Parafoveal telangiectasia has been described as a developmental or acquired vascular anomaly of the foveal avascular zone characterized by irregular capillary dilatation, serous exudation, and macular edema.1 The acquired form of this anomaly has been termed idiopathic juxtafoveal retinal telangiectasia and its etiology has not been established.2 Gass has divided this disorder into 3 groups based on ease of visibility of the telangiectatic vessels, presence of retinal exudation, and degree of capillary occlusion.1 We present a case that, to the best of our knowledge, is the first description of parafoveal telangiectasia seen in association with the syndrome of c alcinosis cutis, R aynaud phenomenon, e sophageal dysmotility, s clerodactyly, and t elangiectasia (CREST syndrome).
In 1986, a 61-year-old white woman was seen by her general practitioner with symptoms indicative of the Raynaud phenomenon affecting both hands. Cutaneous erythema and telangiectatic vessels were observed and the facial skin had a foreshortened appearance with resulting tightness. From these features a diagnosis of scleroderma was established. Approximately 4 years after her initial diagnosis, she developed gastric motility symptoms consisting of dyspepsia and dysphagia. In January 1997, she complained of decreasing vision in her right eye. Examination by her ophthalmologist revealed macular edema and a retinal consultation was obtained. Examination at that time disclosed a visual acuity of 20/200 OD and 20/20 OS. Slitlamp examination results and intraocular pressure were unremarkable. Dilated fundus examination revealed telangiectatic vessels in both maculae with mild retinal thickening seen in the temporal parafoveal region (Figure 1). No evidence of diabetic retinopathy was seen. Fluorescein angiography disclosed a characteristic appearance of late staining involving the temporal hemifovea of both eyes, while the right macula displayed evidence of marked enlargement of the foveal avascular zone with an irregular "moth-eaten" appearance (Figure 2). No intraretinal crystals were noted. On general examination, dermal characteristics of scleroderma were noted on her face, extremities, and chest; sausage-like thickening of her fingers was also observed.
Demonstration of parafoveal telangiectatic changes in the right (left) and left (right) eye.
Left, Midframe fluorescein angiography of right macula. Note evidence of leakage from telangiectatic vessels in parafoveal location in addition to enlargement of foveal avascular zone. Right, Midframe fluorescein angiography of left macula demonstrating staining in the temporal hemifovea.
Although relatively uncommon, CREST syndrome consists of easily identifiable symptoms. Systemic vascular changes are responsible for the telangiectatic vessels, which seem to affect several systems within the body. These vascular abnormalities have been noted in the skin and gastric mucosa,3 but to date have not been identified with retinal findings. However, it is reasonable to suggest that such widespread vascular change could present itself in the eye. Our examination of the patient's retina and the discovery of parafoveal telangiectasia, in the absence of diabetic retinopathy or other causative factors, supports this argument and is important in identifying a potential systemic association. This finding is an important consideration for patients with scleroderma, who may also be seen with unexplained loss of vision.
According to the Gass classification,1 our patient would be categorized as group 3A. Similar cases have been described in the literature with systemic associations different from ours. Grand et al2 have identified a hereditary syndrome in which retinal telangiectasia with capillary nonperfusion is associated with frontoparietal lobe pseudotumor. The histopathologic features of the central nervous system lesions included unusual vasculopathy and necrosis of white matter.2 Lim and Bressler4 have reported a case of group 3A parafoveal telangiectasia associated with marked peripheral ischemia. Our patient did not display central nervous system dysfunction or peripheral retinal ischemia.
The prescence of both juxtafoveal telangiectasia and CREST syndrome in our patient suggests a possible common pathophysiologic basis for the 2 diseases. Investigation of more patients will assist in establishing this association.
Corresponding author: Tom Chang, MD, FRCSC, Section D, 2550 Willow St, Vancouver, British Columbia, Canada V5Z 3N9.
Proctor B, Chang T, Hay D. Parafoveal Telangiectasia in Association With CREST Syndrome. Arch Ophthalmol. 1998;116(6):814-815. doi: