One of the complications of sickle cell disease is retinopathy caused by the deprivation of blood and oxygen due to the inability of sickling red blood cells to access the retinal vasculature.
Our patient, a 19-year-old black woman who had been diagnosed with sickle cell disease at 3 years of age presented with 20/20 vision in both eyes, peripheral neovascularization, and a history of pneumonias in her right eye. The color fundus photographs (Figure 1) show noticeable venous tortuosity, retinal neovascularization, and arterial whitening.
OCT IMAGING
On a SPECTRALIS (Heidelberg Engineering,
Heidelberg, Germany) spectral-domain optical coherence
tomography (SD-OCT) scan taken superior to the
fovea, we saw a slight depression sign (Figure 2). Figure
3 shows the central macula. SD-OCT scans of the inferior
portion of the retina show the inner segment/outer
segment junction and the retinal pigment epithelium
are intact. The inner retina, however, looks as if it has
been “crimped” down (Figure 4). The thinning goes
throughout the inferior retina and then superiorly
through the macular area again with more significant
“crimping.”
SELECTIVE THINNING OF THE INNER RETINA
My colleagues and I are working on a study for which
we have imaged approximately 60 patients with sickle
cell retinopathy using the SPECTRALIS SD-OCT. Some of
the patients have no clinically visible arterial occlusions
or other macular abnormalities, yet they have OCT
findings consistent with arterial infarction. Almost all
have significant temporal (or nasal) macular thinning.
The impetus for this study was that the presumed
notion that infarcts, which occur when the red blood
cells sickle in the eye, often go unnoticed because the
inner retina is most affected, sparing the photoreceptors.
The SPECTRALIS is particularly useful in detecting
these infarcts. The ischemia that occurs, however, causes
irreparable damage to the inner retina, resulting in
selective thinning of the inner retina. We have found
this to be true compared with our controls. Similar
findings have been reported in the literature.1
Jennifer I. Lim, MD, is Professor of Ophthalmology, Director of the Retina Service and the Charles I. Young Chair of Ocular Research at the University of Illinois, Illinois Eye and Ear Infirmary in Chicago. She reports that she has received honoraria from Heidelberg Engineering. Dr. Lim can be reached at +1 312 413 0704; or via e-mail at jennylim@uic.edu.
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