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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 3  |  Issue : 1  |  Page : 6

Choroidal osteoma with choroidal neovascularization treated with bevacizumab in a young male patient


Department of Ophthalmology and Visual Sciences, Paulista School of Medicine, Federal University of São Paulo, São Paulo, Brazil

Date of Submission15-Oct-2020
Date of Acceptance09-Dec-2020
Date of Web Publication10-Feb-2021

Correspondence Address:
Dr. Jorge Henrique Cavalcante Tavares
Department of Ophthalmology and Visual Sciences, Paulista School of Medicine, Federal University of São Paulo, São Paulo
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/pajo.pajo_58_20

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  Abstract 


The purpose of the study is to report a case of choroidal neovascularization (CNV) related to choroidal osteoma treated with intravitreal injections of bevacizumab. This was a case report in which antivascular endothelial growth factor intravitreal injections were used to treat choroidal osteoma-related CNV. A 27-year-old man with a history of blurred vision in his left eye for 15 days presented best-corrected visual acuity (BCVA) of 20/60. Fundus examination, ultrasonography, fluorescein angiography, and optical coherence tomography revealed a nasal and superior juxtapapillary choroidal osteoma with a choroidal neovascular membrane, associated with serous retinal detachment affecting the macula. After three intravitreal injections of bevacizumab, the patient improved to a BCVA of 20/30 and partial resolution of subretinal fluid. Choroidal osteoma with CNV may present a good response to bevacizumab therapy.

Keywords: Bevacizumab, choroid neoplasms, choroidal osteoma, choroidal neovascularization, optical coherence, tomography


How to cite this article:
Cavalcante Tavares JH, Sturzeneker G, Morales MC, Belfort RN. Choroidal osteoma with choroidal neovascularization treated with bevacizumab in a young male patient. Pan Am J Ophthalmol 2021;3:6

How to cite this URL:
Cavalcante Tavares JH, Sturzeneker G, Morales MC, Belfort RN. Choroidal osteoma with choroidal neovascularization treated with bevacizumab in a young male patient. Pan Am J Ophthalmol [serial online] 2021 [cited 2021 Mar 8];3:6. Available from: https://www.thepajo.org/text.asp?2021/3/1/6/309088




  Introduction Top


Choroidal osteoma is a rare benign intraocular choristoma composed of mature bone tissue replacing the choroid.[1] In approximately 90% of cases, it affects young females and is usually located in the posterior pole.[2] Despite being a benign tumor, its typical tendency to slowly grow, to decalcify, and to develop vascular abnormalities may lead to atrophy of overlying photoreceptors and irreversible visual acuity reduction.[3] The probability of a choroidal osteoma to develop choroidal neovascularization (CNV) is 31%–47% in patients followed for 10 years, a complication that is pointed as a major cause of poor visual acuity.[2],[3]

Studying the tumor with fluorescein angiography and optical coherence tomography (OCT) is useful for early identification of vascular abnormalities and treatment planning.[4],[5] Intravitreal injection of anti-vascular endothelial growth factor (anti-VEGF) is currently the main therapeutic procedure recommended to control visual loss due to CNV.[6] We report a case of a young man with serous retinal detachment secondary to CNV associated with a choroidal osteoma who had visual improvement after intravitreal bevacizumab injections.


  Case Report Top


A 27-year-old man was referred to the ocular oncology service with a low visual acuity complaint in his left eye for 15 days. He was amblyopic of the right eye and reported that he had not had ophthalmic follow-up since childhood. His medical history was unremarkable. Best-corrected visual acuity (BCVA) was 20/80 in the right eye and 20/60 in the left eye. There were no changes in anterior-segment examination, and intraocular pressure was 16 mmHg in both eyes, measured with applanation tonometry. Despite the 20/80 visual acuity, funduscopy, OCT, and ultrasonography of the right eye were normal [Figure 1]a.
Figure 1: Fundus photograph montage. (a) Fundus image of the right eye without abnormal findings. (b) Fundus image of the left eye showing a nasal juxtapapillary choroidal osteoma with a choroidal neovascular membrane superior to the optic disc and serous retinal detachment affecting the fovea. Lesion borders are well defined and there is diffuse mottled depigmentation of the overlying pigment epithelium

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An extensive choroidal yellowish-white lesion with pigmentary changes and surface irregularities in the overlying retina was suggestive of choroidal osteoma. The tumor extended from the juxtapapillary area to the nasal and superior mid-periphery of the left eye. The largest dimensions of the tumor were already decalcified. A small hemorrhage within the tumor could be seen superior to the optic disc, alongside a large area of serous retinal detachment that extended temporally, affecting the fovea [Figure 1]b. B-scan ultrasonography showed a highly hyperreflective image in the region of the choroidal plaque, with posterior orbital shadowing, which confirmed the diagnostic hypothesis of choroidal osteoma [Figure 2]. Swept-source OCT (SS-OCT) revealed irregularities in the retinal pigment epithelium (RPE), outer segments of photoreceptors, ellipsoid zone, and external limiting membrane. A round image with hyporeflective lumen and borders inside the outer retina overlying the osteoma, corresponded to outer retinal tubulations. A subretinal hyperreflective image located centrally within the osteoma, superior to the optic disc, was compatible with CNV, associated with subretinal fluid reaching the fovea [Figure 3]a and [Figure 3]b.
Figure 2: Ocular echography of the left eye showing a highly echogenic choroidal lesion with posterior acoustic shadowing. The B-scan showing a slightly elevated and irregular choroidal mass

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Figure 3: Swept-source optical coherence tomography scans previous to treatment. (a) Macular scan showing neurosensory retinal detachment. (b) Horizontal scans through the tumor showing choroidal elevation, an overlying hyperreflective image compatible with choroidal neovascularization (arrowheads), and disorganization of the outer retina. Round structures with hyporeflective lumen and hyperreflective borders inside the outer retina overlying the osteoma were compatible with outer retinal tubulations (arrow). Subretinal fluid is revealed temporal to the osteoma (asterisk)

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Therefore, a regimen of monthly intravitreal injections of bevacizumab (1.25 mg in 0.05 mL) was administered for 3 months. Control of the abnormal vascular network activity was achieved, which resulted in fast regression of the submacular fluid. BCVA of his left eye improved to 20/30, measured 1 month after the third injection. A considerable amount of extramacular serous retinal detachment remained; nevertheless, the patient opted for observation instead of receiving additional intravitreal injections [Figure 4]a and [Figure 4]b.
Figure 4: Swept-source optical coherence tomography scans after treatment. (a) Macular scan showing regression of the neurosensory retinal detachment. (b) Horizontal scans showing persistence of subretinal fluid overlying the lesion (asterisk)

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  Discussion Top


Benign intraocular tumors are sometimes complicated by anatomical disturbance of posterior-segment structures. An atypical case of a young man presenting low visual acuity secondary to an extramacular juxtapapillary choroidal osteoma aggravated by CNV was successfully managed with bevacizumab intravitreal injections.

The large area of decalcified tumor suggested that it had been undiagnosed for a long time. Visual acuity loss typically depends on the development of CNV or chorioretinal changes secondary to decalcification. The pathogenesis of CNV formation is not completely known, but tumor growth and subsequent decalcification could damage the RPE/Bruch's membrane complex, favoring the development of neovascular membrane.[7]

Multimodal imaging in choroidal osteomas has been used for further comprehension and early detection of visual-threatening alterations of the tumor.[8] OCT has a special role in achieving this goal.[4],[5],[8] OCT helps in identifying structural tumor changes that could lead to CNV. Rajabian et al. recently described a case of a patient with a choroidal osteoma who had a 5-year follow-up, showing the initial development of a focal choroidal excavation followed by CNV with the pitchfork sign after 3 years.[9]

In this current case report, CNV, subretinal fluid, choroidal/RPE, and outer retinal irregularities could be documented in a single SS-OCT image. Moreover, round structures with hyporeflective lumen and hyperreflective borders inside the outer retina overlying the osteoma were compatible with outer retinal tubulations. These tubular structures are observed in ocular degenerative and inflammatory diseases usually related to choroidal neovascular membranes and have also been associated with choroidal osteomas.[10]

OCT-angiography (OCT-a) is being intensely used to highlight neovascular membranes in a wide spectrum of chorioretinal diseases, including choroidal tumors. This noninvasive examination allows the acquisition of clear en face view of occult and quiescent choroidal neovascular membranes, which could be difficult to evaluate solely with fluorescein angiography.[11] Multiple authors suggest that OCT-a may play an important role in screening for CNV associated with choroidal osteoma, and that, therefore, it should be performed more frequently.[7],[11],[12]

Intravitreal injections of anti-VEGF drugs have been proposed as the first-line treatment for exudative retinal detachment in choroidal osteomas complicated with CNV, according to the published outcomes of long-term follow-ups.[5],[6] Monitoring of pre- and post-anti-VEGF therapy with OCT-a demonstrates good results in neovascular membrane regression.[5],[11],[12] Since choroidal osteoma is a rare condition, no standard therapeutic regimen has been established. The number of anti-VEGF injections needed to control subretinal fluid is also extremely variable. A retrospective interventional case series analyzed the outcomes of eight patients treated with anti-VEGF intravitreal injections for choroidal osteoma-related CNV, reporting a mean of 12 injections needed to achieve stabilization, while another case series revealed the mean of five injections.[13],[14] Few cases progress to visual acuity worsening despite prompt anti-VEGF treatment. These eyes, however, are frequently associated with decalcification or disciform scar formation in the macular area. Previous reports revealed intravitreal bevacizumab monotherapy as a cost-effective treatment modality.[13],[14],[15] In our case, three injections of bevacizumab led to a fluid-free macula and control of the CNV, which improved the patient's visual complaint.

The limitations of this study were the short follow-up, since the patient did not come back for further evaluations after the 1-month visit following the third bevacizumab injection and the unavailability of complete multimodal imaging in the public healthcare system.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s 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.



 
  References Top

1.
Gass JD, Guerry RK, Jack RL, Harris G. Choroidal osteoma. Arch Ophthalmol 1978;96:428-35.  Back to cited text no. 1
    
2.
Aylward GW, Chang TS, Pautler SE, Gass JD. A long-term follow-up of choroidal osteoma. Arch Ophthalmol 1998;116:1337-41.  Back to cited text no. 2
    
3.
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;123:1658-66.  Back to cited text no. 3
    
4.
Mello PC, Berensztejn P, Brasil OF. Enhanced depth imaging optical coherence tomography of choroidal osteoma with secondary neovascular membranes: Report of two cases. Arq Bras Oftalmol 2016;79:197-9.  Back to cited text no. 4
    
5.
Furino C, Di Antonio L, Grassi MO, Rispoli M, Reibaldi M, Niro A, et al. Choroidal neovascularization due to choroidal osteoma treated with anti-vascular endothelial growth factor therapy: An optical coherence tomography angiography study. Eur J Ophthalmol 2019;29:323-9.  Back to cited text no. 5
    
6.
Khan MA, DeCroos FC, Storey PP, Shields JA, Garg SJ, Shields CL. Outcomes of anti-vascular endothelial growth factor therapy in the management of choroidal neovascularization associated with choroidal osteoma. Retina 2014;34:1750-6.  Back to cited text no. 6
    
7.
Azad SV, Kumar V, Chawla R, Kashyap B, Temkar S, Kumar A, et al. In vivo optical biopsy of choroidal osteoma: A swept source optical coherence tomography-based tumor characterization. Ther Adv Ophthalmol 2020;12:2515841420922740.  Back to cited text no. 7
    
8.
Olguin-Manríquez F, Enríquez AB, Crim N, Meraz-Gutierrez M, Soberón-Ventura V, Ávila I, et al. Multimodal imaging in choroidal osteoma. Int J Retina Vitreous 2018;4:30.  Back to cited text no. 8
    
9.
Rajabian F, Arrigo A, Grazioli A, Sperti A, Bandello F, Battaglia Parodi M. Focal choroidal excavation and pitchfork sign in choroidal neovascularisation associated with choroidal osteoma. Eur J Ophthalmol 2019;1120672119892802. doi: 10.1177/1120672119892802. Online ahead of print.  Back to cited text no. 9
    
10.
Xuan Y, Zhang Y, Wang M, Guo J, Li L, Liu W, et al. Multimodal fundus imaging of outer retinal tubulations in choroidal osteoma patients. Retina 2018;38:49-59.  Back to cited text no. 10
    
11.
Hussam EC, Corinne D, Thibaud M, Emilie A, Laurent K. Contribution of swept-source OCT-angiography in analysis of choroidal osteoma and its quiescent neovascular complications: A case study. Am J Ophthalmol Case Rep 2020;19:100769.  Back to cited text no. 11
    
12.
Carroll WJ, Zhang YS, Jampol LM, Gill MK. The role of optical coherence tomography angiography in ranibizumab-treated choroidal neovascularization in choroidal osteoma. Case Rep Ophthalmol 2020;11:370-6.  Back to cited text no. 12
    
13.
Papastefanou VP, Pefkianaki M, Al Harby L, Arora AK, Cohen VM, Andrews RM, et al. Intravitreal bevacizumab monotherapy for choroidal neovascularisation secondary to choroidal osteoma. Eye (Lond) 2016;30:843-9.  Back to cited text no. 13
    
14.
Mansour AM, Arevalo JF, Al Kahtani E, Zegarra H, Abboud E, Anand R, et al. Role of intravitreal antivascular endothelial growth factor injections for choroidal neovascularization due to choroidal osteoma. J Ophthalmol 2014;2014:210458.  Back to cited text no. 14
    
15.
Rao S, Gentile RC. Successful treatment of choroidal neovascularization complicating a choroidal osteoma with intravitreal bevacizumab. Retin Cases Brief Rep 2010;4:303-5.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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