Case of the Month I January 2019

Case of the Month
January 22, 2019

The Case

The patient was a 68 year old man without visual complaints who was seen for a routine eye examination and was found to have superotemporal yellowish lesions in both eyes. The past medical history was unremarkable. The visual was 20/20 OD and 20/25 OS. Examination revealed early cataracts in each eye and deep yellowish lesions underneath the superotemporal arcade of each macula. OCT showed an area of elevation without disruption of the retinal layers in each eye. B-scan ultrasound revealed a lesion with underlying acoustic shadowing. What is the most likely diagnosis? What treatment, if any, would you recommend?

This patient had sclerochoroidal calcification, in which there is calcium deposition in the sclera that can extend to the choroid. Due to attenuation of ultrasound energy by the calcium, there was shadowing behind the lesion on B-scan ultrasonography. Of interest, the OCT showed disruption of the normal choroidal vasculature, which can be appreciated when looking at the choroid below the lesion compared to the adjacent choroid.

Sclerochoroidal calcification is typically diagnosed in middle or late age, and almost all reported cases are of white patients. It is usually bilateral and presents as one or more deep yellowish masses. Nearly all cases are asymptomatic, though an associated choroidal neovascular membrane has been reported. Unless seen in younger patients, sclerochoroidal calcification is typically idiopathic. It can be seen in hypercalcemic states, such hyperparathyroidism and pseudohyperparathyroidism, and in Gitelman syndrome and Bartter syndrome, which are autosomal recessive disorders that result in hypokalemic alkylosis and calcium salt deposition in various tissues.

Sclerochoroidal calcification can resemble osteomas, which are generally unilateral and they tend to be found in younger adults and children. Osteomas present as well-defined, yellow-orange lesions, while the lesions of sclerochoroidal calcification often manifest as a collection of small lesions or one or more small lesions near a larger collection of calcium, as seen in our patient.

Useful reference: Shields JA, Shields CL. Review: Sclerochoroidal calcification. Retina 2002;22:251-261.

Case Photos

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This patient had sclerochoroidal calcification, in which there is calcium deposition in the sclera that can extend to the choroid. Due to attenuation of ultrasound energy by the calcium, there was shadowing behind the lesion on B-scan ultrasonography. Of interest, the OCT showed disruption of the normal choroidal vasculature, which can be appreciated when looking at the choroid below the lesion compared to the adjacent choroid.

Sclerochoroidal calcification is typically diagnosed in middle or late age, and almost all reported cases are of white patients. It is usually bilateral and presents as one or more deep yellowish masses. Nearly all cases are asymptomatic, though an associated choroidal neovascular membrane has been reported. Unless seen in younger patients, sclerochoroidal calcification is typically idiopathic. It can be seen in hypercalcemic states, such hyperparathyroidism and pseudohyperparathyroidism, and in Gitelman syndrome and Bartter syndrome, which are autosomal recessive disorders that result in hypokalemic alkylosis and calcium salt deposition in various tissues.

Sclerochoroidal calcification can resemble osteomas, which are generally unilateral and they tend to be found in younger adults and children. Osteomas present as well-defined, yellow-orange lesions, while the lesions of sclerochoroidal calcification often manifest as a collection of small lesions or one or more small lesions near a larger collection of calcium, as seen in our patient.

Useful reference: Shields JA, Shields CL. Review: Sclerochoroidal calcification. Retina 2002;22:251-261.

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