A 12-year-old boy visited our clinic for sudden, painless blurred vision and metamorphopsia in his left eye (OS) starting 7 days back. His BCVA was 6/60 OS and 6/6 in right eye (OD). Anterior segment examination was unremarkable in each eye. On fundus examination OS, a yellow-white lesion was seen at the macula with a well-defined geographic border and diffuse, mottled depigmentation of the overlying retinal pigment epithelium (RPE). An elevated gray-green area could be seen at the center of the yellow-white area, with subretinal hemorrhage surrounding it (Top Left). The fundus OD was normal.
B-scan ultrasonography showed a slightly elevated, highly reflective choroidal mass with acoustic shadowing of a “pseudo–optic nerve” (Top Right). The mass persisted even at lower gain. A-scan ultrasonography showed a high-intensity spike. Fluorescein angiography showed early patchy hyperfluorescence with late diffuse staining of the lesion, block fluorescence at the hemorrhage, and leakage at the center—suggestive of a choroidal neovascular membrane (CNVM; Bottom Left). OCT showed a subretinal CNVM with fluid exudation; the RPE was not visible due to back-scattering from subretinal blood (Bottom Right).
The patient was diagnosed with choroidal osteoma with active CNVM OS. Anti-VEGF injections were advised.
Choroidal osteoma is a benign tumor of the choroid composed of mature bone. It is typically found in healthy young females in the second or third decades of life,1 usually as a juxtapapillary lesion that may extend into the macular region.2 Asymptomatic or stable choroidal osteoma can be observed. Long-term poor visual acuity in patients with choroidal osteoma is associated with subretinal fluid, RPE alterations, and subretinal hemorrhage from CNVM3 and decalcification.4
At 10 years of age, 56% to 58% of patients with choroidal osteoma have VA of 20/200 or worse. CNVM occurs in 31% to 47% of patients by 10 years of age,5 and decalcification in 46%.4
The case presented here is a rare one, in that choroidal osteoma was found at an early age in a male patient with marked reduction of vision at his first presentation. Only the macula was involved, with all complications presenting in that compact space: CNVM, subretinal and intraretinal hemorrhage, and serous and hemorrhagic retinal detachment.
Recently, successful treatment of subretinal neovascularization with intravitreal injections of an anti-VEGF agent has been described.6
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1. 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;123:1658-1666.
2. Gass JD, Guerry RK, Jack RL, Harris G. Choroidal osteoma. Arch Ophthalmol. 1978;96:428-435.
3. Ramzi MA, Ahmad MM, Eman K. Review of choroidal osteomas. Middle East Afr J Ophthalmol. 2014;21:244-250.
4. Shields CL, Perez B, Materin MA, Mehta S, Shields JA. Optical coherence tomography of choroidal osteoma in 22 cases: Evidence for photoreceptor atrophy over the decalcified portion of the tumor. Ophthalmology. 2007;114:e53-58.
5. Aylward GW, Chang TS, Pautler SE, Gass JD. A long-term follow-up of choroidal osteoma. Arch Ophthalmol. 1998;116:1337-1341.
6. Guruprasad SA, Neeraj P, Vandana D. Choroidal osteoma with CNVM - successful treatment with intravitreal bevacizumab. Saudi J Ophthalmol. 2011;25:199-202.