Purpose

To present a rare case of Anti-Phospholipid Antibody Syndrome initially presenting as central retinal vein occlusion and ischemic optic neuropathy.

Methods

A 38 year-old hypertensive Filipino male presented with sudden blurring of vision of the right eye associated with an undiagnosed and untreated sudden blurring of vision on the left eye, that occurred when the patient was 15 years old. A comprehensive ophthalmologic and systemic examination was performed.

Results

He had poor visual acuity and pale optic discs on both eyes, and multiple flame-shaped hemorrhages and cotton wool spots following the orientation of the major vessel arcades on the right eye. Fluorescein angiography showed patchy delayed choroidal filling, focal areas of perivascular sheathing, tortuous vessels, and leakage of dye consistent with small to medium-sized vasculitis. Systemic examination revealed optic nerve atrophy and multiple chronic small vessel ischemic white matter changes on cranial MRI, and positive Lupus anticoagulant exemplified by prolonged Dilute Russell Venom Viper Time and aPTT Mixing Studies that persisted upon confirmatory testing after 12 weeks. The patient was diagnosed with APS and was started on anti-coagulant therapy.

Conclusions

Vasculitides such as APS are important risk factors for the development of ocular vaso-occlusive events, which at times can be the initial or only clinical manifestation in such patients. Vasculitic & thrombophilic work-up should be strongly considered in a young patient with unexplained ocular manifestations, in order to promptly diagnose and reduce the risk of visual loss, systemic morbidity or even mortality.