Combined Central Retinal Vein And Artery Occlusion In A Patient With Elevated Level Of Factor VIII: A Case Report

Abstract

Purpose: To report a case of unilateral central retinal vein occlusion (CRVO) with central retinal artery occlusion (CRAO) in a patient with elevated Factor VIII.

Case presentation: A 48-year-old woman presented with a complaint of decreased visual acuity in her left eye that began 6 weeks prior. The patient had diabetes. The best-corrected visual acuity (BCVA) was hand motion, IOP was 34 mmHg, and there was a neovascularization of the iris in the left eye. A complete fundus evaluation including fluorescein angiography showed non-proliferative diabetic retinopathy in the right eye and CRVO with CRAO in the left eye. Systemic evaluation revealed elevated fibrinogen and Factor VIII suggestive of the diagnosis of thrombophilia due to elevated Factor VIII. This symptom was the first sign of the underlying disorder. IOP was normalized 1 week after IOP lowering agents were applied. Intravitreal anti-vascular endothelial growth factor treatment and pan-retinal photocoagulation were performed in the left eye. Additionally, to treat thrombophilia, warfarin treatment was started and flame-shaped retinal hemorrhage with cotton wool patch near the optic disc and around the retinal vascular arcade at the posterior pole had occurred in the right eye during treatment. Then, warfarin treatment was discontinued and retinal hemorrhage was decreased. In the left eye, the BCVA did not change during treatment.

Conclusion: Elevated levels of Factor VIII as an independent risk in the development of venous thromboembolism. Combined cases usually present with severe visual loss and such patients should be thoroughly evaluated to diagnose underlying factors including Factor VIII, and initiate appropriate management at the earliest.

Keywords: central retinal artery occlusion; central retinal vein occlusion; thrombophilia; Factor VIII.

Figure 1

Ultrawide fundus photograph (A) and conventional fundus photograph (B) of the patient at 6 weeks after the symptom occurred. A blurred optic disc margin, with flame-shaped retinal hemorrhages near the optic disc and a few dot-and-blot hemorrhages at the mid-periphery of the retina. Informed consent was submitted by the patient.

Figure 2

Optical coherence tomography of the patient at 6 weeks after the symptom occurred. Increased hyperreflectivity of the inner retinal and nerve fiber layer and diffuse retinal atrophy at temporal side. Informed consent was submitted by the patient.

Figure 3

Fluorescein angiography of the patient at 6 weeks after the symptom occurred. Choroidal circulation was rarely seen; perfusion of retinal veins was faintly appreciable after 1 min, indicating impaired retinal and choroidal circulation. Informed consent was submitted by the patient.