A 40-year-old man with decreasing visual acuity in his left eye over 1 year, diagnosed elsewhere as vein occlusion and treated unsuccessfully by systemic steroids was reported. Retrospective analysis of available previous imaging studies was undertaken, and a retrospective diagnosis of idiopathic macular telangiectasia (IMT) was made. Examination revealed subfoveal neovascularization and retinochoroidal anastomosis (RCA) in his left eye with macular edema and exudates surrounding it. The right eye was normal. Current fluorescein angiography (FA) confirmed the presence of choroidal neovascularization and RCA, and optical coherence tomography (OCT) demonstrated cystoid macular edema and subfoveal neovascularization.
The patient was diagnosed with unilateral idiopathic macular telangiectasia complicated by subretinal neovascularization. The presences of a choroidal neovascularization process and an RCA have not, to our knowledge, been reported in this type of IMT.
Unilateral Idiopathic Macular Telangiectasia with Choroidal Neovascularization
From the Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Daphna Mezad Koursh MD, Department of Ophthalmology, Tel-Aviv Medical Center, 6 Weitzman Street, 64239, Tel-Aviv, Israel.
Accepted: June 12, 2009
Posted Online: March 09, 2010
Exudation from telangiectatic vessels confined to the juxtafoveal region without a known cause was termed “idiopathic juxtafoveolar retinal telangiectasis” by Gass and Owakawa.1 Blurred vision and metamorphopsia are the most frequent complaints, and diagnosis is made by irregular capillary dilatation in early stages and leakage in later stages, as seen on fluorescein angiography (FA).1 Gass and Owakawa1 had classified idiopathic juxtafoveolar retinal telangiectasis according to biomicroscopic and FA features. Gass and Blodi2 later revised the classification into three groups. Although the precise pathogenesis of juxtafoveolar telangiectasis is unknown, it is believed that each group represents a different etiology. Yannuzzi et al.3 recently published a series of 36 patients with idiopathic macular telangiectasia (IMT) and proposed that the earlier classification should be modified to simplify it into three distinct types: aneurismal telangiectasia (type I), perifoveal telangiectasia (type II), and occlusive telangiectasia (type III). We present a case that shares characteristics of both type I and type II IMT (Yannuzzi’s classification), and propose extending the present classification to accommodate this variation.
A 40-year-old man was referred for evaluation due to gradually decreasing visual acuity (VA) in his left eye over the past year. Medical history was noncontributory. He had been diagnosed elsewhere as having retinal vein occlusion and was treated unsuccessfully with systemic steroids for several months. Previous imaging studies were available, and color fundus photographs of the affected eye demonstrated monocular signs of sensory retinal elevation in the fovea with pigmentary changes in the center. There were three yellowish lesions temporal to the macula with retinal edema inferior to the lowest one (Fig. 1A). (FA) of the left eye demonstrated a telangiectatic pattern of subfoveal hyperfluorescence increasing in intensity and size over time, with late accumulation in cystic pattern (Figs. 1B and C).
Figure 1. One Year Before Presentation. (A) Color Photograph Displaying a Sensory Retinal Elevation in the Fovea as Well as Pigmentary Change in the Center. There are Three Yellowish Lesions Temporal to the Macula: the Inferior One Seems to be Telangiectatic with a Few Nearby Retinal Hemorrhages and Retinal Edema Underneath. (B) This Mid-Transit Fluorescein Angiogram Demonstrates a Telangiectatic Pattern of Subfoveal Hyperfluorescence that Increased in Intensity and Size over Time. (C) Fluorescein Angiogram, Late Phase: Subfoveal Hyperfluorescence Accumulating in a Cystic Pattern.
On presentation to our clinic, his best-corrected VA was 20/20 in the right eye and 1/12-2 in the left. There was no history of unisometropia. Slit-lamp examination of the anterior segment was normal in both eyes. Funduscopic examination was normal in the right eye and revealed a grayish, oval-shaped deep subfoveal lesion with surrounding macular edema and intraretinal lipids in the left eye. A dilated vessel angulating from the superior temporal arcade and deepening perpendicularly to the center of the foveal avascular zone (FAZ) was observed, findings compatible with retinochoroidal anastomosis (RCA) (Fig. 2A). FA showed an early filling of the abnormal vessel, which angulated from the superior temporal arcade toward the FAZ, also compatible with RCA. Subfoveal leakage with late accumulation in a cystic pattern was observed (Figs. 2B and C). An optical coherence tomography (OCT) scan demonstrated thickening of the macula with hydrated cysts and a neovascular process beneath it. The appearance of RCA, severe exudation and increase in subfoveal leakage within a one-year period confirmed that there had been consideration of deterioration of the macular pathology.
Figure 2. At Presentation. (A) Color Photograph Shows a Grayish Oval-Shaped Deep Subfoveal Lesion with Macular Edema and a Circinate Type of Exudates Surrounding it. The Dilated Vessel that Can be Seen to Enter the Center of the Foveal Avascular Zone (FAZ) Resembles a Retinochoroidal Anastomose (RCA). (B) Fluorescein Angiogram, Transit Phase: a Subfoveal Leakage with an Abnormal Vessel Entering the Foveal Avascular Zone (FAZ) that is Filled Very Early in the FA and is Very Likely a Retinochoroidal Anastomose (RCA). (C) Fluorescein Angiogram, Late Phase: Subfoveal Hyperfluorescence Accumulating in a Cystic Pattern.
When first FA was performed, our patient had unilateral perifoveal aneurismal telangiectasia, with cystoid macular edema and suspected midperiphery involvement. While these features correspond with type I IMT: aneurismal telangiectasia, the concomitant presences of a choroidal neovascularization process and an RCA have not, to our knowledge, been reported in this type of IMT. These features might be found in type II IMT (macular perifoveal telangiectasia), but that type is almost always bilateral and devoid of cystic changes and aneurismal pattern. As such, we propose that our case represents unilateral retinal telangiectasis IMT-I with subfoveal neovascularization rather than an example of the typical bilateral idiopathic parafoveal telangiectasis presenting as in a unilateral variation. Our reasoning is based on laterality, the features of aneurismal dilations detected on FA as well as on the accumulation of fluorescein within cystoid spaces in the late phase prior to CNV presentation. Although not ascertained, it is also possible that the multifocal areas of fluorescence, which were temporal to the macula, represent additional areas of retinal telangiectasis.
- Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol. 1982;100:769–780.
- Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis: update of classification and follow-up study. Ophthalmology. 1993;100:1536–1546.
- Yannuzzi LA, Bardal AMC, Freund KB, Chen KJ, Eandi CM, Blodi B. Idiopathic macular telangiectasia. Arch Ophthalmol. 2006;124:450–460. doi:10.1001/archopht.124.4.450 [CrossRef]