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Diagnosis and management of idiopathic sclerochoroidal calcification Christian Larson, OD Minneapolis VAMC Optometry Resident Abstract: Sclerochoroidal calcification is a rare, benign disorder that presents in elderly patients and is commonly misdiagnosed as a choroidal malignancy. Sclerochoroidal calcification may be indicative of an underlying systemic condition that produces dystrophic electrolyte levels. I. Case History a. Patient demographics: 81 year old white male b. Chief complaint: Physician directed diabetic eye exam c. Ocular history: Pseudophakia OU, no history of diabetic retinopathy d. Medical history: Type II diabetes mellitus, hypertension, hyperlipidemia, sleep apnea, osteoarthritis, chronic back pain, anxiety, hearing loss, and chondrocalcinosis e. Current medications: Olanzapine, citalopram, mirtazapine, hydrochlorothiazide diltiazem, losartan, simvastatin, diclofenac, gentamicin ointment, psyllium, colchicine II. Pertinent Findings a. Two amelanotic elevated lesions, one disc diameter in size are observed in superior temporal arcades, not noted at last dilated exam 4 years prior b. Spectral OCT confirms mildly elevated sub-choroidal mass with intact overlying retina c. B-Scan shows hyper-reflection with posterior echoing d. Previous blood plasma testing showed elevated blood glucose and a decreased glomerular filtration rate. Plasma calcium levels were normal. III. Differential Diagnoses a. Sclerochoroidal calcification i. Idiopathic, typically benign elevated white mass ii. Presents in older, white males, bilaterally, in superior temporal arcades most commonly iii. B-scan shows hyperreflectivity with posterior echoing b. Choroidal osteoma i. Commonly presents in younger patients, located around optic nerve, and is often large in size ii. May result in choroidal neovascularization iii. B-scan shows hyperreflectivity with posterior echoing c. Choroidal lymphoma i. May present as amelanotic lesion ii. B-scan shows medium internal reflectivity iii. Systemic lymphoma must be treated, may be life threatening d. Choroidal melanoma i. May present as amelanotic lesion ii. B-scan shows medium internal reflectivity iii. Metastasis is common, vision prognosis is poor, may be life threatening IV. Diagnosis and Discussion a. Sclerochoroidal calcification i. Fits demographics of patient ii. Typically bilateral presentation iii. Condition is normally benign and idiopathic iv. Choroidal neovascularization and serous retinal detachments have been reported v. Condition is frequently misdiagnosed, has been treated as though it were a melanoma vi. Visual prognosis is typically very good, as macula is not commonly affected vii. Underlying systemic diseases can lead to dystrophic calcium levels viii. Echoing on ultrasonography is important in helping distinguish this calcified tumor from softer sub-choroidal lymphomas and melanomas V. Treatment/Management a. Regular dilated eye exams to monitor for retinal detachment, CNVM recommended b. Blood work should be updated to rule out any underlying systemic pathologies i. Evaluate for kidney function/electrolyte levels ii. Potential treatment of associated systemic conditions c. Proper documentation and patient education to prevent unnecessary intervention d. No treatment of condition is usually warranted e. Bibliography i. Damato BE, Heimann H, Kalirai H, Coupland SE. Age, Survival Predictors, and Metastatic Death in Patients With Choroidal Melanoma: Tentative Evidence of a Therapeutic Effect on Survival. JAMA Ophthalmol. 2014;132(5):605-613. ii. Honavar SG, Shields CL, Demirci H, Shields JA. Sclerochoroidal Calcification: Clinical Manifestations and Systemic Associations. Arch Ophthalmol. 2001;119(6):833-840. iii. Lee B, Pulido JS, Buettner H, Salomão D, Zent CS, Link TP. Intravascular B-Cell Lymphoma (Angiotropic Lymphoma) With Choroidal Involvement. Arch Ophthalmol. 2006;124(9):13571359. iv. Leys A, Stalmans P, Blanckaert J. Sclerochoroidal Calcification With Choroidal Neovascularization. Arch Ophthalmol. 2000;118(6):854-857. v. Schachat AP, Robertson DM, Mieler WF, et al. Sclerochoroidal Calcification. Arch Ophthalmol. 1992;110(2):196-199. vi. Shields CL, Furuta M, Thangappan A, et al. Metastasis of Uveal Melanoma Millimeter-by-Millimeter in 8033 Consecutive Eyes. Arch Ophthalmol. 2009;127(8):989-998. vii. Shields CL, Sun H, Demirci H, Shields JA. Factors Predictive of Tumor Growth, Tumor Decalcification, Choroidal Neovascularization, and Visual Outcome in 74 Eyes With Choroidal Osteoma. Arch Ophthalmol. 2005;123(12):1658-1666. viii. Shields JA. Sclerochoroidal Calcification in Calcium Pyrophosphate Dihydrate Deposition Disease (Pseudogout). Arch Ophthalmol. 1997;115(8):1077-1079. ix. Wong CM, Kawasaki BS. Idiopathic Sclerochoroidal Calcification. Optom Vis Sci. 2014; 91(2):32-37. VI. Clinical Pearls/Conclusion a. B-scan’s ability to show hyper-reflection with echoing can be vital in diagnosis b. Blood work should be ordered or available to rule out any other underlying systemic conditions c. Sclerochoroidal calcification should be considered a differential diagnosis to prevent what may be unnecessary intervention. Spotting mildly elevated, amelanotic lesions during a dilated exam can be an unsettling experience. Consideration of additional risk factors and performance of ultrasonography can allow providers to make an accurate diagnosis of a relatively benign condition.