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Journal of Pediatric Ophthalmology & Strabismus, 2023;60(6):e79–e82
Published Online:https://doi.org/10.3928/01913913-20231026-02

Abstract

Lacrimal gland dacryops are an unusual type of benign epithelial neoplasm. Most of the literature refers to dacryops of the lacrimal palpebral lobe, with orbital lobe involvement less frequent. The authors report a case of dacryops in a 10-year-old child involving the orbital lobe of the lacrimal gland. [J Pediatr Ophthalmol Strabismus. 2023;60(6):e79–e82.]

Introduction

Lacrimal gland epithelial cysts, also known as dacryops, are a rare type of benign epithelial neoplasm. They can appear in any location where there is glandular tissue. Lacrimal palpebral lobe cysts, also called simple dacryops, are by far the most frequent. Involvement of the orbital lobe has also been described, but it is rare. Other locations are accessory glandular (Krause, Wolfring, and Poppof) and ectopic glandular cysts.1

We report a case of dacryops in a 10-year-old child involving the orbital lobe of the lacrimal gland.

Case Report

The patient consented to publish this case report by signing an informed consent.

A 10-year-old boy with episodes of epilepsy was referred to the ophthalmology service after a protocol brain magnetic resonance imaging study. The examination incidentally revealed the presence of an 8-mm diameter nodular lesion in the superior and temporal angle of the right orbit, contacting with the superior and medial part of the lacrimal gland. The lesion displayed hypointensity on T1 and hyperintensity on T2 (Figure 1). No other alterations were found in that study.

Figure 1.
Figure 1.

T2-weighted magnetic resonance image. (A) Coronal and (B) axial sections are shown, which highlights the presence of a hyperintense nodular lesion 8 mm in diameter located in the orbital superotemporal quadrant with involvement of the medial component of the lacrimal gland.

The patient was completely asymptomatic and had visual acuity of 1.0 in both eyes. Examination results were unremarkable, with no palpable mass, ptosis, or proptosis. Intrinsic and extrinsic ocular motility were preserved, and orthophoria was found in primary gaze position. The study of the anterior segment and the fundus showed no pathology either, and the intraocular pressure was 16 mm Hg in both eyes.

Computed tomography displayed a low-density cystic lesion adjacent to the medial margin of the lacrimal gland. Although orbital bone was normal, the most probable diagnosis was dermoid cyst. A multidisciplinary committee in which experts in the field of oculoplastics and orbit assessed the case and it was agreed that in accordance with established protocols, orbital formations with cystic morphology, particularly those exhibiting characteristic attributes of dermoid cysts, usually require surgical intervention as a preventive measure due to the potential risk of rupture and the subsequent possibility of developing secondary intraorbital inflammation. After the removal and analysis of the aforementioned cystic entity, in the event that its completely benign nature was certified, the committee would recommend exclusively regular observation, possibly complemented by imaging studies for follow-up purposes.

Under general anesthesia, a right superior orbitotomy was performed through the superior skin crease. Intraoperatively, the nodule had a cystic appearance and was located behind the septum medially to the lacrimal gland (Figure 2). A complete excision without rupture of the cyst was possible and, finally, we closed using 6-0 absorbable sutures. The sample dimensions were 10 × 10 × 7 mm.

Figure 2.
Figure 2.

An intraoperative view of the orbitotomy with a superior skin crease approach is provided. The presence of a cystic-appearing lesion located posterior to the septum and medially to the lacrimal gland stands out.

A further histological study was done with hematoxylin–eosin staining in which a cyst covered with a simple, non-stratified cuboidal epithelium was observed. In this case, the presence of neither skin appendages nor keratinized epithelial cells was observed, which would be typical findings of a dermoid cyst. Moreover, a subsequent immunohistochemical analysis for cytokeratin AE1-AE3 positive confirmed the epithelial nature of the cyst lining (Figure 3).

Figure 3.
Figure 3.

Histological study. (A) Hematoxylin–eosin (original magnification ×100) showing a cystic wall with a non-stratified epithelial lining. (B) Hematoxylin–eosin (original magnification ×225) showing a cuboidal epithelium without signs of atypia. (C) Immunohistochemical staining for cytokeratin AE1-AE3 positive, confirming the epithelial nature of the cyst lining.

Discussion

Histologically, lacrimal gland epithelial cysts or dacryops are delimited by a fibrous wall and lined with a double layer of non-keratinized columnar epithelium.2 An epidermal-type coating with traces of keratin or the presence of skin appendages is exceptional and suggests the diagnosis of a dermoid cyst.3 Other variations from normality secondary to inflammatory processes are the presence of fibrosis and dystrophic calcification.4

Lacrimal gland epithelial cysts can appear in several orbital or palpebral locations. In 1986, Bullock et al1 proposed a classification of the lacrimal duct cyst based on the locations of lacrimal gland tissue: (1) palpebral lobe cysts, (2) orbital lobe cysts, (3) cysts of the accessory lacrimal glands of Krause (in conjunctival fornix) and Wolfring (in superior tarsal border), and (4) cysts of ectopic lacrimal gland tissue (may be intraorbital).

The orbital location of lacrimal cysts is unusual, despite dermoid/epidermoid cyst being the most prevalent cystic orbital lesion found in children.5 In a retrospective series by Shields6 in which 1,264 consecutive collected orbital tumors, only 12 received the diagnosis of epithelial cyst.

Although the origin of acquired dacryops in adults has traditionally been associated with chronic inflammatory phenomena, its etiology remains uncertain, because most appear without a known pre-disposing factor. In this report, we present a case of infantile dacryops, which are presumed to be of congenital origin and which may be dormant for a period of time until they become manifest.

Periorbital or superficial cysts usually come to the attention of caregivers during the first year of life and present as a painless nodule or bulge on the orbital rim. On the other hand, those located at the orbital level may not be noticed until young adulthood with a gradual-onset, painless proptosis.7 In our case, the patient was completely asymptomatic and the diagnosis came from a routine imaging study for other causes.

Characteristically, orbital dacryops usually appear in children as a slow-growing mass in the region of the lacrimal fossa. Unlike palpebral epithelial cysts, which are by far the most frequent, they may not be visible and may be difficult to palpate.4 On inspection, patients may present with ptosis, exophthalmos, and inferonasal displacement of the globe. Due to its location, if the cyst reaches a considerable volume, it can extend deep into the orbit.

Imaging tests are a resource to obtain information about the anatomical relationship of the tumor with the surrounding structures, as well as to distinguish it from other orbital masses. Ultrasonography images reveal a cyst surrounded by its walls, with poorly reflective content and posterior echoic enhancement.8 On computed tomography they are seen as a low-density, well-defined rounded lesion with normal bony orbit. If it is performed with contrast, the masses are shown with an enhancement of their walls, with a homogeneous material of fluid density.9 Magnetic resonance imaging has been found useful to indicate the presence of cysts, which appear as hypointense on T1 and hyperintense on T2. Finally, nuclear medicine modalities can be supportive, due to Tc-99 uptake by epidermal cysts, although they are not frequently used in clinical practice.10

There are no established treatment protocols. The indications for surgery are those derived from the mass effect itself and unacceptable aesthetic defect. The objective of dacryops surgery is the complete exeresis of the lesion with intact walls, respecting the healthy glandular tissue as much as possible. Various techniques such as cryotherapy-assisted surgical excision have been tried to facilitate the procedure. In cases where dacryops visualization is difficult, trypan methylcellulose blue injection into the cyst may be useful to allow better visualization, although its efficacy is limited.4

Although the surgical results are generally good, there is a risk of complications to be taken into account. A lacrimal production defect is plausible in cases in which the lacrimal gland is damaged, primarily by either the tumor itself or secondarily because of surgery. Another difficulty occurs when complete withdrawal is impossible. In such cases, marsupialization can offer symptomatic improvement, although there will be a risk of recurrence.1

Conclusion

Imaging tests in clinical practice may be a source of incidental findings, as represented in this case. The presence of an orbital nodular lesion in a patient should lead to a thorough examination. In this case, a multidisciplinary committee met and an excisional biopsy was recommended. The usual finding in biopsies of pediatric orbital nodules is dermoid cyst. However, the presence of an epithelial cyst of the lacrimal gland was found, which corresponds to an infrequent benign tumor. The symptoms may appear in relation to the volume and involvement of orbital structures. Although there are no protocols in relation to management, a multidisciplinary approach is recommended to assess cases individually, being able to opt for excision as in the current case.

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