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Older man presents with acute unilateral decrease in vision
The right eye had a superior temporal yellowish placoid lesion in the mid-periphery with subtle retinal whitening of the superior macula.
A 78-year-old Caucasian man presented to New England Eye Center after he experienced acute painless loss of peripheral vision in his right eye upon waking. He described the vision changes as a “grayish to brownish irregular” spot. He denied flashes, floaters and headaches.
His medical history was significant for well-controlled hypertension, smoldering multiple myeloma, prostate cancer status post-prostatectomy a few years prior, and skin melanoma of the right arm in 2012 status post-excision with clean margins and no known metastasis. His medications were hydrochlorothiazide and omeprazole.
Examination
The patient’s best corrected visual acuity was 20/25-2 in the right eye and 20/20 in the left eye. Pupillary examination showed no evidence of an afferent pupillary defect or anisocoria in light or dark. Extraocular movements and IOPs were all within normal limits. On slit lamp biomicroscopy, there were +1 nuclear sclerotic and posterior subcapsular cataracts in both eyes, and posterior segment examination of the right eye revealed a superior temporal yellowish placoid lesion in the mid-periphery with subtle retinal whitening of the superior macula (Figure 1). The left posterior segment was normal.
Enhanced depth imaging optical coherence tomography of the lesion showed a thin retina with an underlying thin to absent choroid with scleral elevation and a thin layer of subretinal fluid. OCT of the macula demonstrated thickened indistinguishable inner retina layers (Figure 2). Fluorescein angiography of the right eye revealed progressive intensification of hyperfluorescence of the lesion, indicating staining and poor perfusion of the superior retinal artery branches with distinctive Hollenhorst plaque seen superior to the optic nerve (Figure 3). Indocyanine green revealed hypofluorescence of the lesion due to poor to absent choroidal circulation (Figure 4). B-scan ultrasonography revealed an elevated echodense focus with orbital shadowing and hyperreflectivity (Figure 5).
Figure 1. Fundus photo of the right eye shows a superior temporal yellowish placoid lesion in the mid-periphery and moderate retinal whitening superior to the macula. There is a Hollenhorst plaque just superior to the optic nerve.
Images: Sodhi AK, Duker JS
Figure 2. Enhanced depth imaging OCT of the lesion shows a thin retina with underlying thin to absent choroid with scleral elevation with a thin layer of subretinal fluid. The macula demonstrates thickened indistinguishable inner retina layers.
Figure 3. Fluorescein angiography of the right eye staining of the lesion and poor perfusion of the superior retinal artery branches with distinctive Hollenhorst plaque seen superior to the optic nerve.
Figure 4. Indocyanine green reveals hypofluorescence of the lesion due to poor to absent choroidal circulation.
Figure 5. B-scan ultrasonography reveals an elevated echodense focus with orbital shadowing and hyperreflectivity.
What is your diagnosis?
Acute vision loss
The patient presented with two uncommon and unrelated retinal conditions: idiopathic sclerochoroidal calcification and a hemiretinal artery occlusion from a visible embolus.
The diagnosis of idiopathic sclerochoroidal calcification can be made clinically based on funduscopic appearance and appearance on OCT, fluorescein angiography, indocyanine green and B-scan. The differential diagnosis of an amelanotic choroidal mass lesion includes choroidal metastasis, choroidal lymphoma, choroidal osteoma, amelanotic choroidal melanoma, amelanotic choroidal nevus and choroidal granuloma. Choroidal osteomas can appear very similar in appearance but typically present in healthy young women and are most often juxtapapillary. Considering the patient’s history of multiple myeloma and prostate cancer, we reviewed the literature and found no choroidal mass lesions associated with these conditions. Choroidal metastases from skin melanoma are exceedingly rare and usually darkly pigments.
Due to the new hemiretinal artery occlusion, the patient was admitted to the hospital, where his workup included MRI and MRA of the head and neck. A 40% to 50% left carotid stenosis was detected, but the right carotid artery was normal. Transesophageal echocardiogram uncovered a small patent foramen ovale seen only with Valsalva, while ultrasound of the lower extremities showed bilateral acute to early subacute deep venous thrombosis.
Lab results were positive for very elevated factor 8 and mild elevated homocysteine, but calcium and phosphorus levels were normal. Neurological consultation concluded that due to the small size of the patent foramen ovale, paradoxical embolus was an unlikely cause for the retinal artery occlusion.
Discussion
Sclerochoroidal calcification is a rare finding that is often discovered in older Caucasian individuals around the age of 70 to 80 years, but when seen in younger patients, it is frequently associated with systemic conditions. These lesions are usually idiopathic but can be dystrophic calcium deposition, due to severe ocular trauma and chronic intraocular inflammation, or metastatic calcification, due to abnormal calcium-phosphorus metabolism from hyperparathyroidism, vitamin D intoxication, sarcoidosis, hypophosphatemia or chronic renal failure.
Sclerochoroidal calcification is generally considered to be predominantly bilateral, as found in approximately 40% to 80% of cases in the literature. It manifests as multiple discrete yellow placoid lesions superotemporally, usually near insertion of oblique extraocular muscles in a similar way that Cogan scleral calcification plaques insert near horizontal muscles.
Ancillary testing helps confirm the diagnosis. Enhanced depth imaging OCT shows decreased choroidal vascular caliber directly below the lesion and a relatively normal choroid adjacent to the lesion. The lesion also causes a mass effect by elevating the retina and compressing the large choroidal vessels. Sometimes these lesions are associated with focal retinal atrophy secondary to possible decreased choroidal and outer retina perfusion. Typically these lesions do not have associated subretinal fluid, but our case did. On fluorescein angiography, the lesions show early and late staining, and B-scan shows elevated echodense focus with orbital shadowing and hyperreflectivity. Systemic evaluation includes screening tests for calcium-phosphorus metabolism and primary renal tubular hypokalemia metabolic alkalosis syndromes, such as Bartter and Gitelman syndromes.
The majority of patients are asymptomatic and have an excellent prognosis. There have been reports in the literature of approximately 1% of these lesions developing choroid neovascularization, retinal hemorrhages and subretinal fluid; therefore, routine follow-up is advised. Because misdiagnosis of these lesions has led patients to undergo extensive laboratory testing, imaging and unnecessary treatment, recognition of the clinical and ancillary testing features can help avoid this.
Follow-up
After 3 days of inpatient admission, the patient was discharged and placed on Coumadin (warfarin, Bristol-Myers Squibb). He will be followed in our clinic for the hemiretinal arterial occlusion.