June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Unilateral Occlusive Juxtafoveolar Telangiectasis: A 27-year Natural History Study
Author Affiliations & Notes
  • Sawsan R Nowilaty
    Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
  • Dhoha M Alhamad
    Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
  • Footnotes
    Commercial Relationships   Sawsan Nowilaty, None; Dhoha Alhamad, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 3680. doi:
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      Sawsan R Nowilaty, Dhoha M Alhamad; Unilateral Occlusive Juxtafoveolar Telangiectasis: A 27-year Natural History Study. Invest. Ophthalmol. Vis. Sci. 2017;58(8):3680.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Occlusive juxtafoveolar telangiectasis (JFT group 3 of Gass and Blodi) is a rare and classically bilateral condition. Its stages of development and long-term natural history are not well described. The purpose of this report is to demonstrate the evolution of the foveal capillary obliterations and telangiectasis observed in a unilateral case followed for 27 years.

Methods : Prospective retinal clinical, photographic and fluorescein angiographic documentation through 27 years of follow up of an occlusive JFT in the right eye of a 48-year-old man.

Results : At presentation, visual acuity in the right eye was 20/30. Visibly obliterated perifoveal capillaries, irregular enlargement of foveal avascular zone (FAZ), parafoveal telangiectasis with aneurysmal dilations of terminal capillaries and minimal late staining on fluorescein angiography without macular edema were observed. Systemic work up for diabetes, hypertension, retinal vein occlusion, sickling hemoglobinopathy, prior irradiation, collagen vascular disease, carotid artery stenosis or central nervous system vasculopathy remained negative.
Through the next 27 years, a slow progressive retinal capillary dropout continued centrifugally in each parafoveal sector according to a predictable pattern starting with tiny intraretinal hemorrhages, then segmental narrowing and whitening of the precapillary arterioles, followed by obstruction of the arteriolar segment on angiography and replacement with capillary remodeling and aneurysmal dilations. The latter disappeared progressively from the center as the obliterative process extended centrifugally to adjacent arterioles. No ischemic retinal whitening or edema were observed. After 27 years, the FAZ has enlarged to an oval zone measuring 4 disc areas, bordered by blunted obliterated capillaries and terminal capillary aneurysmal dilations with no macular edema. Visual acuity was 20/100. Left eye stayed normal with a visual acuity of 20/20.

Conclusions : In occlusive juxtafoveolar telangiectasis retinal precapillary obliterations precede the visible retinal capillary telangiectasis and aneurysmal dilations. The obliterative process is slow but relentless, and as it expands centrifugally, centrally located capillary channels and aneurysms disappear while new capillary telangiectasis and aneurysmal dilations develop at the border of the enlarged foveal avascular zone.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

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