A case of choroidal osteoma in a 17-year-old female

Abstract

A 17-year female was referred for complaints of blurring and decreased vision in the right eye. The patient underwent fundus examination, optical coherence tomography and B-scan ultrasonography. A well-defined, yellow-orange colored lesion was present in the posterior pole. This lesion created an echodense lesion on B-scan even with low gain. The patient was diagnosed with choroidal neovascularization (CNV) associated with choroidal osteoma. The patient was treated with seven consecutive intravitreal anti-vascular endothelial growth factor (VEGF) injections over 14 months. Intravitreal anti-VEGF was effective for CNV associated with choroidal osteoma. Long- term follow-up is necessary to resolve the fluids, possible development of CNV, and possible of bilateral occurrence.

Introduction

Choroidal osteoma, first described by Gass et al. in 1978 as a benign ossifying tumor of unknown etiology, is characterized by a slightly irregular elevated yellow–white lesion that typically occurs in healthy females in their early twenties or thirties.[1,2] It occurs unilateral in approximately 80% of cases and is located in the posterior pole.[1] Its diagnoses is based on fundus fluorescein angiography (FFA) and B-scan ultrasound. Associated complications include choroidal neovascularization (CNV) and subretinal hemorrhage.[3] To date, no associations have been reported with ocular or systemic diseases;[2] however, some reports suggest links with Stargard's and polypoidal maculopathies.[4]

Case Report

We report the case of a 17-year-old female who presented to the hospital with complaints of blurring and decreased vision and loss of part of temporal visual field in the right eye. The patient had no systemic disease or family history of retinal lesions. Her visual acuity was 20/125 and 20/20 in the right and left eyes, respectively. The anterior segment was unremarkable bilaterally. Fundus examination of the right eye revealed a subretinal orange 4 × 3 disc diameter lesion with irregular distinct margins located in the superior retina with partial macular involvement [Figure 1a and b]. The fundus examination of the left eye was unremarkable. B-scan indicated an echodense lesion sized 7.2 mm × 8.3 mm × 1.1 mm, with thick choroidal hemorrhage, strong shadowing, and subretinal calcification next to the optic disc. FFA showed dye leakage in the foveal region (CNV) and dye staining in an area of chronic retinal pigment epithelium (RPE) decompensation overlying the lesion [Figure 2]. This hyperfluorescence progressively increased in intensity in the mid-to-late phase. FFA indicated no feeder vessels. Fourier-domain optical coherence tomography (FD-OCT) indicated hyperreflectivity and subretinal fluid [Figure 3a and b]. Unilateral choroidal osteoma with secondary CNV was confirmed.

Figure 1.

Fundus examination: Right eye peripapillary juxtafoveal osteoma of the choroid measures 3 to 4 times disc diameters and is associated with subfoveal choroidal neovascularization (a). After treatment of four consecutive intravitreal anti-vascular endothelial growth factor injections with anatomical improvement over more than 1 year of follow-up (b)

Figure 2.

Fundus fluorescein angiography at 35-s transit reveals dye leakage in the foveal region (choroidal neovascularization) and dye staining in the area of chronic retinal pigment epithelium decompensation overlying the choroidal osteoma

Figure 3.

Spectral- or Fourier-domain optical coherence tomography: a wide range of tumor reflectivity patterns was demonstrated from hyporeflective to isoreflective to hyperreflective; photoreceptor loss was noted over the decalcified areas (a). After four consecutive intravitreal treatment with anti-vascular endothelial growth factor injections (b)

Treatment was started with intravitreal anti-vascular endothelial growth factor (VEGF) injection; no laser treatment was performed. The patient received intravitreal anti-VEGF injection every 2 months. OCT was performed at each visit, indicating subretinal fluid. After 6 months, the clinical examination indicated a dry macular that was confirmed with OCT. There was functional improvement and anti-VEGF injection continued for a total of seven injections over a period of almost 14 months. At the last visit, FFA indicated a stable dry macula, no CNV. The lesion size and vision were stable. The patient remains under observation.

Discussion

Although choroidal osteoma is a benign lesion, calcification occurs mostly in the subretinal space disrupting the RPE due to CNV with permanent loss of vision. Currently, there is no standard treatment for choroidal osteoma. Hence, treatment is targeted toward resolving subretinal fluid and management of CNV.[2] Recent studies have reported that anti-VEGF injection was effective in resolving choroidal vessels, regression of CNV with improvement of vision, and varying duration of follow-up.[5] The characteristics of the lesion and patient response may determine the frequency of anti-VEGF injections. Improvement in vision depends on the location of the lesion, decalcification, RPE involvement, presence of CNV, and resolution of subretinal fluid and hemorrhages.[2] Long-term follow-up is necessary to resolve the fluids, possible development of CNV, and possible of bilateral occurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's patents have given their consent for the patient's images and other clinical information to be reported in the journal. The patient's patents understand that the patient's name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.