SDWhite Dot Syndrome

A45-year-old woman with scotoma in both eyes that had been enlarging over the course of 1 week was referred to me by her treating ophthalmologist. The patient denied the presence of other symptoms including headaches, tinnitus, transient visual obscurations, and nausea or vomiting. She had a history of hypertension. She was screened by her referring physician for syphilis, Lyme disease, and Bartonellosis with negative results for all. Her ophthalmologist had diagnosed the patient with papilledema.

The patient's visual acuity was 20/20 in the right eye and 20/25 in the left eye and her color vision was intact. The only significant finding was that she had a bit of anterior vitreous cell. The patient's fundus images showed significant disc edema in each eye–more on the right than the left, as well as faint whitish spots deep in the retina in a peripapillary distribution.

The patient's fluorescein angiogram (FA) showed early blocked fluorescence corresponding to the areas of these white dots with some staining in the later phases. FA also showed some disk leakage in both eyes.

One week later, the patient complained of further enlargement of her scotoma and blurring of her vision. Her visual acuity was relatively unchanged. Her visual acuity was relatively unchanged. We did notice a few fine keratic precipitates in her cornea and she had some cell in her anterior chamber. Static perimetry revealed enlargement of blind spots in both eyes.

Fundus exam of the right eye at this visit (now 1 week after first presentation) now demonstrated peripheral white dots (Figure 1) as well as small areas of apparent retinitis—there was whitish change in the retina as well as some hemorrhage. The left eye demonstrated similar peripheral findings. The FA now demonstrated granular areas of hyperfluoresence in the peripapillary regions (especially nasally) in each eye (OD, Figure 2). Indocyanine green (ICG) angiography showed hypofluoresence corresponding to these peripapillary lesions (Figure 3).

Spectral-domain optical coherence tomography (SDWhite OCT) scans from the SPECTRALIS system (Heidelberg Engineering, Heidelberg, Germany) for this patient are shown in Figure 4. The OCT revealed areas of elevation underneath the retinal pigment epithelial (RPE) layer that corresponded to the peripapillary white-dot lesions.

The patient returned 2 weeks later and reported that her vision was improving with the blurriness subsiding and her blind spots becoming smaller. Her fundus images (Figure 5) and OCTs (Figures 6) showed that sub-RPE deposits had nearly disappeared and the disc edema had resolved.

CONCLUSION
We are still unsure as to the precise diagnosis for this patient, although we know that it falls within the spectrum of white dot syndrome. Two specific diagnoses that one might consider are multiple evanescent white dot syndrome (MEWDS) or acute posterior multifocal pigment placoid epitheliopathy (AMPPE). MEWDS, however, is usually unilateral, and there usually is no accumulation of inflammatory material below the RPE, just disruption of the photoreceptor layer. In addition, retinal whitening has not been previously described in patients with MEWDS. The case is also atypical for AMPPE as there was never a typical placoid appearing lesion. However, we did uncover a previous atypical case of AMPPE reported in 1972 by Kirkham et al1 that described the connection with papillitis, vasculitis, and retinal whitening. Overall, when considering the spectrum of white dot syndromes, this patient seems to fit best somewhere between AMPPE and multifocal choroidits. Perhaps, as we evaluate this patient over time, a more specific diagnosis will be possible. Regardless, multimodal imaging with the SPECTRALIS— in particular our ability to correlate the angiographic findings with the sub-RPE lesions on OCT—was very helpful in studying the disease course in this patient and distinguishing between diagnoses.

Srinivas R. Sadda, MD, is an Associate Professor of Ophthalmology at the Doheny Eye Institute and University of Southern California in Los Angeles. He shares in royalties from intellectual property licensed to Topcon Medical Systems from Doheny Eye Institute and has served as a consultant to Heidelberg Engineering. The Doheny Image Reading Center has received research support from Carl Zeiss Meditec. He can be contacted at +1 323 442 6522; or via e-mail at: SSadda@doheny.org.