Sequential Central Retinal Vein and Ophthalmic Artery Occlusions in a Pediatric Case of Primary Antiphospholipid Syndrome

Abstract

Purpose To report a case of sequential central retinal vein occlusion and ophthalmic artery occlusion in a patient with primary antiphospholipid syndrome. Methods Observational case report. Color fundus photography, fluorescein angiography, and optical coherence tomography were used to document the progression of a central retinal vein occlusion and sequential development of an ophthalmic artery occlusion within a 1-week period in a patient with primary antiphospholipid syndrome. Results A 15-year-old boy presented with unilateral blurry vision due to a central retinal vein occlusion along with other systemic symptoms. Within a 1-week period, he developed an ophthalmic artery occlusion in the same eye, with resulting bare light perception vision. Extensive evaluation by the pediatrics and rheumatology services led to a diagnosis of primary antiphospholipid syndrome as the etiology for the occlusions. Conclusion This case report illustrates the rapid sequential venous and arterial ocular thrombosis in a patient with primary antiphospholipid syndrome.

Ophthalmologic manifestations in primary antiphospholipid syndrome patients: A cross-sectional analysis of a primary antiphospholipid syndrome cohort (APS-Rio) and systematic review of the literature

“…The most represented feature of posterior involvement was retinal vasculitis (60%), followed by vitritis (38%), retinal detachment (15%), posterior scleritis (7%), and central retinal artery occlusion (7%). In some papers, 11,12,18,19,62 both PAPS and secondary APS were included, so there were more ocular findings, such as cotton-wool spots, choroidal infarcts, serous macular detachment, and vitritis, which are also seen in other immunological diseases, especially SLE.…”

“…22,26,27,32 APS has also been associated with central retinal vein occlusion, 7,23,27,32–46 branch retinal vein occlusion,, 24,25,34,37,41,45,47–51 central retinal artery occlusion and branch retinal artery occlusion, 37,43,52–58 or the coexistence of both arterial and venous bed thromboses in the same patient. 32,36,59–64 Venous stasis retinopathy, 65,66 vitreous 67,68 and intraretinal hemorrhages, 67 retinal vasculitis, 69,70 cotton wool spots, 67,71 neovascularization, 72 diffuse retinal periphlebitis, 73 choroidal infarction, 74 and unexplained choroidal embolization 75 were already described as case reports.…”

“…Choroidal neovascular membrane 62,76,77 has been reported as a complication of APS due to changes in blood viscosity, leading to damage of choriocapillaris and retinal pigmented epithelium. 78…”

Central Retinal Vein Occlusion in 12-Year-Old Girl With Methylenetetrahydrofolate Reductase Mutation: A Case Report and Review of the Literature

“…To our knowledge, there are at least 24 case reports of pediatric CRVO in the literature to date with patients ranging from 20 months to 18 years. Among these, neovascularization occurred in six cases, [4][5][6][7][8][9] four being diagnosed at presentation. [5][6][7][8] The longest time interval previously reported between the initial CRVO and neovascularization was 8 months in a case of a CRVO secondary to antiphospholipid syndrome.…”

Ophthalmological manifestations in antiphospholid syndrome – case series

“…Chociaż do zajęcia tętnicy środkowej siatkówki dochodzi stosunkowo rzadko (ok. 6%), przebieg i rokowanie w przebiegu CAPS są zazwyczaj niepomyślne. W literaturze opisywano ciężkie przypadki obustronnego centralnego zamknięcia żył siatkówki oraz zamknięcie tętnicy środkowej siatkówki w przebiegu CAPS (14,15). Podobnie w opisywanym przez nas przypadku -zator gałęzi tętnicy środkowej siatkówki w OP stanowił jedną ze składowych APS o katastrofalnym przebiegu, obok zajęcia naczyń nerkowych i OUN.…”