Giant Conjunctival Cyst of the Orbit in a Patient with Previous Enucleation

Abstract

Introduction

Conjunctival cysts of the orbit development are a rare complication in enucleated patients. They result from the entrapment of conjunctival epithelium within the orbit.

Case Presentation

We report the case of a 25-year-old man with a history of enucleation for a retinoblastoma of the right eye who presented with difficulty in fitting his eye prothesis. On his past medical records, there was no reference to the placement of any orbital implant at the time of the surgery. Biomicroscopy of the right eye revealed a thickened bulbar conjunctiva, an inferior symblepharon, and a translucid central area with vascularization. Imaging was remarkable for a cystic cavity filling the whole right orbit. Biopsy revealed the diagnosis of a conjunctival cyst, and drainage was performed, alleviating the patient’s symptoms.

Conclusion

Orbital conjunctival cysts may pose a clinical problem, and treatment modalities include surgical excision, absolute alcohol injection, and trichloroacetic acid injection.

Introduction

Conjunctival cysts of the orbit development are a possible complication in enucleated patients. They result from the entrapment of conjunctival epithelium within the orbit. These cysts pose a clinical problem, and treatment modalities include surgical excision, absolute alcohol injection, and trichloroacetic acid injection.

Case Presentation

We present the case of a 25-year-old male who complained of difficulty adapting his right eye prothesis. He had a history of enucleation of the right eye due to a retinoblastoma in France when he was 4 years old. No orbital implant was placed at that time. Ophthalmological evaluation revealed a thickened bulbar conjunctiva with a central translucid round area, vascularized, and an associated inferior symblepharon (Fig. 1). Orbital computed tomography (CT) was obtained and revealed a cyst-like structure on the right orbit, bilobated, with hyperdense walls and hypodense content. Magnetic resonance imaging (MRI) image was described as “raised anteroposterior diameter of a cystic-like structure with an ovoid morphology.” There was a structure with a T1 signal which was identical to an atrophied optic nerve, and there was no contrast uptake that indicated an expanding lesion (Fig. 2). These exams were compared with previous orbital CT from 6 years before (after a trauma incident), in which the lesion was not apparent. Drainage of the cystic content and biopsy with partial resection were performed (Fig. 3). Pathology exam revealed fragments of fibrous tissue with a cystic structure covered with conjunctival-like epithelium, with no evidence of dysplasia or malignancy (Fig. 4). Diagnosis of a giant conjunctival cyst of the orbit was assumed. The CARE Checklist has been completed by the authors for this case report, attached as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000535598).

Fig. 1.

Biomicroscopy of the right anophthalmic socket of the patient.

Fig. 2.

Orbital MRI revealing a cyst-like structure on the right orbit.

Fig. 3.

CT of the same patient 6 years earlier, with the hyperdensity of the prothesis and normal right orbit volume.

Fig. 4.

Giant conjunctival cyst of the orbit. The histopathological analysis of the biopsy material showed tissue fragments lined by multilayered cuboidal non-keratinized epithelium, without goblet cells (H&E, a ×200 magnification; b ×400 magnification); and tissue fragments lined by multilayered cuboidal epithelium, focally having goblet cells (yellow asterisk), compatible with conjunctival type epithelium (H&E, c ×200 magnification; d ×400 magnification). Foci of squamous, non-keratinizing epithelium were also present in some of the tissue fragments (H&E, c ×200 magnification; d ×400 magnification). In a subepithelial location, there was a mild to moderate chronic inflammatory infiltrate and mild fibrosis. There were neither signs of dysplasia nor of carcinoma structures. There was also no evidence of the previously diagnosed retinoblastoma. The morphological findings are compatible with a conjunctival cyst.

Discussion

Conjunctival cysts are an infrequent cause of orbital cysts. Their walls consist of stratified squamous epithelium, non-keratinized, and can be primary (congenital) or secondary (spontaneous or subsequent to inflammatory conditions of the conjunctiva) [1]. More than half of secondary conjunctival cysts occur in enucleated patients [2, 3]. The reported incidence varies between 3% (Smit et al. [4]) and 7% (Tyers and Collin) in enucleated patients with an implant placement. This incidence is substantially lower in cases in which no implant was placed, although these may still occur, as reflected in this case report. McCarthy et al. [5] postulated that these cysts may occur using three different pathways: either because of the growth of conjunctival tissue placed within the orbit during enucleation, incarceration of an inverted tongue of conjunctiva after wound closure, or epithelial downgrowth after implant extrusion. In all of the mechanisms, there is buried conjunctiva in the anophthalmic socket. Secondary implantation is deemed to carry the higher risk of conjunctival seeding and cystic development, as the accurate closure of the Tenon’s capsule and conjunctiva is more difficult and the orbital structures have already been disturbed [4]. Symptoms caused by these cysts result from its gradual increase in size. They include progressive inability to retain an eye prothesis, pressure and discomfort sensations in the orbit, and serous discharge [6]. Transillumination may signal the translucid aspect of these cysts, as in our case [7]. In the present case, the patient could not provide a clear history of the type of surgery performed. Medical records from that time claimed that the cause for enucleation was a retinoblastoma and did not mention the placement of any orbital implant. The incidence of conjunctival cysts in patients without implants is not available in the literature, possibly because the majority of patients have one placed to prevent or treat post-enucleation socket syndrome disfiguring symptoms. Clinically, our patient presented an indicative sign of conjunctival cyst, which is its translucid appearance of trasillumination due to its liquid component. The presence of an ovoid structure in the right orbit on the CT/MRI and the initial lack of medical history brought by the patient led to the description from the neuroradiology department of a dismorphic eyeball, later demystified by the patient’s medical records and tissue biopsy. In a patient who has undergone enucleation and presents with an orbital mass, there are several potential causes that should be considered in the differential diagnosis. Infectious and inflammatory causes were considered, but the slow enlargement of the lesion during years rendered this hypothesis less probable. Despite being rare, malignant tumors may develop in an anophthalmic orbit. The most common types of tumors of the anophthalmic orbit include squamous cell carcinoma, melanoma, and sebaceous gland carcinoma. Benign lesions such as dermoid and epidermoid cysts and a mucocele were also considered in our differential diagnosis [8]. Nevertheless, the clinical history and aspects of our patient’s lesion, combined with the MRI T1 and T2 sequences, lead to a conjunctival cyst of the orbit as our most diagnosis. Drainage of the liquid component of the cyst led to a recovery of the ability to fit our patient’s prothesis. Recurrence has not developed within our patient’s 2-year follow-up after his cyst drainage and partial resection. Nevertheless, an option between marsupialization, absolute alcohol, or trichloroacetic acid injection would be a suitable first-line approach, in our opinion, in the case of recurrence. Closure of the Tenon capsule and conjunctiva in two different layers is an essential step in order to prevent conjunctival cyst development [4]. When it develops, complete excision and marsupialization may be tried. However, complete removal is frequently difficult, especially because of the disturbed anatomy of the orbit with fibrosis and adherence of the tissues [6]. When these options fail, alcohol injection and TCA injection may represent alternatives. Hornblass and Bosniak [2] described two cases of orbital cysts after enucleation which were successfully treated with absolute alcohol injection. Aslani and Owji [9] attempted the same technique on 1 patient, with a recurrence after 3 months. Fraunfelder [1] reported a technique of approaching these cases with TCA injection. Aslani and Owji [9] performed this technique on 4 patients, under local anesthesia, with successful results. Pain was noted in a patient with a deep penetrating cyst, and they recommended that general anesthesia be performed in these patients to avoid this side effect.

Take home messages would be that orbital conjunctival cysts are an entity to bear in mind during the follow-up of anophthalmic sockets. Also, surgical options such as drainage carry a high risk of recurrence, and it is important to consider different options if it develops. Other causes of orbital lesions after enucleation must be excluded, such as neoplasms.

Statement of Ethics

This study protocol was reviewed, and the need for approval was waived by comissão de ética CHUdSA on the date of February 10, 2023. Written informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.

Conflict of Interest Statement

The authors have no financial disclosures.

Funding Sources

No funding or grant support was received.

Author Contributions

All authors attest that they meet the current ICMJE criteria for authorship. João Ponces Ramalhão, Beatriz Vieira, Diogo Rodrigues, and Miguel Afonso: acquisition of data and drafting work. João Ponces Ramalhão, Pedro Baptista, Nuno Jorge Lamas, and Maria Araújo: contribution to conception of work and revising critically important intellectual content. João Ponces Ramalhão: corresponding author. Diogo Rodrigues, Beatriz Vieira, Miguel Afonso, Pedro Baptista, Nuno Jorge Lamas, and Maria Araújo: co-authors.

Funding Statement

No funding or grant support was received.

Data Availability Statement

All data generated or analyzed during this study are included in this article and its online supplementary material. Further inquiries can be directed to the corresponding author.