|Year : 2022 | Volume
| Issue : 2 | Page : 490-493
A rare case of rapid evolution of choroidal osteoma
Manoj Soman1, Asmita Indurkar2, Jay U Sheth1, Unnikrishnan Nair1
1 Department of Vitreoretinal Services, Chaithanya Eye Hospital and Research Institute; Department of Vitreoretinal Services, Chaithanya Innovation in Technology and Eyecare (Research), Trivandrum, Kerala, India
2 Department of Vitreoretinal Services, Chaithanya Eye Hospital and Research Institute, Trivandrum, Kerala, India
|Date of Submission||28-Aug-2021|
|Date of Acceptance||24-Nov-2021|
|Date of Web Publication||13-Apr-2022|
Jay U Sheth
Surya Eye Institute and Research Center, Mumbai, Maharshtra
Source of Support: None, Conflict of Interest: None
We present a case of a 19-year-old male with a history of right-sided orbital pseudotumor with suspected Burkitt's lymphoma. His best-corrected visual acuity (BCVA) was 6/36 in the right eye (RE) and 6/6 in the left eye (LE). On fundus examination, the patient had a large choroidal osteoma at the posterior pole with macular scarring, along with two additional satellite osteomas inferior and superonasal to the disc in OD, while OS was unremarkable. After conservative management with oral corticosteroids, the patient demonstrated an additional choroidal osteoma lesion nasal to the disc in the OD which had evolved over a period of merely 3 months. To the best of our knowledge, this is the first reported case to document such a rapid development of choroidal osteoma. This case highlights the need for frequent follow-up of these patients for early detection and monitoring of these lesions.
Keywords: Burkitt's lymphoma, choroidal osteoma, orbital pseudotumor
|How to cite this article:|
Soman M, Indurkar A, Sheth JU, Nair U. A rare case of rapid evolution of choroidal osteoma. Indian J Ophthalmol Case Rep 2022;2:490-3
Choroidal osteoma is a rare benign intraocular tumor of the choroid composed of mature cancellous bone, predominantly seen in young healthy women. Often an isolated finding, these tumors show slow progression usually over many years and are mostly seen in the peripapillary region, sometimes with extension to the macula., We report an observation of a rapid new occurrence of choroidal osteoma lesions in a suspected case of Burkitt's lymphoma. Written informed consent was obtained from the patient for publication of this case report and the accompanying images.
| Case Report|| |
A 19-year-old boy presented to us for evaluation of a localized swelling in the lower lid with complaints of decreased distance vision in the right eye. He gave a history of right orbital proptosis in 2012 which was insidious. Radio imaging (a computed tomography and magnetic resonance imaging of the brain and orbit) was significant for hyperintense superior extraconal mass. He had undergone right orbital tumor excision in a neurosurgical practice elsewhere with histopathology revealing fibrocollagenous tissue with inflammatory infiltrate composed of lymphocytes and plasma cells, suggesting a diagnosis of orbital pseudotumor, and was treated with oral steroids. Recent imaging revealed diffuse infiltration of sinuses and nasal cavity with diffuse enlargement of bilateral parotids and submandibular gland. An extraconal orbital lesion, below the roof and floor of orbits, with compression of extraocular muscles, was noted. A clinical diagnosis of Burkitt's Lymphoma was suspected. He was advised biopsy for immunophenotyping and started on a short course of oral steroids by the consulting physician. He was referred for an ophthalmology evaluation.
The visual acuity at presentation was 6/36 in the right eye (OD) and 6/6 in the left eye (OS). There was mild proptosis, and a firm rubbery mass in the infraorbital region, separate from skin and bone, was made out in OD [Figure 1]f. Hertel's exophthalmometry was significant for a 2-mm difference, confirming proptosis in OD. Restriction of extraocular movements in OD was seen. Fundus examination of OD revealed a large, well-defined flat orange lesion with scalloped margins involving the posterior pole extending beyond the arcades suggestive of choroidal osteoma with pale area within the lesion with visible underlying choroidal vessels [Figure 1]a and [Figure 1]c. Central macular scarring and retinal pigment epithelial (RPE) pigmentation was noted, suggesting an overlying regressed choroidal neovascular membrane (CNVM). Two satellite lesions, one superonasal to disc approximately 2024 × 1601 microns and another inferior to disc 1524 × 842 microns, were seen [Figure 1]a and [Figure 1]c. Examination of the left eye was normal.
|Figure 1: (a) Color Fundus picture shows an orange lesion of osteoma (yellow arrow), scalloped margins with overlying central scarred CNVM and two satellite lesions (blue arrow) (b) New lesion noted at 3 months follow-up (green arrow). (c) Multicolor imaging shows a green orangish area of choroidal osteoma with ill-defined margins. The nasal extent of osteoma is better delineated with the area of decalcification seen as green-yellow in multicolor imaging. (d) Fundus autofluorescence (FAF) shows isoautofluorescence in the peripheral area of choroidal osteoma with hypoautofluorescence in the area of decalcification with RPE loss and choroidal neovascular membrane in the central macular region. (e) Ultrasound depicts the typical hyperechoic choroidal lesion with acoustic shadowing seen in the posterior pole. (f) Profile of the patient, showing right proptosis|
Click here to view
Ultrasound B-scan showed a hyperechoic lesion with marked acoustic shadowing which persisted on low gain [Figure 1]e. Spectral-domain optical coherence tomography (SD-OCT) was significant for a choroidal mass causing compression of overlying vessels with a typical sponge-like appearance [Figure 2]. A subfoveal sub-RPE scar with loss of outer retinal layers was also seen [Figure 2]. Fundus autofluorescence (FAF) imaging showed isoautofluorescence, which confirmed the absence of decalcification in a major part of the lesion, with macular hypoautofluorescence in the area of scarring and partial decalcification [Figure 1]d. Dye-based angiography was deferred in view of previous iatrogenic subglottic stenosis and difficult intubation. OCT-angiography showed an inactive vascular network in the avascular slab [Figure 2]c,[Figure 2]d,[Figure 2]e,[Figure 2]f. The patient was re-examined 3 months later. The primary choroidal osteoma lesions remained unchanged; however, a prominent new lesion of choroidal osteoma approximately 1822 × 1796 microns was noted nasal to the optic disc [Figure 1]b. Simultaneous the superonasal osteoma lesion also showed signs of altered margins. The baseline B-scan through this region was re-assessed at this stage which was noted to be normal [Figure 3]. As the patient was asymptomatic, observation was again recommended with periodic follow-up examinations.
|Figure 2: (a and b) Spectral-domain optical coherence tomography (SD-OCT) shows a choroidal tumor with a sponge-like pattern and choroidal excavation with RPE photoreceptor detachment due to decalcification. Overlying fibrosed choroidal neovascular membrane (CNVM) with focal areas of EZ and RPE loss is also noted. Submacular/sub-CNVM area shows an irregular hyperreflective choroidal mass with loss of detail. (c and d) OCT-angiography (d–f) with structural OCT (c) showing an inactive vascular network in the avascular slab (f)|
Click here to view
|Figure 3: Baseline ultrasound image illustrating the normal scan through the nasal quadrant at the loci of appearance of new osteoma lesion which appeared 3 months later|
Click here to view
| Discussion|| |
Choroidal osteoma is a rare tumor, often demonstrating decalcification, tumor growth, RPE changes, and development of CNV during its course. Choroidal osteoma may also exist as multiple tumors with a bilateral presentation. Tumor growth is reported in 40%–64%, and is often documented along the non-calcified margins at a mean rate of 0.37 mm/year. Faster growth over 1–2 years has been reported in some case reports., Uneven pseudopodial enlargement at margins has been documented by Aylward et al. We report a unique case of choroidal osteoma in a young male with the documented rapid occurrence of new lesions over a short span of 3 months. Such rapidly occurring new lesions have not been reported in the literature to the best of our knowledge. Sambricio et al. described the occurrence of new lesions not associated with the primary lesion in the same eye after 7 years.
The structure of choroidal osteomas and the observation that they may develop in a previously unaffected eye suggests that they are derived from reactive metaplasia of mesenchymal or the adjacent pigment epithelial cells. Although choroidal osteoma occurs primarily in healthy individuals, it has been infrequently reported in other conditions, including pregnancy, Stargardt's maculopathy, orbital inflammatory pseudotumor, Langerhans cell histiocytosis, and intraocular inflammation such as choroiditis. Occurrence of new lesions with enlargement over a few months was seen following recurrent inflammatory pseudotumor in a young woman, speculating the role of hormonal factors and inflammation as a contributory factor for growth. In this particular case also, the patient has a history of pseudotumor orbital mass and is a clinical case of Burkitt's, pending further investigations. Calcification can occur in lymphoma due to abnormal secondary hypercalcemia. However, the occurrence of calcification in lymphomas before treatment is extremely rare, with only a few cases reported in the literature but none involving the ocular tissue. The rapidity of the onset of new lesions in this case could be explained by such possible biochemical events, especially in the setting of lymphoma. In addition, inflammation due to a secondary increase in inflammatory cytokines or pseudotumor growth could induce a secondary heterotopic calcification.
| Conclusion|| |
We believe that our case adds important new information about the presentation of choroidal osteoma in patients with coexistent diseases where the pattern of presentation and progression may be different. Though benign, choroidal osteoma in such patients may have rapid progression with the occurrence of new lesions and may need shorter follow-ups.
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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Trimble SN, Schatz H, Schneider GB. Spontaneous decalcification of a choroidal osteoma. Ophthalmology 1988;95:631-4.
Katz RS, Gass JDM. Multiple choroidal osteomas developing in association with recurrent orbital inflammatory pseudotumor. Arch Ophthalmol 1983;101:1724-7.
Deuri A, Ghosh D, Ekka J, Agarwalla V. Multiple choroidal osteomas in a boy-a rare presentation: A case report. J Med Case Reports 2019;13:238.
Kamalden TA, Lingam G, Sundar G. Bone remodeling in choroidal osteoma monitored by fundus photography and spectral-domain optical coherence tomography. Ocul Oncol Pathol 2015;1:12-7.
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;123:9.
Pámer Z, Kovács B. A case of a fast-growing bilateral choroidal osteoma. Retina 2001;21:657-9.
Mizota A, Tanabe R, Adachi-Usami E. Rapid enlargement of choroidal osteoma in a 3-year-old girl. Arch Ophthalmol 1998;116:1128-9.
Aylward GW. A long-term follow-up of choroidal osteoma. Arch Ophthalmol 1998;116:1337-41.
Sambricio J, Fernández-Reyes M, De-Lucas-Viejo B, Bengoa-González Á, Mencía-Gutiérrez E. A second new choroidal osteoma in the same eye: Differences between them with new imaging techniques. Case Rep Ophthalmol Med 2015;2015:684956.
McLeod BK. Choroidal osteoma presenting in pregnancy. Br J Ophthalmol 1988;72:612-4.
Figueira EC, Conway RM, Francis IC. Choroidal osteoma in association with Stargardt's dystrophy. Br J Ophthalmol 2007;91:978-9.
Azadi P, Khodabande A, Riazi Esfahani M, Ghassemi F, Aliabadi N. Bilateral choroidal osteoma associated with langerhans cell histiocytosis, a coincidence? J Curr Ophthalmol 2019;31:109-12.
Tebben PJ, Singh RJ, Kumar R. Vitamin D-mediated hypercalcemia: Mechanisms, diagnosis, and treatment. Endocr Rev 2016;37:521-47.
Laufer L, Barki Y, Schulman H, Bernstein T, Niv A, Yaniv I. Calcification in an untreated case of Burkitt's lymphoma: Radiographic, ultrasound and CT diagnosis. Pediatr Radiol 1994;24:180-1.
[Figure 1], [Figure 2], [Figure 3]