  • Macular pigment
  • Macular telangiectasia (Mac Tel 2)
  • Müller cells
  • Parafoveal telangiectasis
  • Perifoveal telangiectasis
  • Retinal angiomatous proliferation
  • Retinal telangiectasis
  • Subretinal neovascularization
  • Zeaxanthin

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Idiopathic macular telangiectasia type 2 (idiopathic juxtafoveolar retinal telangiectasis type 2A, Mac Tel 2). / Wu, Lihteh; Evans, Teodoro; Arevalo, J Fernando.

In: Survey of Ophthalmology, Vol. 58, No. 6, 11.2013, p. 536-559.

Research output: Contribution to journalArticle

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