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Case Reports
, 113 (1), 48-57

Idiopathic Juxtafoveal Retinal Telangiectasis: New Findings by Ultrahigh-Resolution Optical Coherence Tomography

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Case Reports

Idiopathic Juxtafoveal Retinal Telangiectasis: New Findings by Ultrahigh-Resolution Optical Coherence Tomography

Lelia A Paunescu et al. Ophthalmology.

Abstract

Objective: To investigate the capabilities of ultrahigh-resolution optical coherence tomography (UHR OCT); to compare with the commercially available OCT standard-resolution system, StratusOCT, for imaging of idiopathic juxtafoveal retinal telangiectasis (IJT); and to demonstrate that UHR OCT provides additional information on disease morphology, pathogenesis, and management.

Design: Retrospective, observational, interventional case series.

Participants: Nineteen eyes of 10 patients diagnosed with IJT in at least one eye.

Method: All patients were imaged with UHR OCT and StratusOCT at the same visit. A subset of patients was also imaged before and after treatment of IJT.

Main outcome measures: Ultrahigh- and standard-resolution cross-sectional tomograms of IJT pathology.

Results: Using both standard- and ultrahigh-resolution OCT, we identified the following features of IJT: (1) a lack of correlation between retinal thickening on OCT and leakage on fluorescein angiography, (2) loss and disruption of the photoreceptor layer, (3) cystlike structures in the foveola and within internal retinal layers such as the inner nuclear or ganglion cell layers, (4) a unique internal limiting membrane draping across the foveola related to an underlying loss of tissue, (5) intraretinal neovascularization near the fovea, and (6) central intraretinal deposits and plaques. In 63% of cases, the presence of abnormal vessels and a discontinuity of the photoreceptor layer correlated with visual acuity.

Conclusions: Ultrahigh-resolution OCT improves visualization of the retinal pathology associated with IJT and allows identification of new features associated with it. Some of these features, such as discontinuity of the photoreceptor layer, are revealed only by UHR OCT.

Figures

Figure 1
Figure 1
A, A commercially available optical coherence tomography (OCT) standard-resolution system (StratusOCT) image of a normal human macula. B, Ultrahigh-resolution (UHR) OCT of the normal macula at the same location. The images demonstrate the ability to visualize intraretinal layers that can be correlated with retinal anatomy. Most of the major intraretinal layers can be visualized in the StratusOCT image, but the ganglion cell layer (GCL) and external limiting membrane (ELM) are much better visualized in the UHR OCT image. INL = inner nuclear layer; IPL = inner plexiform layer; IS = inner segment; NFL = nerve fiber layer; ONL = outer nuclear layer; OPL = outer plexiform layer; OS = outer segment; RPE = retinal pigment epithelium. Red, highly backscattering layers; blue, low-backscattering layers.
Figure 2
Figure 2
Patient 1. A, Color fundus photography of the left eye of a unilateral patient with idiopathic juxtafoveal retinal telangiectasis (IJT) classified as group 1B (Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 1982;100:769–80) (Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology 1993;100:1536–46). The color photography depicts some macular edema. B, C, Ultrahigh-resolution optical coherence tomography image of a patient with unilateral 1B (IJT). Scans taken at 180° (B) and 240° (C). Both images demonstrate a foveal cystoid, small intraretinal cystoids, and fluid accumulation under the sensory retina. A Müller cell is shown in B, and intraretinal blood vessels are shown in C. The photoreceptor layer is shown intact in both images. ELM = external limiting membrane; IS/OS = junction between the photoreceptor inner segment and outer segment.
Figure 3
Figure 3
Patient 2. A, Red-free photography of the right eye of a bilateral patient with idiopathic juxtafoveal retinal telangiectasis (IJT) classified as group 2A (Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 1982;100:769–80) (Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology 1993;100:1536–46). Photography is enhanced in the foveal region to show cystoid structure. B, Ultrahigh-resolution optical coherence tomography (OCT) image of the right eye of a patient with bilateral 2A IJT. The image demonstrates a foveal cystoid. All the other intraretinal tissue layers, including the photoreceptor layer, are shown intact. C, Red-free photography of the left eye of a bilateral patient with IJT classified by Gass (see above) as group 2A. Photography is enhanced in the foveal region to show cystoid structure. D, Ultrahigh-resolution OCT image of the left eye of a patient with bilateral 2A IJT. The image demonstrates a foveal cystoid and small intraretinal cystoids in the temporal side of the fovea. The photoreceptor layer is shown with a disruption in the foveola region; however, the peripheral photoreceptor layer is intact. The external limiting membrane (ELM) is shown intact throughout the macula. In addition, the internal limiting membrane (ILM) is shown across the macula, forming what we call the ILM drape. The photoreceptor segment disruption correlates with the larger visual acuity loss in the left eye.
Figure 4
Figure 4
Patient 3. A, Color photography of the right eye of a bilateral patient with idiopathic juxtafoveal retinal telangiectasis (IJT) classified as group 2A (Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 1982;100:769–80) (Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology 1993;100:1536–46). A normal retina is depicted. B, Commercially available optical coherence tomography (OCT) standard-resolution system (StratusOCT) image of a bilateral patient with group 2A IJT. The image shows a normal-looking retinal scan and perhaps a thinner fovea. Quantitative measures indicate a thinner macula, including the foveola. C, Ultrahigh-resolution (UHR) OCT image of a bilateral patient with group 2A IJT. The image shows a normal-looking retina in the peripheral macula with some thinning of the photoreceptor layer and a thinner fovea. In the foveola, a small disruption of the photoreceptor layer and a cystlike structure can be visualized. A Müller cell connecting the inner and outer retinal layers is also visible. Both StratusOCT and UHR images are taken at a 90° scan orientation.
Figure 5
Figure 5
Patient 4. A, Fluorescein angiography of the left eye of a patient with bilateral group 2A idiopathic juxtafoveal retinal telangiectasis (IJT). The red-free photograph shows a yellow central spot in the foveola. Angiography shows some late leakage. B, Ultrahigh-resolution optical coherence tomography image of the left eye of a patient with bilateral group 2A IJT, taken at a 150° scan orientation. The image demonstrates a yellow deposit and small cystoids in the ganglion cell layer and inner nuclear layer with elevation of the inner retina. Loss of the photoreceptor segment junction (IS/OS) signal is shown centrally where the photoreceptor junction is intact peripherally. ELM = external limiting membrane.
Figure 6
Figure 6
Patient 5. A, Late-phase fluorescein angiography of the right eye of a patient with bilateral group 2A idiopathic juxtafoveal retinal telangiectasis (IJT). The angiography shows leakage near the foveola. B, Ultrahigh-resolution optical coherence tomography (OCT) image of the right eye of a patient with bilateral group 2A IJT, taken at a 30° scan orientation. The image demonstrates a large blood vessel near the fovea, a small cystoid in the ganglion cell layer (GCL), an internal limiting membrane (ILM) drape across the fovea, and complete loss of the photoreceptor layer centrally, with a thinning of the retina but intact photoreceptor layer peripherally. C, Late-phase fluorescein angiography of the left eye of a patient with bilateral group 2A IJT. The angiography shows a pattern similar to that of a macular hole, with some leakage near the foveola. D, E, Ultrahigh-resolution OCT image of the left eye of a patient with bilateral group 2A IJT, taken at 150° (D) and 180° (E) scan orientations. Both images demonstrate large blood vessels near the fovea, small cystoids in the GCL and inner nuclear layer, an ILM drape across the fovea creating a lamellar hole, and complete loss of the photoreceptor layer centrally but an intact photoreceptor layer peripherally.
Figure 7
Figure 7
Patient 6. A, Late-phase fluorescein angiography (FA) of the right eye of a patient with bilateral group 2A idiopathic juxtafoveal retinal telangiectasis (IJT). The FA shows changes similar to that of choroidal neovascularization (CNV). B, Ultrahigh-resolution optical coherence tomography (OCT) image of the right eye of a patient with bilateral group 2A IJT, taken at a 90° scan orientation. The image demonstrates changes near the fovea that could be correlated with retinal pigment epithelium (RPE) plaque and CNV. Small cystoids are present in the inner nuclear layer, and complete loss of the photoreceptor layer is detected centrally. The retina is thickened, which is associated with CNV. C, Late-phase FA of the left eye of a patient with bilateral group 2A IJT. The FA shows hyperfluorescence associated with leakage. D, Ultrahigh-resolution OCT image of the left eye of a patient with bilateral group 2A IJT, taken at a 90° scan orientation. The image demonstrates fluid accumulation in the fovea underneath the junction between the photoreceptor inner segment and outer segment, but with an intact external limiting membrane and RPE. A small internal limiting membrane (ILM) drape is shown in the foveal region.

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