A, Geographic yellow subretinal lesion underlies the superotemporal retinal vascular arcade. B, B-scan ultrasonography demonstrates an acoustically solid mass with shadowing and thickness of 1.7 mm. C, The lesion is hyperautofluorescent. D, Infrared reflectance imaging shows bright clumps. E, Enhanced depth imaging optical coherence tomography demonstrates an elevated scleral mass with a “rocky” surface (arrows). There is thinning of the overlying choroid and outer nuclear layer as well as absence of the external limiting membrane and inner segment–outer segment junction. Slight irregular thickening of the retinal pigment epithelium is present.
Fung AT, Arias JD, Shields CL, Shields JA. Sclerochoroidal Calcification Is Primarily a Scleral Condition Based on Enhanced Depth Imaging Optical Coherence Tomography. JAMA Ophthalmol. 2013;131(7):960-963. doi:10.1001/jamaophthalmol.2013.67
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Sclerochoroidal calcification (SC) is found in elderly white individuals with calcium pyrophosphate deposition in the sclera and/or choroid, appearing as a typical yellow subretinal mass usually along the retinal vascular arcades.1- 4 Most cases are idiopathic, but some have a systemic relationship.1,2 We review multimodal imaging findings of SC.
Clinical and imaging data of 17 SC lesions in 13 eyes of 9 patients diagnosed at the Ocular Oncology Service, Wills Eye Institute, Philadelphia, Pennsylvania, were analyzed retrospectively. Institutional review board approval was obtained, and all patients provided written informed consent.
All patients underwent ocular examination, fundus photography, B-scan ultrasonography, fundus autofluorescence imaging using the Topcon TRC-50DX Retinal Camera (Topcon America; excitation light bandwidth, 580 nm; barrier filter bandwidth, 695 nm), infrared reflectance imaging, and enhanced depth imaging optical coherence tomography (EDI-OCT) using the Heidelberg Spectralis HRA + OCT (Heidelberg Engineering Inc). Analysis of the imaging was performed by 2 independent observers (A.T.F. and J.D.A.), with consensus used to resolve disagreements.
There were 17 SC lesions in 13 eyes of 9 patients (5 male), 5 of whom had unilateral SC. All patients were white, and the mean age was 74 years (median, 73 years; range, 60-88 years). The referring diagnoses were SC (n = 3), choroidal tumor (n = 3), choroidal nevus (n = 1), choroidal melanoma (n = 1), and choroidal metastasis (n = 1). The mean best-corrected visual acuity was 20/33 (median, 20/25; range, 20/20-20/150).
All lesions were postequatorial and most were superotemporal (n = 12). The shapes of the lesions were geographic (n = 9), circular (n = 4), ill-defined (n = 3), and annular (n = 1). All lesions were orange to yellow. The mean greatest basal diameter was 3.5 mm (range, 1.0-7.0 mm), and the mean thickness on B-scan ultrasonography was 1.6 mm (range, 1.0-3.5 mm). All lesions were acoustically solid with shadowing suggestive of calcification. Orbital computed tomography performed on 1 patient demonstrated bilateral radiodense scleral lesions, confirming calcification.
On fundus autofluorescence imaging, 16 lesions were homogeneously hyperautofluorescent and 1 was homogeneously hypoautofluorescent with a hyperautofluorescent halo. Thirteen of 17 SC lesions had adequate infrared reflectance imaging and EDI-OCT. Of these, the lesions were hyperreflective (n = 11) or hyporeflective (n = 2) on infrared reflectance imaging. On EDI-OCT, all lesions originated within the sclera with thinning or absence of the overlying choroid (mean thickness, 28 μm; range, 0-119 μm) (Table 1 and Table 2). The lesions were elevated with undulating contour (n = 11) or flat (n = 2). The anterior scleral surface was irregular and “rocky” (n = 5) (Figure) or smooth and “rolling” (n = 8). The lesions were moderately reflective (n = 10) with bright anterior band (n = 3). The posterior margin of the lesion, and therefore the thickness, could not be identified in any case. By EDI-OCT, the mean diameter was 3689 μm (range, 2345-5804 μm). Overlying features included outer nuclear layer thinning (n = 2), external limiting membrane disruption (n = 1), inner segment–outer segment junction absence (n = 2), subretinal fluid (n = 1), retinal pigment epithelium alteration (n = 4), and pigment epithelial detachment (n = 1).
The clinical and ultrasonographic findings of SC lesions are consistent with previous reports.1- 3 Nearly all lesions were homogeneously hyperautofluorescent. Fundus hyperautofluorescence is typically related to increased lipofuscin in diseased retinal pigment epithelium. However, in most of our cases, the retinal pigment epithelium appeared normal clinically, with normal thickness on EDI-OCT. Therefore, we propose that thinning or absence of the overlying choroid, documented on EDI-OCT, could have allowed for unmasking of underlying scleral hyperautofluorescence. Eleven of the 13 SC lesions were hyperreflective on infrared reflectance imaging, consistent with reflectance from scleral collagen. Alternatively, intrinsic hyperautofluorescence of calcific tissue within SC may explain our fundus autofluorescence imaging findings.
The most remarkable finding on EDI-OCT was that all SC lesions originated from within the sclera, with thinning or absence of the overlying choroid. In addition, the irregular surface contour is unlike choroidal nevus or small melanoma, which display a gentle, uniform anterior slope. The rocky configuration may be unique to SC and, to our knowledge, has not previously been reported with other choroidal conditions on EDI-OCT. The inability to identify the posterior margin of each lesion may relate to the reflectivity of the calcific material, thickness, or both.
To our knowledge, there are only 2 reports of EDI-OCT of SC, and both had this rocky configuration.5,6 A clinicopathologic correlation of SC in 3 globes from 2 patients found 2 types of scleral calcification, including tophuslike conglomeration of calcium pyrophosphate crystals and diffuse noncrystalline calcification.7 The uvea was uninvolved except in one section where 2 large crystalline deposits were visualized projecting into the outer choroid. These pathologic observations correlate with our observations on the scleral localization of this condition on EDI-OCT.
The use of multimodal imaging can assist in establishing the diagnosis of SC. Six of the 9 patients (67%) were referred with a diagnosis other than SC. The differential diagnosis of SC includes choroidal osteoma, metastasis, amelanotic nevus or melanoma, lymphoma, and granuloma.1- 3 One main dissimilarity is that these lesions arise primarily from the choroid, whereas SC appears to be primarily a scleral rather than sclerochoroidal condition.
Corresponding Author: Dr C. L. Shields, Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, 840 Walnut St, Ste 1440, Philadelphia, PA 19107 (email@example.com).
Published Online: May 30, 2013. doi:10.1001/jamaophthalmol.2013.67
Author Contributions: Dr C. L. Shields had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Conflict of Interest Disclosures: None reported.
Funding/Support: This work was supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (Drs C. L. Shields and J. A. Shields).