Choroidal osteoma is a rare, benign ossifying tumor, typically presenting in the juxtapapillary region in young healthy females with no history of systemic or ocular disease in their second or third decade of life.1–3 The most striking feature of this rare entity is a slightly elevated, well-defined mass, which is yellowish orange in appearance, as characteristically observed on ophthalmoscopic examination.2,4 There may be variation in color due to depigmentation, hyperplasia, or thinning of the overlying retinal pigment epithelium (RPE).1
Choroidal osteoma has been reported to rarely present in association with pregnancy and in eyes with a history of ocular diseases such as optic atrophy, optic neuritis in Behçet’s disease, retinitis pigmentosa, Stargardt’s disease, and bilateral optic nerve sheath calcification.3,5–9 To the best of our knowledge, it has never been shown to present in an eye with previous BRVO. This report describes the de novo appearance of choroidal osteoma occurring years after laser photocoagulation for BRVO.
A 62-year-old Asian man with a known history of diabetes mellitus, hypertension, and asthma presented in April 2009 at the New England Eye Center, Tufts Medical Center, Boston, with an asymptomatic yellowish orange lesion on fundus examination of his left eye during a regular follow-up visit for bilateral branch retinal vein occlusion (BRVO). He had a history of BRVO with macular edema for 10 years in the right eye and 8 years in the left eye that had been successfully treated with sectoral panretinal photocoagulation (PRP) and focal laser bilaterally (Fig. 1). For the past 6 years, he had maintained stable vision of 20/100 in the right eye and 20/30 in the left eye.
Figure 1. (A) Red-free fundus image of the left eye depicting branch retinal vein occlusion. Note the presence of intraretinal hemorrhage typically involving the superotemporal quadrant (black arrow), as well as scattered areas of hemorrhage temporal to the optic nerve head and involving the macular region (green box). (B) Red-free fundus image of the left eye after laser photocoagulation, showing the resolving intraretinal hemorrhages. Note that the previously observed scattered areas of hemorrhage temporal to the optic nerve head and in the macular region have now resolved, and small hard exudates have appeared typically in the macular region (black arrow). These exudates disappeared completely on follow-up dilated fundus examinations, after which the patient maintained stable vision of 20/30 in his left eye for 6 years.
At the time of presentation, his best-corrected visual acuity (BCVA) was 20/200 in the right eye and 20/30 in the left eye. On dilated fundus examination, both eyes showed areas of sectoral PRP, sclerotic vessels in the periphery, and scarring with subretinal fibrosis, owing to the history of laser photocoagulation for BRVO. However, fundus photography of the left eye revealed a well-circumscribed yellowish orange lesion in the macular region, while optical coherence tomography (OCT) showed a slight disruption of the RPE (Fig. 2). The lesion was observed on follow-up visits.
Figure 2. (A) Color fundus photograph of the left eye showing a yellowish orange, well-defined lesion in the macular region (black arrow). (B) OCT image of the left eye, showing a mild disruption of the retinal pigment epithelium (red arrow). There is no evidence of choroidal neovascularization or subretinal fluid collection.
The patient presented 1.5 years after the initial appearance of the lesion with sudden distortion of vision in his left eye and was evaluated with fundus photography, which again revealed the previously observed lesion (Fig. 3). Fluorescein angiography (FA) and OCT features were consistent with CNV and subretinal fluid collection (Fig. 3). A B-scan ultrasonogram revealed a hyper-reflective choroidal mass in the posterior pole, with posterior acoustic shadowing, consistent with the diagnosis of choroidal osteoma (Fig. 3).
Figure 3. (A) Color fundus photograph of the left eye showing a yellowish orange, well-defined lesion with surface depressions and elevations in the macular region (black arrow). (B) OCT image of the left eye, showing an irregular platelike hyper-reflectivity of the lesion (yellow asterisk) extending into the retina, with atrophy of the overlying retinal pigment epithelium (green arrowheads). Subretinal fluid collection can also be observed (red arrow). (C) Fluorescein angiography of the left eye showing leakage of the dye in the region of macula consistent with choroidal neovascularization secondary to the choroidal lesion (black arrow). (D) B-scan ultrasonography of the left eye showing a highly reflective choroidal lesion in the posterior pole (red arrow) and acoustic shadowing just posterior to the mass (green arrow).
The patient was treated with 1.25 mg/0.05 mL intravitreal bevacizumab, leading to subjective improvement in vision. He received a total of eight injections during a period of 14 months, at intervals ranging from 1 to 3 months, for recurrent subretinal fluid collection due to the CNV. His visual acuity then stabilized without therapy for 8 months. At the time of his last follow-up, the BCVA in the left eye was 20/25, and fundus photography and OCT showed a substantial improvement (Fig. 4).
Figure 4. (A) Color fundus photograph of the left eye following completion of treatment with intravitreal bevacizumab, showing a reduction in size, as well as the elevations and depressions of the lesion in the region of macula (black arrow). (B) OCT image of the left eye showing a substantial reduction in the size of the lesion (yellow asterisk). Note that the subretinal fluid collection has resolved with treatment.
This is the first report of a de novo appearance of a choroidal osteoma in an eye with a history of BRVO treated with laser photocoagulation many years prior. The clinical appearance, B-scan ultrasonography, and OCT features were characteristic of choroidal osteoma, as described above.
Gass et al. in 1978 first described four healthy young women with characteristic ophthalmoscopy appearance of a slightly elevated juxtapapillary, yellowish orange choroidal lesion with well-defined margins.4 The pathogenesis of choroidal osteoma is obscure,5 although choristomatous, inflammatory, and hereditary etiologies have been suggested.9–12 These tumors are generally slow-growing, leading to a slow but progressive decrease in vision.2 In a follow-up study of 36 patients, the probability of loss of visual acuity (20/200 or worse) was more than 50% by 10 years.12 CNV is the most frequent cause of visual impairment in choroidal osteoma.6 Despite the benign nature of the tumor, vision is usually compromised due to gradual atrophy of the overlying retina, atrophy of the RPE, subretinal detachment, and accumulation of subretinal fluid or hemorrhage associated with CNV.2,3,13
Ultrasonography and computed tomography (CT) are valuable investigation modalities, revealing high tissue density due to calcification.2 With B-scan ultrasonography, the tumor is acoustically dense at high sensitivity, and shadowing is seen posterior to it, creating the appearance of a pseudo-optic nerve. CT scan reveals a plaque with the same density as bone at the level of choroid.1 Fundus autofluorescence has also been described to assist in diagnosis.14 Recently, OCT imaging is being used to determine the features of choroidal osteoma and demonstrates a platelike hyperintense appearance, involving RPE atrophy, specifically at an early stage of the tumor’s evolution.1
In our patient, choroidal osteoma appeared de novo in an eye previously treated with laser photocoagulation for BRVO. This unique appearance may either be related to a dysfunction of the RPE owing to the history of frequent macular edema secondary to BRVO or a late complication of laser photocoagulation. However, a definitive comment cannot be made in this regard because this occurrence may be purely coincidental.
The definitive treatment for CNV associated with choroidal osteoma has not yet been elucidated. Although photocoagulation, surgical removal of CNV, photodynamic therapy, and transpupillary thermotherapy have been investigated, long-term vision is usually not well-preserved.2 Intravitreal bevacizumab has been shown to be effective in preventing visual impairment in eyes with choroidal osteoma associated with CNV.2,13
In conclusion, we describe the de novo appearance of choroidal osteoma in an eye treated with laser photocoagulation many years prior for BRVO. CNV developed secondary to the lesion, which was treated with intravitreal bevacizumab. The response to treatment was stable, leading to both subjective and anatomic improvement.
- Ide T, Ohguro N, Hayashi A, et al. Optical coherence tomography patterns of choroidal osteoma. Am J Ophthalmol. 2000;130(1):131–134. doi:10.1016/S0002-9394(00)00503-1 [CrossRef]
- Kubota-Taniai M, Oshitari T, Handa M, Baba T, Yotsukura J, Yamamoto S. Long-term success of intravitreal bevacizumab for choroidal neovascularization associated with choroidal osteoma. Clin Ophthalmol. 2011;5:1051–1055. doi:10.2147/OPTH.S22219 [CrossRef]
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- Casaroli-Marano RP, Molina JJ, Adán A, Corretger X. Bilateral choroidal osteoma associated with optic neuritis in Behçet’s disease. Ophthalmic Surg Lasers Imaging. 2010;9:1–4.
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- Song JH, Bae JH, Rho MI, Lee SC. Intravitreal bevacizumab in the management of subretinal fluid associated with choroidal osteoma. Retina. 2010;30(6):945–951. doi:10.1097/IAE.0b013e3181c720ca [CrossRef]
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