Bilateral Central Retinal Vein Occlusion in a Patient with Scleroderma

Abstract

A 53-year-old man with scleroderma, pulmonary fibrosis, cardiac decompensation and secondary polycythaemia, but no arterial hypertension, developed central retinal vein occlusion (CRVO) in the left eye. 1.5 years later, during the treatment with systemic steroids and anticoagulants, he developed CRVO in the right eye, and a further half year later, secondary glaucoma in the left eye and loss of the visual acuity to counting fingers at 2.5 m in the right eye and at 0.5 m in the left. Retinal vascular changes, pulmonary and cardiac insufficiency and secondary polycythhaemia, symptoms of scleroderma, most probably contributed to the development of bilateral CRVO.

Prevalence of ocular manifestations in systemic sclerosis patients

“…It should be noted that in the course of systemic sclerosis numerous complications within internal organs occur, including myocardial fibrosis, pulmonary fibrosis and secondary polycythaemia, which may promote development of thrombi in retinal veins [10, 16, 30]. On the other hand, fibrosis of blood vessel may result in the occlusion of retinal arterial vessels [31].…”

Retinal findings in systemic sclerosis: a comparison with nailfold capillaroscopic patterns

“…Only a few published case reports have referred to occlusion of retinal arteries or veins in SSc. [2][3][4][5] Retinal vascular changes may reflect cerebral and sometimes general vascular changes. 6 On the other hand, architectural abnormalities of the microvasculature, which are also characteristic of SSc, are easily visualised by wide field microscopy at the nailfold capillary bed.…”

Central retinal vein occlusion: A patient with systemic sclerosis

“…Besides SSc, the presented patient had no related systemic diseases that could cause CRVO and this manifestation of scleroderma was reported only few times in the literature 9,10,14,17 . The cases reported by Saari et al 9 and Gomes et al 10 suffered from other systemic diseases such as secondary polycythemia, cardiovascular insufficiency and pulmonary fibrosis which could contribute to the pathogenesis of CRVO and therefore, scleroderma could not be considered as the direct cause of CRVO. On the other hand, Malik and Al Habash 17 excluded diabetes, hypertension, blood dyscrasias, clotting disorders and sickle cell disease as the causes of CRVO.…”

“…Although patients with SSc are known to have variety of vascular abnormalities, retinal vascular changes are not well understood. Only a few published case reports have referred to occlusion of retinal arteries or veins in SSc 2,[8][9][10] . The aim of this paper was to review the variety of ocular manifestations in patients with systemic sclerosis.…”