A 44-year-old woman affected by CREST syndrome also known as the limited cutaneous form of systemic sclerosis, was referred with decreased vision in both eyes. The patient has suffered from gastric motility symptoms consisting of dyspepsia and dysphagia. The general practitioner’s physical examination revealed Raynaud phenomenon affecting hands, cutaneous erythema and telangiectatic vessels on the facial skin and a foreshortened appearance with resulting skin tightness. She had been using colchicine for CREST syndrome for six-years and had no other ocular and systemic diseases. Family history of the patient was also unremarkable.
On ocular examination, the best-corrected visual acuities were 20/50 in the right eye and 20/25 in the left eye. The intraocular pressures were within normal limits and anterior segment examinations were also normal. On fundus examination, abnormal foveal reflex and retinal telangiectatic vessels in both macula without any exudate were seen. Any other evidence of diabetic retinopathy or other retinal vascular diseases was seen.
Fundus fluorescein angiography clearly demonstrated the characteristic appearance of retinal telangiectatic vessels without any leakage in both eyes, while the right macula disclosed evidence of marked enlargement of the foveal avascular zone with an irregular “moth-eaten” appearance.
On spectral-domain optical coherence tomography examination, focal irregular hyperreflectivity at the outer nuclear layer and the outer side of the outer plexiform layer in both eyes in addition to disorganization of inner retinal layers at the the temporal parafovea of the right eye were detected.
Optical coherence tomography angiography analysis revealed the perifoveal telangiectatic vessels and irregularities and enlargement of the foveal avascular zone in the slabs of superficial and deep capillary plexus.
Idiopathic macular telangiectasia type 3 is a rare, acquired, eye disease characterized by progressive visual loss, due to bilateral juxtafoveolar capillary occlusions, capillary telangiectasia, and minimal exudation. It is associated with systemic or cerebral vascular occlusive disease. It has been poorly understood, and most of the reported cases were associated with a systemic vascular or cerebral disease. In Gass series, type 3 was described in 7 of 140 (5%) patients. However, Yannuzi simplified this classification in 2006, and called type 1 as aneurysmal telangiectasia and type 2 as perifoveal telangiectasia. Features of the third type were not observed, so it has been omitted due to its rarity.
Credit: Kemal Tekin, M.D., from Ulucanlar Eye Training and Research Hospital
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