Choroidal osteoma is a rare, benign, often unilateral tumor characterized by the growth of cancellous bone involving the choroid.1 The diagnosis is mainly clinical; multimodal imaging and ultrasonography provide useful information for the diagnosis.
We present a case of a young female patient (10 years old) complaining of bilateral visual acuity (VA) reduction (right eye [OD] > left eye [OS]). Best-corrected VA (BCVA) was 20/63 for OD and 20/25 for OS. Fundus examination revealed bilateral, almost symmetric, round orange-yellow lesion involving the entire posterior pole and the papillary area (Figures 1A and 1B), suggestive of choroidal osteomas. Macular retinal hemorrhage was found OD. Ultrasonography B-scan examination showed bilateral, slightly elevated, hyperechogenic lesion, with posterior sound attenuation typical of choroidal osteomas (Figures 1A and 1B). Structural optical coherence tomography (OCT) showed the presence of a big, subfoveal, mainly hyperreflective lesion with posterior shadow effect OD combined with subretinal exudation, atrophy of external retinal layers at the borders of the lesion, and the loss of physiologic foveal profile (Figure 1C). Little subfoveal hyperreflective accumulation with attenuation of the ellipsoid zone was detected OS (Figure 1D). Choroidal vessels resulted not clearly visible in OU; latticework reflective pattern with hyperreflective dots and hyporeflective cavernous spaces were detected in parafoveal scans. Swept-source OCT angiography (OCTA) (PLEX Elite 9000; Carl Zeiss Meditec, Dublin, CA) detected a foveal avascular zone enlargement of the deep capillary plexus in OU, with partially preserved choriocapillaris (Figures 2A and 2B). Interestingly, OCTA clearly showed the presence of a CNV in OD (Figure 2A); however, OCTA also detected a CNV in OS without signs of exudation on structural OCT (Figure 2B).
Multimodal imaging of bilateral choroidal osteoma. Ultra-widefield multicolor images showed the presence of an extensive yellowish lesion corresponding to echogenicity attenuation showed by ultrasound for the right eye (OD) (A) and left eye (OS) (B), respectively. Macular hemorrhage is shown OD, as well. Structural optical coherence tomography (OCT) showed subretinal mixed-reflective material accumulation OD corresponding to choroidal neovascularization (C); Structural OCT detected hyperreflective retinal bands rarefaction and multiple small hyperreflective subretinal accumulations OS (D).
Optical coherence tomography angiography (OCTA) of bilateral choroidal osteoma. OCTA reconstructions show the following finds for the right eye (OD) (A): preserved superficial capillary plexus, foveal avascular zone enlargement in the deep capillary plexus, well-defined choroidal neovascularization (CNV) in the avascular zone, partially preserved choriocapillaris, and a complex peri-lesional vascular organization with thin tortuous vessels surrounding the lesion with poorly defined choroidal vessels. B-scan structural OCT with over-imposed flow information is provided, as well. The same findings are also visible in the left eye (B). After a single 1.0 mg/0.025 mL aflibercept injection, total exudation regression and CNV reduction was seen OD (C: baseline; D: 3-month follow-up).
The patient underwent a single 1.0 mg/0.025 mL aflibercept (Eylea; Regeneron, Tarrytown, NY) injection OD under anesthesia. After 3 months, BCVA improved to 20/25, and structural OCT showed the total regression of the exudation in OD, together with a reduction of the hyperreflective lesion (Figures 2C and 2D). OCTA confirmed these findings, documenting a reduced CNV lesion. OS was found stable in terms of both BCVA and imaging findings; the combined use of OCT and OCTA allowed to document the clinical inactivity of the CNV along the entire follow-up, suggesting the possible “quiescent” nature of the lesion.2–4
In conclusion, multimodal imaging allowed a deep study of microstructural changes occurring in our patient. Moreover, these approaches resulted very usefully also during follow-up as they allowed to reliably document anti-vascular endothelial growth factor effects on the lesion in OD and the stability of the nonexudative CNV in OS, without the need of invasive investigations.
- Alameddine RM, Mansour AM, Kahtani E. Review of choroidal osteomas. Middle East Afr J Ophthalmol. 2014;21(3):244–250. doi:10.4103/0974-9233.134686 [CrossRef]
- Querques G, Srour M, Massamba N, et al. Functional characterization and multimodal imaging of treatment-naive “quiescent” choroidal neovascularization. Invest Ophthalmol Vis Sci. 2013;54(10):6886–6892. doi:10.1167/iovs.13-11665 [CrossRef]
- Carnevali A, Cicinelli MV, Capuano V, et al. Optical coherence tomography angiography: A useful tool for diagnosis of treatment-naïve quiescent choroidal neovascularization. Am J Ophthalmol. 2016;169:189–198. doi:10.1016/j.ajo.2016.06.042 [CrossRef]
- Carnevali A, Sacconi R, Querques L, et al. Abnormal quiescent neovascularization in a patient with large colloid drusen visualized by optical coherence tomography angiography. Retin Cases Brief Rep. 2018;12Suppl 1:S41–S45. doi:10.1097/ICB.0000000000000648 [CrossRef]