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Ophthalmology and Visual Sciences

Idiopathic juxtafoveal telangiectasia

Idiopathic juxtafoveal telangiectasia

Category(ies): Retina, Vitreous

Idiopathic juxtafoveal telangiectasia, type II
Contributor: Christopher Kirkpatrick, MD
Photographer: Brice Critser, CRA

Idiopathic juxtafoveal telangiectasia type II (aka macular telangiectasia) is a rare idiopathic condition that is characterized by telangiectatic vessels in the juxtafoveolar region, most commonly temporal to the fovea, of both eyes. Additional findings include graying of the parafoveal retina, superficial crystalline deposits, subfoveal cavities, and right-angle vessels. Symptoms include blurred vision, metamorphopsia and paracentral scotomas. Fluorescein angiography will highlight the parafoveal telangiectatic vessels that show early hyperfluorescence with late leakage. OCT will show subfoveal cystoid spaces without cystoid macular edema. Fundus autofluorescence is very diagnostic in that the normal hypoautofluorescence at the fovea is lost and will show abnormally increased foveal autofluorescence. These findings are all demonstrated in the left eye of the patient shown here with very similar findings in the right eye

Idiopathic juxtafoveal telangiectasia fundus photo
Fundus Photo
Idiopathic juxtafoveal telangiectasia Autofluorescence
Autofluorescence
Idiopathic juxtafoveal telangiectasia early phase
Early phase fluorescein angiogram
Idiopathic juxtafoveal telangiectasia Late phase fluorescein angiogram
Late phase fluorescein angiogram
Idiopathic juxtafoveal telangiectasia OCT
Optical coherence tomography

Juxtafoveal telangiectasia
Contributor: Andrew Doan, MD, PhD
fundus Macular pigmentation and edema
Macular pigmentation and edema
FFA with leakage
Juxtafoveal telangiectasia with leakage
magnified FFA with leakage
High magnification of fovea on FFA

Vitreoretinal traction (VRT) in the setting of Juxtafoveal Telangiectasia
Contributor: Aaron M. Ricca, MD

A 78-year-old man presented to our clinic with progressive blurring of his central vision in both eyes. Color fundus photography demonstrated a gray sheen temporal to the fovea in both eyes, which is characteristic of juxtafoveal telangiectasia (JXT). In his fovea in both eyes, there was a large, elevated round pseudo-hole due to concurrent vitreomacular traction as demonstrated by optical coherence tomography. The fundus autofluorescence images showed an absence of normal foveal hypofluorescence, replaced by an area of hyperfluorescence, a finding that is virtually pathognomonic for JXT.

Reference:
  1. Ryan SJ. Retina. 5th ed. London: Saunders/Elsevier; 2013.

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Ophthalmic Atlas Images by EyeRounds.org, The University of Iowa are licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.