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HEMOGLOBIN C TRAIT PRESENTING WITH BILATERAL CENTRAL RETINAL VEIN OCCLUSION
Adam J Neuhouser1, Riley N Sanders, Manojna Konda
1Department of Ophthalmology, Jones Eye Institute, Little Rock, Arkansas.
Insights
This case study details bilateral sequential central retinal vein occlusion (CRVO) in a patient with hemoglobin C trait. It suggests hemoglobin C may contribute to retinal vascular occlusive disease.
Area of Science:
- Ophthalmology
- Hematology
- Vascular Biology
Background:
- Central retinal vein occlusion (CRVO) is a significant cause of vision loss.
- Sickle cell retinopathy is a known complication of certain hemoglobinopathies.
- Hemoglobin C trait is less commonly associated with severe vascular complications.
Purpose:
To report a case of bilateral sequential, central retinal vein occlusion (CRVO) in a man with newly diagnosed hemoglobin C trait.
Methods:
A 67-year-old man presented with a one-month history of declining visual acuity. He was diagnosed with left CRVO. Bilateral temporal retinal ischemia and arteriovenous anastomoses on fluorescein angiography suggestive of sickle cell retinopathy prompted a systemic work-up. Hemoglobin electrophoresis revealed an underlying hemoglobin C trait. Six-months after his initial presentation, the patient developed symptomatic right CRVO.
Results:
The initial left CRVO was complicated by optic disc swelling and macular edema. Intravitreal antiangiogenic therapy was initiated and the macular edema resolved. The left eye subsequently developed an epiretinal membrane which was surgically removed. Macular edema in the right eye also resolved after intravitreal antiangiogenic therapy.
Conclusion:
This is the first reported case of bilateral CRVO in a case of hemoglobin C trait. It is possible that erythrocyte inflexibility, caused by hemoglobin C-induced dehydration and crystallization, acted concomitantly with hypertension to produce occlusive microangiopathy. This case highlights the need for further investigation in patients presenting with central retinal vein occlusion, especially when bilateral, or when retinal angiography reveals bilateral pathology.
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