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In 1991, an 80-year-old woman had metamorphopsia and visual loss in the left eye. Visual acuity was 20/25 OD and 20/30 OS. Visual fields plotted on a Goldmann perimeter showed a large inferonasal scotoma in both eyes. Fundoscopy revealed bilateral tumorlike, sclerochoroidal calcifications localized, with the largest part anterior to the upper temporal arcades, and extended, with a smaller part posterior to the arcades (Figure 1). These lesions corresponded with the visual field defects.
Fundus photographic views at initial examination show superotemporal sclerochoroidal calcifications in both eyes. Note a hemorrhage located centrally and a rim of hemorrhages located inferiorly of the calcifications at the border of a subretinal membrane in the left eye. No hemorrhages were observed in the right eye.
In the left eye, we noted choroidal neovascularization at the inferior border of the sclerochoroidal calcifications, hemorrhages surrrounding the membrane, and a single hemorrhage centrally in the area of the calcifications. The fluorescein angiography of the left eye showed hypofluorescence of the sclerochoroidal calcifications in the arterial phase. In the venous phase, a well-defined choroidal membrane was identified that was 1 disc area large. Moreover, progressive irregular fluorescence of the sclerochoroidal calcifications was noted, with window defects in areas with depigmentation of the retinal pigment epithelium, and with several hyperfluorescent lesions suspicious for small vascular tufts lying beneath the retina on the surface of the calcifications (Figure 2, left). In the late phases of the fluorescein angiogram, we noted intense hyperfluorescence of the sclerochoroidal calcifications in both eyes (Figure 2, right). Superior to the central hemorrhage and temporal to the neovascular membrane at the inferior border of the sclerochoroidal calcifications, we noted 2 well-demarcated zones of more profound depigmentation of the retinal pigment epithelium. These areas showed pronounced late hyperfluorescence and were suspicious for foci of subretinal neovascularization. After laser photocoagulation of the well-defined choroidal membrane, formation of a flat atrophic scar was observed. This scar enlarged during the 7-year follow-up period (Figure 3, right). The sclerochoroidal calcifications and the 2 foci suspicious for subretinal neovascularization remained unchanged and no new hemorrhages were noted.
Left, Venous-phase fluorescein angiogram of the left eye at initial examination demonstrates a well-defined subretinal neovascular membrane. Right, Late-phase angiogram of the same eye shows diffuse leakage from the membrane and staining of the sclerochoroidal calcifications. Note also the linear and pinpoint early hyperfluorescent lesions on the surface of the calcifications and profound late hyperfluorescence of a focus superior to the central hemorrhage, as well as another focus temporal to the neovascular membrane at the inferior border of the calcifications.
Fundus photographic views after 7 years show a hemorrhage at the inferior border of the sclerochoroidal calcifications in the right eye. A large atrophic laser scar is noted in the left eye.
In the right eye, we observed a similar aspect of the sclerochoroidal calcifications, with 1 more obvious lesion at the inferior border of the mass. This lesion was clinically atrophic but was associated with intense hyperfluorescence and with recurrent hemorrhages (Figure 3, left). The atrophic aspect of the lesion remained unchanged and we never diagnosed a choroidal neovascular membrane. However, we presume that a similar lesion has preceded the neovascular growth in the left eye.
Ultrasonography showed a mass with 100% spikes and progressive attenuation of the spikes in both eyes (Figure 4, top). On the B scan, the characteristic findings of highly reflective calcification with acoustic shadowing were seen (Figure 4, bottom). A computed tomographic scan confirmed the presence of a large calcified mass in the posterior pole of both eyes (Figure 5). The sclerochoroidal calcifications remained nearly unchanged during the long follow-up period, with only a mild increase of the associated atrophic changes. A recent indocyanine green angiography was performed, with the aim to detect occult choroidal neovascularization at an early stage. The indocyanine green angiogram revealed patchy hypofluorescence in all phases in the areas with calcifications and atrophic changes. Moreover, there was a faint late hyperfluorescence at the borders of the mass. No peculiar hyperfluorescence was noted of the lesion with recurrent hemorrhages in the right eye. During the patient's last examination, a bilateral cataract was noted, and visual acuity was 20/30 OU.
Top, Ultrasonography shows a 100% spike and progressive attenuation of the spikes. Bottom, B scan shows highly reflective calcification with acoustic shadowing.
Axial computed tomographic section shows a large sclerochoroidal calcified mass in both eyes.
Our patient had a multinodular goiter and mild hyperthyroidism that were treated medically. Screening of calcium metabolism showed no disturbances and no arguments for primary hyperparathyroidism, but it was confirmed that the patient had secondary hyperparathyroidism caused by vitamin A deficiency, which is a common finding in elderly persons.
Sclerochoroidal calcifications are usually observed in the midperipheral fundus of elderly patients who are asymptomatic. The lesions are frequently bilateral and localized in the superotemporal quadrant.1-4 Two types of sclerochoroidal calcifications are identified: relatively flat plaque-like lesions and elevated tumorlike lesions, ranging up to 6 mm in height.3
Recently, a first case of sclerochoroidal calcifications with choroidal neovascularization was reported.5 In this woman (aged 74 years), bilateral sclerochoroidal calcifications were discovered during a routine examination. One plaque of calcifications was associated with lipid exudates and serous detachment. Fluorescein angiography was indicative of ill-defined choroidal neovascularization. Argon laser photocoagulation induced regression of serous detachment and exudates, and there was no recurrence during a 2-year follow-up (S. Y. Cohen, MD, personal communication, December 14, 1998).
We report the second case in the world literature of sclerochoroidal calcifications with a well-defined subretinal membrane and several foci with hemorrhages and hyperfluorescence suspicious for choroidal neovascularization. During the 7-year follow-up, these foci did not evolve to subretinal membranes and there was no recurrence of choroidal neovascularization from the well-defined membrane after laser treatment.
Several studies of series of patients with sclerochoroidal calcifications report an absence of choroidal neovascularization. Thirty-four cases have been studied by Sivalingam et al,1 Munier et al,2 and Schachat et al3 and no choroidal neovascularization was observed in their cases. With fewer than 50 reported cases without choroidal neovascularization, the incidence of this complication is probably extremely low. Moreover, sclerochoroidal calcifications do not expand close to the macular area, and the risk of visual loss because of associated choroidal neovascularization seems to be extremely low.
On the other hand, the case of Cohen et al5 and our experience show that serous detachment and exudative lesions may occur in the context of sclerochoroidal calcifications that are complicated by choroidal neovascularization, and that laser treatment may be effective in reducing the risk of developing additional visual field loss. In 2 cases treated with lasers, no recurrence of the choroidal neovascularization was observed.
Corresponding author: Anita M. Leys, MD, PhD, University Hospitals Leuven, Department of Ophthalmology, Capucijnenvoer 33, 3000 Leuven, Belgium (e-mail: email@example.com).
Leys A, Stalmans P, Blanckaert J. Sclerochoroidal Calcification With Choroidal Neovascularization. Arch Ophthalmol. 2000;118(6):854–857. doi:
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