Spontaneous Branch Retinal Artery and Central Retinal Vein Occlusion with Sickle Cell Trait: A Case Report
Objective:
To report a case of branch retinal artery occlusion (BRAO) and central retinal vein occlusion (CRVO) in a young adult with sickle cell trait, and to emphasize its potential implications
Background:
Sickle cell trait has been considered a generally benign condition and instances of spontaneous BRAO/CRVO unrelated to trauma or other comorbidities in these patients are limited.
Results:
18-year-old female with no significant medical history presented to the hospital with acute onset painless monocular vision loss in the left eye. Visual field confrontation testing revealed superior altitudinal visual filed defect. Fundoscopic exam showed segmental retinal whitening suggestive of BRAO and tortuosity of vessels raising concerning for CRVO. Patient denied OCP, cigarette use, DM or family history of thrombosis. Work up including CTA H&N, DSA and MRI brain did not show evidence of stenosis, vascular malformations or acute infarct respectively. TEE was negative for intracardiac shunt or thrombus. Basic labs including ESR, CRP were within normal limits. CSF studies obtained showed no pleocytosis, normal protein (25) and glucose (57). Hypercoagulable labs obtained, including lupus anticoagulant antibodies and ANA were negative. Patient was negative for JAK2, factor V Leiden and prothrombin mutations. She had normal protein C and S activity. A sickle cell preparation and hemoglobin electrophoresis confirmed the presence of sickle cell trait: Hemoglobin A 56%, Hemoglobin S 33%, Hemoglobin F <1% . The presence of hemoglobin S, which can increase blood viscosity and promote turbulent blood flow in small blood vessels, may predispose individuals to thrombotic events, particularly in the microcirculation of the retina.
Conclusions:
This case underscores the need to monitor patients with sickle cell trait and to suggest its potential to manifest as ischemic events.
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