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Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 749-751

Bilateral choroidal osteoma associated with unilateral limbal dermoid in an infant

1 Institute of Ophthalmology, Hospital Clínic, Barcelona, Spain
2 Department of Ophthalmology, Hospital Sant Joan de Deu, Barcelona, Spain

Date of Submission29-Jan-2021
Date of Acceptance21-Mar-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Maria T Carrion-Donderis
Hospital Clínic Barcelona, Department of Ophthalmology, c/Sabino Arana, 1, Barcelona
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_245_21

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Ocular choristomas are uncommon. In the choroid, two types of ectopic tissues have been described, smooth muscle and osseous tissue, being the latter the most frequent. Choroidal osteomas are benign ossifying tumors, mostly unilateral, with unknown etiology. They are usually found in young women and are rarely diagnosed in infants. Herein we present an 8-year-old girl with congenital unilateral limbal dermoid who, over the years, developed bilateral choroidal osteomas outside the framework of systemic diseases.

Keywords: Choristoma, choroidal osteoma, limbal dermoid

How to cite this article:
Carrion-Donderis MT, Carreras E, Díaz J, Català-Mora J. Bilateral choroidal osteoma associated with unilateral limbal dermoid in an infant. Indian J Ophthalmol Case Rep 2021;1:749-51

How to cite this URL:
Carrion-Donderis MT, Carreras E, Díaz J, Català-Mora J. Bilateral choroidal osteoma associated with unilateral limbal dermoid in an infant. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 18];1:749-51. Available from: https://www.ijoreports.in/text.asp?2021/1/4/749/327728

A choristoma is defined as a congenital overgrowth of normal tissue in an abnormal location.[1] Ocular choristomas are uncommon and the predominant locations include the epibulbar region, the ocular adnexae, and the choroid.[1] Epibulbar dermoid choristomas are most frequently located at the limbus, in the lower outer quadrant, and usually involve both conjunctiva and cornea.[2],[3] In the choroid, two types of ectopic tissues have been described, smooth muscle and osseous tissue, being the latter the most frequent.[1] Choroidal osteomas (CO) are mostly found in young women and 75–80% have unilateral involvement.[1],[4] Herein we present the case of an 8-year-old girl with a congenital unilateral limbal dermoid who, over the years, developed bilateral CO outside the framework of systemic diseases.

  Case Report Top

A 9-month-old girl was referred to our center to evaluate a congenital tumor located at the inferior corneal limbus in the left eye, involving both cornea and conjunctiva [Figure 1]. The diagnosis of limbal dermoid was made and periodic controls were performed in order to detect potential complications related to the limbal dermoid. Over the years, bilateral macular deep orange-yellow lesions were detected. Characteristically, these slow-growing lesions presented geographic borders and branching vessels on their surface [Figure 2]a and [Figure 2]b. An ocular ultrasound showed high acoustic reflectivity and posterior shadowing, consistent with calcium. These findings lead to the diagnosis of bilateral CO. Visual acuity (VA) was assessed monocularly at a viewing distance of 3 m with isolated letter optotypes (H, O, T, or V). The patient was asked to identify the optotypes verbally or by matching optotypes on a lap card. VA at the age of 5 was 1.0/0.2 (right eye/left eye), with astigmatic refractive amblyopia in the left eye. VA stabilized at 1.0/0.4 until the age of 8, when the vision of both eyes decreased to 0.9/0.2, respectively. Optical coherence tomography (OCT) and OCT angiography of the left eye showed choroidal neovascularization (CNV) with subretinal fluid (SRF) [Figure 2]g and [Figure 2]h, but no CNV was observed in the right eye despite the presence of SRF [Figure 2]e and [Figure 2]f. Fluorescein angiography (FA) was performed in order to ascertain the presence of CNV in the right eye but FA early frames did not reveal any CNV. However, FA showed late diffuse staining and hypofluorescent large choroidal vessels in the late frames in both eyes [Figure 2]c and [Figure 2]d. Currently the CNV in her left eye is being treated with intravitreal bevacizumab on “treat and observe” basis with good outcome, and her right eye is under close observation with spontaneous resolution of the SRF.
Figure 1: Limbal dermoid involving cornea and conjuntiva located at the inferior corneal limbus

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Figure 2: Bilateral choroidal osteomas. (a and b) Retinographies showing bilateral macular deep orange-yellow lesions with geographic borders and branching vessels on their surface. (c and d) Late frames of fluorescein angiography showing late diffuse staining and hypofluorescent large choroidal vessels. (e and g) Optical coherence tomography: hyperreflective external layers with subretinal fluid and choroidal mass with hyperreflective dots suggesting bone lamellae. (f and h) Optical coherence tomography angiography

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  Discussion Top

Choroidal osteoma is a benign ossifying tumor of the choroid and it is typically found in females in the second or third decade of life.[5] Due to its silent nature, patients may be asymptomatic and diagnosis is not usually made early in life.[1],[5] Symptomatic patients mostly report a mild decrease in vision, but visual field defects, metamorphopsia, or severe visual loss have also been described.[1],[4],[5] In our patient, early diagnosis of bilateral CO was achieved due to the periodic controls of the epibulbar choristoma, as this corneal tumor may induce significant astigmatic refractive errors.[6]

Pathogenesis of CO is unknown. It has been proposed choristomatous, inflammatory, traumatic, hormonal, abnormal calcium metabolism, environmental or hereditary etiology.[5] In addition, some authors have found associations between CO and other entities such as Langerhans cell histiocytosis and familiar limbal dermoids.[7],[8] However, our patient did not have any history of trauma, intraocular inflammation, systemic diseases, drugs or toxins exposure, or similar familial cases. In addition, she is prepuberal, no endocrinologic abnormalities were detected, and the study of calcium metabolism was unremarkable. Some syndromes have been described in association with ocular choristomas, such as linear nevus sebaceous syndrome and Goldenhar syndrome.[5] Our patient does not have systemic abnormalities to support the diagnosis of the aforementioned syndromes. Nonetheless, the association of limbal dermoid as epibulbar choristoma and bilateral CO as choroidal choristomas in our patient might be in accordance with the theory of CO as of choristomatous etiology. Some facts present in our patient that agree with this theory are an early onset of CO, absence of preceding ocular disease processes, association with an epibulbar choristoma and peripapillary location, a site favored by other developmental tumor.[5],[9] Some features of CO reported as atypical for a choristoma are the sexual preponderance in females and the potential for growth in adulthood. Thus, bone remolded and reshaped during adulthood does not completely exclude choristomatous etiology.[5] Nevertheless, the presence of a limbal dermoid and CO in our patient might just be an epiphenomenon, as there is no sufficient information to establish a significant association.

Regarding the visual loss reported in patients with CO, the principal cause is the development of choroidal neovascularization, present in approximately 30% of cases.[10] Nevertheless, not all eyes with SRF have concomitant CNV; this has been hypothesized to be the result of multiple pinpoint RPE leakage sites over the osteoma.[4] In our patient, intravitreal treatment with an anti-VEGF medication was chosen due to the detection of CNV in her left eye, achieving good anatomical and visual response. Treatment of CNV secondary to CO is not yet standardized but some authors have reported good outcomes with anti-VEGF treatment.[10]

  Conclusion Top

Choroidal osteoma is a rare tumor that might appear during childhood even though this pathology is typically found in females in the second or third decade of life.[5] As vision-threatening complications may develop, such as choroidal neovascularization, periodic controls should be made in order to allow an early treatment and diminish the likelihood of visual loss.[10]

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

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflicts of interest

The authors declare that there is no conflict of interest.

  References Top

Kim BH, Henderson BA. Intraocular choristoma. Semin Ophthalmol 2005;20:223-9.  Back to cited text no. 1
Benjamin SN, Allen HF. Classification for limbal dermoid choristomas and branchial arch anomalies. Presentation of an unusual case. Arch Ophthalmol (Chicago, Ill 1960) 1972;87:305-14.  Back to cited text no. 2
Sommer F, Pillunat LE. [Epibulbar dermoids--clinical features and therapeutic methods]. Klin Monbl Augenheilkd 2004;221:872-7.  Back to cited text no. 3
Alameddine RM, Mansour AM, Kahtani E. Review of choroidal osteomas. Middle East Afr J Ophthalmol 2014;21:244-50.  Back to cited text no. 4
[PUBMED]  [Full text]  
Shields CL, Shields JA, Augsburger JJ. Choroidal osteoma. Surv Ophthalmol 1988;33:17-27.  Back to cited text no. 5
Robb RM. Astigmatic refractive errors associated with limbal dermoids. J Pediatr Ophthalmol Strabismus 1996;33:241-3.  Back to cited text no. 6
Magli A, De Marco R, Capasso L. Presence of bilateral limbal dermoids and choroidal osteomas in a family with inherited limbal dermoids. Ophthalmic Genet 1999;20:101-6.  Back to cited text no. 7
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.  Back to cited text no. 8
Aylward GW, Chang TS, Pautler SE, Gass JD. A long-term follow-up of choroidal osteoma. Arch Ophthalmol (Chicago, Ill 1960) 1998;116:1337-41.  Back to cited text no. 9
Papastefanou VP, Pefkianaki M, Al Harby L, Arora AK, Cohen VML, 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. 10


  [Figure 1], [Figure 2]


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