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Presumed solitary circumscribed retinal astrocytic proliferation (PSCRAP) is a recently described retinal tumour that is a distinct entity from other white lesions of the retina such as astrocytic hamartoma and acquired astrocytoma.
With foveal involvement, the patient reported a gradual change in vision in adulthood, supporting the hypothesis that these lesions are acquired rather than congenital. This unique case reports a patient with multifocal PSCRAP lesions in 1 eye, whereas PSCRAP lesions have been previously reported only as solitary.
A 33-year-old, asymptomatic man with no personal or family history of tuberous sclerosis or neurofibromatosis was referred for evaluation of 2 pearly-white concretions in the retina of the left eye with no associated subretinal fluid, hemorrhage, or traction. His medical history was significant for asthma and type 2 diabetes mellitus. Visual acuity at presentation was 20/25 OD and 20/30 OS. Anterior segment was unremarkable. Dilated fundus examination (Fig. 1A, B) revealed a normal-appearing optic disc with 2 peripapillary pearl white retinal lesions, one temporal to the optic disc measuring approximately 1.0–1.2 mm in size and another small, white lesion superotemporal to the optic disc measuring approximately 0.5 mm in size (Fig. 1A). There was retinal pigment epithelial hyperplasia adjacent to or surrounding the lesions (Fig. 1B). Fundus autofluorescence demonstrated moderate hyperautofluorescence of both lesions (Fig. 1C). Optical coherence tomography–angiography (OCTA; Zeiss Cirrus 5000, Carl Zeiss, North York, Ont.) demonstrated the presence of an outer intraretinal mass deep to the overlying retinal vasculature (Fig. 1D). The superficial retinal vascular plexus appeared intact with no intrinsic vascularity. The vasculature, similar to the neuroretinal layers overlying the lesion, appeared compressed forward. Spectral domain optical coherence tomography (OCT) (SD-OCT; Zeiss Cirrus 5000) demonstrated a hyper-reflective, intraretinal mass with an abrupt elevation, optical shadowing, and a smooth surface with draping of the overlying retinal tissue (Fig. 1E, F). The lesions appeared to be originating from the outer retina or retinal pigment epithelium (RPE) with no underlying subretinal fluid. This was more apparent in Figure 1F as the lesion was much smaller, clearly demonstrating the preserved inner retinal layers. B-scan ultrasonography demonstrated absence of calcium in both lesions. The lesions remained stable with no changes after 6 months of follow-up.
PSCRAP was first described by Shields et al. in 2011.
Table 1 summarizes all published cases of PSCRAP and the corresponding characteristics of these lesions. PSCRAP can be distinguished from similar-appearing lesions such as astrocytic hamartomas by the appearance on OCT.
This is in contrast to OCT analysis of astrocytic hamartomas, which show an elevated or dome-shaped appearance confined to the RNFL, which is hyper-reflective, with subtle posterior shadowing of the deeper retinal structures and a normal RPE.
In addition, OCTA in the present case demonstrated a normal retinal vasculature of the surrounding superficial plexus as well as no intrinsic vascularity within the PSCRAP lesions. This is consistent with intravenous fluorescein angiography and OCTA in previous reports.
With the aid of extended depth SD-OCT more recently, it was postulated that PSCRAP lesions may originate from fibrous metaplasia of the RPE or, alternatively, may be of retinal glial origin rather than derived from retinal astrocytes as first suggested.
However, on fundus autofluorescence imaging, RPE metaplasia should appear hypoautofluorescent because there is no intrinsic or extrinsic lipofuscin production. Both lesions in our case produced slightly increased autofluorescence. Lipofuscin and calcium may appear hyperautofluorescent, but this was not present on clinical examination or B-scan ultrasonography.
To our knowledge, this is the first case of multifocal PSCRAP lesions in the same eye confirmed with multimodal imaging. All previous reports of PSCRAP described these lesions as unifocal and solitary in nature (see Table 1).