Case of the Month

Edited by Robert N. Johnson, MD

Figure 1: Wide-angle color fundus photo of the right eye. Note the several discrete yellowish lesions superotemporally.

Case History

A 74-year-old white male presented for routine evaluation following macular hole surgery with successful closure in the left eye 13 years prior.* He had no ocular complaints. Past ocular history included bilateral pseudophakia, myopic degeneration, and glaucoma. Past medical history was notable for colon cancer treated 15 years ago, hyperlipidemia, and allergic rhinitis, treated with an oral statin and steroid nasal spray, respectively. He denied any history of ocular trauma, renal disease, or endocrine problems.

On examination, visual acuity was 20/25 in the right eye and 20/125 in the left eye. The anterior segment examination was normal in both eyes.

Posterior segment examination of the right eye revealed multiple yellow subretinal lesions grouped superotemporally in the mid-peripheral fundus (Figure 1). Dilated examination of the left fundus was unremarkable with no evidence of similar-appearing lesions.

Wide-field fundus autofluorescence of the right eye showed hyper-autofluorescence corresponding to the subretinal lesions seen on clinical exam (Figure 2). Enhanced depth imaging spectral domain optical coherence tomography (EDI SD-OCT) through the lesions revealed scleral masses associated with overlying choroidal thinning and a mixed jutted and smooth topography (Figure 3). B-scan ultrasonography revealed elevated, solid, hyper-reflective lesions with posterior shadowing (Figure 4).

Figure 2: Wide-field autofluorescence image of the right eye. Note the hyper-autofluorescence of the superotemporal lesions.

Figure 4: B-scan ultrasound through the area of the lesions. Note the orbital shadowing and dense hyperreflectivity of the lesions.

Differential Diagnosis

Choroidal osteoma, amelanotic nevus or melanoma, ocular adnexal/choroidal lymphoma, granuloma, choroidal metastases, sclerochoroidal calcifications.

Diagnosis and Additional History

Given the typical appearance and features of the lesions on multi-modal imaging, the diagnosis of sclerochoroidal calcifications was made. Upon review of previous fundus photographs, it was noted that the sclerochoroidal calcifications were first detected 10 years prior and had grown in both size and number over the ensuing decade (Figure 5A-C). Laboratory work up to search for abnormal calcium-phosphorous metabolism was negative.

Discussion

Sclerochoroidal calcifications represent depositions of calcium pyrophosphate in the scleral and choroid. They are typically asymptomatic and incidentally discovered as discrete yellow placoid subretinal lesions, classically located in the superotemporal mid-periphery. They may be bilateral or unilateral, and tend to affect elderly white individuals.¹

Sclerochoroidal calcifications are typically idiopathic but occasionally are associated with abnormalities of calcium-phosphorous metabolism, including hyperparathyroidism, pseudohypoparathyroidism, vitamin D intoxication, sarcoidosis, hypophosphatemia, and chronic renal failure; or renal tubular hypokalemic metabolic alkalosis syndromes, such as Bartter and Gitelman syndromes.² These treatable disorders can be ruled out with systemic testing.

The correct diagnosis of sclerochoroidal calcifications, which are often misdiagnosed as a malignant tumor, relies on knowledge of their typical appearance and classic location and is aided by multi-modal imaging. EDI SD-OCT may show scleral masses and an anterior scleral surface topography that has been termed “rocky and rolling”—that is, irregular and jutted and/or smooth and undulating.³ B-scan ultrasonography shows elevated, solid, hyper-reflective lesions with posterior shadowing consistent with calcifications. Fundus autofluorescence shows homogenous hyperautofluorescence in the areas of the lesions, which may reflect intrinsic hyperautofluorescence of calcific tissue or unmasking of underlying scleral hyperautofluorescence in the setting of thinned or absent overlying choroid.³

 An unusual feature of the case reported here is the growth in size and number of the calcifications over a decade of follow up, with the first changes noted after the first year. In the Shields et al⁴ series of 179 eyes with sclerochoroidal calcifications, none were noted to enlarge or develop new lesions over 45 months follow up. Boutboul et al,⁵ however, reported a case of sclerochoroidal calcification growth over a 24-year follow up period in a patient with familial chondrocalcinosis. As noted above, work up for abnormal calcium-phosphorous metabolism in the patient reported here was negative.

Sclerochoroidal calcifications rarely may be associated with the development of choroidal neovascularization, and therefore patients should be routinely followed.⁶

*This Case of the Month was adapted from the article entitled “Enlargement of Sclerochoroidal Calcifications: Multimodal Imaging Update” by Geraldine R. Slean, Ananda Kalevar, Judy Chen, and Robert Johnson, published in Retinal Cases & Brief Reports on October 9, 2017.

References

1. Shields JA, Shields CL. CME review: sclerochoroidal calcification: the 2001 Harold Gifford Lecture. Retina 2002;22:251–261.
   Honavar SG, Shields CL, Demirci H, Shields JA. Sclerochoroidal calcification: clinical manifestations and systemic associations. Arch Ophthalmol 2001;119:833–840.
2. 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;131:960–963.
3. Shields CL, Hasanreisoglu M, Saktanasate J, et al. Sclerochoroidal calcification: clinical features, outcomes, and relationship with hypercalcemia and parathyroid adenoma in 179 eyes. Retina 2015;35:547–554.
4. Boutboul S, Bourcier T, Heligon J-P, et al. Familial pseudotumoral sclerochoroidal calcification associated with chondrocalcinosis. Br J Ophthalmol 2004;88:1094–1095.
5. Bessette AP, Singh AD. Multimodal imaging of choroidal neovascularization associated with sclerochoroidal calcification. Ocul Oncol Pathol 2016;2:234–238.