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. 2014:2014:782923.
doi: 10.1155/2014/782923. Epub 2014 Aug 21.

First Reported Case of Proliferative Retinopathy in Hemoglobin SE Disease

Paul Baciu  1 , Christopher Yang  1 , Aldo Fantin  1 , Deborah Darnley-Fisch  1 , Uday Desai  1
Affiliations

First Reported Case of Proliferative Retinopathy in Hemoglobin SE Disease

Paul Baciu et al. Case Rep Ophthalmol Med. 2014.

Abstract

We report the first case of proliferative sickle cell retinopathy in a patient with hemoglobin SE (Hb SE) disease. Only a few dozen cases of Hb SE disease have been reported previously, and none had evidence of proliferative retinopathy. A 56-year-old African American man presented to our clinic for routine examination and was found to have sea-fan peripheral neovascularization bilaterally without maculopathy. Hemoglobin analysis revealed Hb SE heterozygosity. Sector laser photocoagulation to areas of nonperfusion in both eyes resulted in regression of the peripheral neovascularization over a period of 6 months. Although Hb SE disease is rare, the incidence of Hb SE disease is postulated to rise in the future. Awareness of its potential ocular complications is needed to appropriately refer these patients for screening.

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Figures

Figure 1
Figure 1
Fundus photography of both eyes. Right eye (a) showing sclerotic occluded vessels (black arrows) and early sea-fan neovascularization (white arrow) in the inferotemporal periphery. Left eye (b) shows sea-fan neovascularization (black arrow) with fibrovascular proliferation (white arrows).
Figure 2
Figure 2
Fluorescein angiography of both eyes showing peripheral neovascularization with leakage (white arrows) and capillary nonperfusion.
See this image and copyright information in PMC

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