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.

Fig. 1

Color montage photograph of the right eye on presentation showing vascular tortuosity, vessel sheathing, white-centered intra-retinal hemorrhages, blurring of the optic disk margins, and peripapillary edema. These findings appeared consistent with a CRVO.

Fig. 2

Fluorescein angiogram (FA) at presentation of the right eye. A. Early and (B) late frames. C. A montage FA. There is delayed filling of the venules, venous congestion, and phlebitis consistent with a nonischemic CRVO.

Fig. 3

Time-domain optical coherence tomography at presentation of the right eye. A. A temporal-to-nasal scan through the fovea shows no central cystoid macular edema. B. Retinal thickness map shows peripapillary retinal thickening.

Fig. 4

A. Color photograph of the right eye 1 week after presentation shows retinal whitening, diffuse intraretinal hemorrhages, macular edema, and severe vascular tortuosity, congestion, and sheathing. B–D. Fluorescein angiogram reveals markedly delayed and incomplete retinal and choroidal vascular filling with severe ischemia, consistent with worsening of the CRVO and the development of an ophthalmic artery occlusion.

Fig. 5

Spectral domain optical coherence tomography through the fovea of the right eye at 1 week after presentation shows diffuse retinal edema and disturbance of all retinal layers.