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Year : 2022  |  Volume : 4  |  Issue : 1  |  Page : 53

Anterior segment optical coherence tomography findings in epibulbar choristomas: Case Series

1 Department of Clinical Sciences, Division of Health Sciences, University of Monterrey; Fundación Destellos de Luz, I.B.P, San Pedro Garza García, México
2 Department of Pathology, Hospital Christus Muguerza Alta Especialidad, Nuevo Leon, México

Date of Submission15-Aug-2022
Date of Decision22-Oct-2022
Date of Acceptance24-Oct-2022
Date of Web Publication22-Nov-2022

Correspondence Address:
Manuel Garza-Leon
Av. Ignacio Morones Prieto 4500-Pte, Zona Valle Poniente, 66238 San Pedro Garza García, N.L.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_43_22

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Epibulbar choristomas are benign congenital ocular tumors characterized by abnormal growth in the epibulbar tissue, eyelids, and choroids. Two cases of epibulbar choristomas are described; the first one is a 39-year-old woman who presented a temporally conjunctival mass that had progressed in size accompanied by foreign body sensation and epiphora. Optical coherence tomography (OCT) and surgical resection were performed with a histopathological diagnosis of dermolipoma. The second case is a 7-year-old boy with a congenital corneal lesion in the nasal sector, elevated, vascularized, and with decreased visual acuity. In both cases, OCT showed a thinned epithelium with hyporeflective subepithelial fusiform lesions. OCT is an auxiliary method for the diagnosis of conjunctival lesions, such as epibulbar choristomas.

Keywords: Anterior segment, choristomas, optical coherence tomography

How to cite this article:
Gonzalez-Hernandez M, Martinez-Solis F, Paz-Gonzalez M, Garza-Leon M. Anterior segment optical coherence tomography findings in epibulbar choristomas: Case Series. Pan Am J Ophthalmol 2022;4:53

How to cite this URL:
Gonzalez-Hernandez M, Martinez-Solis F, Paz-Gonzalez M, Garza-Leon M. Anterior segment optical coherence tomography findings in epibulbar choristomas: Case Series. Pan Am J Ophthalmol [serial online] 2022 [cited 2023 Mar 28];4:53. Available from: https://www.thepajo.org/text.asp?2022/4/1/53/361714

  Introduction Top

Dermolipomas are subconjunctival lesions considered as part of the choristomas, being the most frequent congenital epibulbar tumor. They are characterized by abnormal growth of epidermal tissue in the epibulbar area of the eye, eyelids, and choroid.[1] Histologically, they are classified into bone choristomas, complex choristomas, epibulbar dermoids, and dermolipomas, the latter being the most frequent. According to a study by Alarcón-Henao et al., the most frequent location in this type of tumor is the temporal (59%), followed by the superior temporal (36%). Composed of subconjunctival adipose tissue, which clinically is observed as a yellow, mobile, soft consistency mass, without the presence of annexes; in the event of orbital involvement, it can limit eye movements and exophthalmos, which together with the esthetic issue, are the most common causes of consultation.[1]

The association between epibulbar choristomas and Goldenhar syndrome is widely recognized. It is characterized by epibular dermoids or dermolipomas, periauricular appendages, and bone and multisystemic alterations.[2]

In most cases, epibulbar choristomas are limited to the ocular surface; however, dermolipomas account for 0.6% of orbital tumors in children. The subconjunctival lipodermoid may be the forward lip of a large lipodermoid in the orbit. Extension of epibulbar dermolipomas deeply into the orbit is an indication for limited surgery to prevent such complications as motility restriction and obliteration of conjunctival fornices.[3]

Using anterior segment optical coherence tomography (OCT) as a noninvasive auxiliary method has been useful for the diagnosis of lesions in the conjunctiva and cornea;[4] however, the specific use of OCT in lesions such as epibulbar choristomas has not been reported.

  Case Reports Top

Case 1

A 39-year-old woman, presented with no relevant history. The patient observed a conjunctival mass for 2 years which has progressively increased in size, accompanied by epiphora and foreign body sensation.

Ophthalmological examination reported visual acuity of 20/20 in the right eye (RE) and 20/20 in the left eye (LE). Biomicroscopy revealed a temporal subconjunctival lesion in LE [Figure 1]a and [Figure 1]b.
Figure 1: (a) Left eye temporal subconjunctival vascularized, pinkish-yellow lesion, (b) without the presence of adnexa. (c) Fragments composed of mature adipocytes, partially delimited by dense septa of richly vascularized fibroconnective tissue, presenting focal areas of recent hemorrhage. Being consistent with a benign choristoma. (d) OCT images show the area of the scan (red line) and a thinned conjunctival epithelium (46 μm) and many slightly hyperreflective subepithelial areas with fusiform hyporeflective spaces of various sizes (red asterisk space of 256 μm high and 1379 μm wide) that are similar than the dense septa of fibroconnective tissue seen in histopathology (red arrow shows areas of histopathology study). OCT: Optical coherence tomography

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Due to its yellowish appearance and its superior temporal position, subconjunctival prolapse of orbital fat was the most important differential diagnosis, other possible diagnosis was a conjunctival lymphoma or an epibulbar dermoid.

Due to the increase in the size of the lesion and the symptoms, we performed surgical resection of the lesion, with histopathologic analysis. The histopathological findings were consistent with a benign choristoma [Figure 1]c.

An OCT (Primus, Carl Zeiss Meditec, Inc., Dublin, USA) was performed showing a thinned conjunctival epithelium and multiple and variable in size subepithelial hyporeflective spaces [Figure 1]d.

Case 2

A 7-year-old boy presented with congenital partial corneal opacity, as well as a conjunctival lesion accompanied by epiphora, foreign body sensation, and decreased visual acuity in LE.

The right eye had a visual acuity of 20/20 and no alterations. The left eye had 20/200 vision and on slit-lamp examination revealed a conjunctival lesion in the upper nasal quadrant with a corneal opacity adjacent to the conjunctival lesion [Figure 2]a and [Figure 2]b.
Figure 2: (a) Left eye with an elevated, vascularized, yellow, with the presence of hair follicles conjunctival lesion in the upper nasal quadrant), and (b) a corneal opacity adjacent to the conjunctival lesion. (c) OCT images show the area of the scan (red line) and the conjunctiva shows a thinned conjunctival epithelium (86 μm) and some fusiform hyporeflective subepithelial areas, (d) corneal OCT image shows a normal thickness epithelium (68 μm) with a hyperreflective area with peripheral subepithelial thickening (192 to 227 μm) shadowing of the underlying tissue that decreases (105 μm) as it approaches the center of the cornea. OCT: Optical coherence tomography

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The OCT (Primus, Carl Zeiss Meditec, Inc., Dublin, USA) of the conjunctiva shows a thinned conjunctival epithelium and some fusiform hyporeflective subepithelial areas [Figure 2]c. A normal-thickness epithelium with a hyperreflective area with peripheral subepithelial thickening shadowing of the underlying tissue that decreases as it approaches the center of the cornea [Figure 2]d.

Due to its location close to the limbus and coloration, the most important differential diagnoses were: conjunctival lymphoma, amelanocytic nevus and epibulbar dermoid. Due to the age of the patient and the evolution of the lesion, besides those already mentioned, a possible Goldenhar syndrome is considered due to its association with the presence of congenital epibulbar choristomas.

  Discussion Top

OCT, being a noninvasive method and well tolerated by patients, has been described as an in vivo optical biopsy tool for the diagnosis and follow-up of a wide variety of conjunctival degenerative and neoplastic pathologies, such as superficial squamous neoplasms, conjunctival lymphomas, conjunctival amyloidosis, and nevi of different characteristics;[4],[5] however, the tomographic characteristics of dermolipomas are poorly understood. The use of OCT has been reported in cases of patients with epibulbar dermoids and dermolipomas secondary to pathologies such as Goldenhar syndrome, to evaluate the anterior chamber and corneal involvement,[6] without describing the characteristics of the specific conjunctival lesion.

Epibulbar choristomas manifest more commonly from birth, except for dermolipoma, which is asymptomatic until the adult stage,[1] in which a progression of the size of the lesion begins, as in the first reported case where the patient reports an evolution of 2 years before consulting. A clinical diagnosis is made through clinical history and ophthalmological examination, which reports a yellowish subconjunctival lesion in the temporal sector. Differential diagnoses include subconjunctival orbital fat prolapse, conjunctival lymphoma, epibular dermoid, and subconjunctival dermolipoma.[1] Due to the similarity in clinical characteristics, the use of OCT can be helpful in differentiating them, finding a thinned epithelium, with hyporeflective subepithelial fusiform areas similar to the cystic spaces described in patients with conjunctival nevi.[4] When approaching the clinical diagnosis and the pathology report, the diagnosis of a choristoma composed of adipose tissue divided by connective tissue septa is verified, which is consistent with the hyporeflective subepithelial areas of the OCT. To describe, OCT findings can help to differentiate conjunctival lesions from malignant ones which can be described in the OCT like an abrupt change from normal tissue to abnormal findings, the subepithelial dark and homogenous lesions, and most important a hyperreflective and thickened epithelium.

In the second case, a congenital conjunctival lesion is observed in a child under 7 years of age, in the upper nasal sector, yellow, elevated, and vascularized with the presence of hair follicles that also makes us think of an epibular choristoma; when analyzed by OCT, it reports characteristics similar to those of the first case and that differ from other lesions such as lymphoma or amelanotic melanoma due to the presence of hyporeflective areas and the presence of adnexa and corneal involvement.[5],[7]

OCT, like any other technology, has its indications, scope, and limitations and in the case where orbital invasion is suspected, the most important limitation of the use of OCT is that it does not allow us to know the posterior limits of the lesions within the orbit; this information is important for planning a biopsy with the most precise knowledge possible regarding the total size of the lesion, whether it is single or multiple, characteristics of its edges, whether there are other affected tissues or the presence of posterior nutrient vessels or associated vascular alterations or not.

In conclusion, the presence of a thinning of the conjunctival epithelium with hyporeflective fusiform areas in an anterior segment OCT study may be helpful for the diagnosis of epibulbar choristomas; however, information on the deeper limits can be hindered by the artifacts related to tissue composition.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Alarcón-Henao, T., Bernal-Uruchurtu, G., Salcedo-Casillas, G., Gómez-Leal, A., & Rodríguez-Reyes, A. A. (2004). Coristomas epibulbares. Características clinicopatológicas. Revista Mexicana de Oftalmología, 78(4), 182-187.  Back to cited text no. 1
Schmitzer S, Burcel M, Dăscălescu D, Popteanu IC. Goldenhar syndrome – Ophthalmologist's perspective. Rom J Ophthalmol 2018;62:96-104.  Back to cited text no. 2
Mansour AM, Barber JC, Reinecke RD, Wang FM. Ocular choristomas. Surv Ophthalmol 1989;33:339-58.  Back to cited text no. 3
Venkateswaran N, Galor A, Wang J, Karp CL. Optical coherence tomography for ocular surface and corneal diseases: A review. Eye Vis (Lond) 2018;5:13.  Back to cited text no. 4
Venkateswaran N, Mercado C, Tran AQ, Garcia A, Diaz PF, Dubovy SR, et al. The use of high resolution anterior segment optical coherence tomography for the characterization of conjunctival lymphoma, conjunctival amyloidosis and benign reactive lymphoid hyperplasia. Eye Vis (Lond) 2019;6:17.  Back to cited text no. 5
Tiwari U, Kujur R, Paul R, Singh N. Anterior segment optical coherence tomography (ASOCT) in Goldenhar syndrome. J Clin Ophthalmol Res 2014;2:159-60.  Back to cited text no. 6
  [Full text]  
Shousha MA, Karp CL, Perez VL, Hoffmann R, Ventura R, Chang V, et al. Diagnosis and management of conjunctival and corneal intraepithelial neoplasia using ultra high-resolution optical coherence tomography. Ophthalmology 2011;118:1531-7.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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