Case Reports and Small Case Series
March 1999

Idiopathic Juxtafoveolar Retinal Telangiectasis and Pigment Epithelial Hyperplasia: An Optical Coherence Tomographic Study

Author Affiliations

Copyright 1999 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.1999

Arch Ophthalmol. 1999;117(3):405-406. doi:

One patient with bilateral idiopathic juxtafoveolar retinal telangiectasis (IJRT) revealing pigment hyperplasia in 1 eye was investigated by means of fluorescein angiography and optical coherent tomography (OCT).

Report of a Case.

A 63-year-old man was referred to our department for gradual loss of central vision in both eyes. The visual acuity was 0.6 in right eye and 0.3 in the left eye. The Amsler test result was negative in both eyes. Biomicroscopic evaluation revealed in the right eye a small black stellate plaque temporal to the macula (Figure 1). In the left eye, a small intraretinal hemorrhage was revealed temporal to the macula. Early phases of fluorescein angiography showed minimal evidence of capillary dilation, while in the late phases, a mild staining surrounding the foveolar retina temporally was evident. This aspect was more marked in the right eye. The retina in the macular area of both eyes appeared flat by the OCT. The small plaque of pigment epithelial hyperplasia, in the right eye, was revealed by the OCT line scans as a highly reflective white-red area within the inner retina (Figure 2). A dark intraretinal area was detected under the hyperreflective band, due to its full screening effect, which produces a sort of barrier effect.

Figure 1.
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Fluorescein angiographic photograph of the right eye. A pigmented stellate plaque envelops the right angle venules temporal to the fovea.

Figure 2.
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In the optical coherence tomogram (OCT), the colorimetric scale varies, for higher relative reflectivity, from white to red, and for lower reflectivity, from green to dark blue. The OCT horizontal line scan (white line on Figure 1) of the areas corresponding to the small plaque revealed a highly reflective white-red area (white arrow) within the inner retina. A dark intraretinal area was detected under the hyperreflective band (white asterisks). The fovea is indicated by the white arrowhead.


Based on different biomicroscopic and fluorangiographic patterns, Gass and Blodi1 have classified IJRT into 3 groups. Group 1 patients have easily visible retinal telangiectasis and capillary and retinal exudation, group 2 patients have occult telangiectasis and minimal exudation, and group 3 patients have easily visible telangiectasis and parafoveal capillary occlusion. Group 2 is often associated with superficial retinal crystalline deposits, right angle venules, and, sometimes, small plaques of pigment hyperplasia.

Retinal pigment proliferation and migration into the inner retina to form black stellate plaques was hypothesized by Gass and Blodi. However, a histological study showing the epithelial proliferation into the inner retina in some cases of retinal telangiectasis was never reported.

In this case of type 2 IJRT, the OCT line scans of the small plaque lesion showing the hyperreflective band (white-red pseudocolor) within the inner retina could be consistent with the hypothesis reported by Gass and Blodi.

The potential of OCT examination for detecting macular diseases was already pointed out in other studies.25 The images reported herein confirm that OCT is a new modality that can be used in conjunction with other diagnostic procedures to study patients affected by macular diseases. Moreover, OCT images allow proper interpretation to specific morphologic alterations, as in the present case, where histological examination was not available.

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Article Information

Reprints: Rosario Brancato, MD, Department of Ophthalmology and Visual Sciences, Scientific Institute H. San Raffaele, University of Milan, Milan, Italy.

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