Introduction: Dentigerous cysts (DCs) involving the orbit are extremely rare. The authors report a unique case of stand-alone orbital DC associated with ectopic canine tooth masquerading as a lacrimal sac diverticulitis with superadded preseptal cellulitis. Case Presentation: A 21-year-old lady presented with left inferomedial swelling, associated with pain and redness of 1-week-duration. A lacrimal sac diverticulitis with preseptal cellulitis was suspected and imaging was requested in view of progression. Computed tomography scan of orbit revealed a well-defined heterogenous mass, with associated hyperdense lesion within the mass, prompting a possibility of a foreign body granuloma. Surgical exploration and excision confirmed the diagnosis of an ectopic canine tooth with associated DC along with excessive adhesion of the cyst to the lacrimal sac wall. Maxillary sinus did not show any abnormality. Lacrimal irrigation was patent with no recurrence noted at 6-month follow-up. Conclusion: This report highlights the presentation of DC with associated ectopic canine tooth as an isolated orbital mass, initially manifesting as preseptal cellulitis with lacrimal sac diverticulitis. Recurrent episodes of infection can lead to synechiae formation, rendering adjacent lacrimal sac more prone to injury. Cyst excision with meticulous dissection yields good outcome.

Dentigerous cysts (DCs) are developmental odontogenic cysts which can rarely occur in association with either an ectopic or a missing tooth [1]. The component of the tooth itself can incite an inflammatory reaction leading to the formation of an epithelium-lined cyst. Most commonly implicated are the third molars, followed by maxillary canines as they erupt late [2]. Maxillary antrum is the most common non-dentate location for DC associated with tooth. However, the occurrence of this distinct clinical entity in the orbit is extremely rare [3‒8].

DCs are usually slow-growing [2]. Symptomatology depends upon the location and the size of the cyst. DCs affecting the orbit can present with globe dystopia, abaxial proptosis, localized periocular swelling, limited ocular motility, palpable mass, epiphora due to secondary acquired lacrimal duct obstruction (SALDO), and visual loss [3‒6, 8, 9]. The present case describes an exceedingly rare case of a DC arising from an ectopic tooth and masquerading as a lacrimal sac diverticulitis with preseptal cellulitis. A written informed consent was obtained from the patient. 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/10.1159/000543279).

A 21-year-old lady presented to the Dacryology clinic with painful swelling over the inner angle of the left eye (LE) of 1-week duration. History of occasional epiphora was present since childhood, which was tolerable. There was no history of trauma or discharge. Her visual acuity in both eyes was 20/20, with normal ocular examination. External examination revealed upper and lower eyelid edema and erythema, with associated displacement of the globe superotemporally (shown in Fig. 1a). The caruncle tissue was thickened with excessive rounding of the medial canthus. No associated conjunctival hyperemia was noted. There was absence of discharge from either punctum. Minimal mechanical limitation of adduction was noted. A week-long course of anti-inflammatory and antibiotic drugs reduced the pain and inflammation, but the swelling continued to progress (shown in Fig. 1b). Lacrimal irrigation from the upper punctum at follow-up revealed a patent lacrimal drainage system (LDS). Owing to the inferomedial location of the lesion with associated preseptal cellulitis which subsided on anti-inflammatory medications and a history of epiphora since childhood, a lacrimal sac diverticulum was suspected.

Fig. 1.

Clinical photograph showing left upper and lower eyelid edema with features of preseptal cellulitis (a). Following course of antibiotics and anti-inflammatory drugs, inflammation subsided with persistent inferomedial swelling (b). CT scans orbit, coronal scans, soft tissue window showing a cystic lesion in the inferomedial aspect of left orbit with a hyperdense structure within the cyst (c). 3D-CT image showing tooth like structure adherent to the orbital surface of maxilla (d). OPG showing normal dentition (e). Postoperative clinical image demonstrating resolution of signs with minimal scarring in the tear trough area (f).

Fig. 1.

Clinical photograph showing left upper and lower eyelid edema with features of preseptal cellulitis (a). Following course of antibiotics and anti-inflammatory drugs, inflammation subsided with persistent inferomedial swelling (b). CT scans orbit, coronal scans, soft tissue window showing a cystic lesion in the inferomedial aspect of left orbit with a hyperdense structure within the cyst (c). 3D-CT image showing tooth like structure adherent to the orbital surface of maxilla (d). OPG showing normal dentition (e). Postoperative clinical image demonstrating resolution of signs with minimal scarring in the tear trough area (f).

Close modal

Computed tomography (CT) scan of the orbit and paranasal sinuses showed a large, heterogenous, radio-lucent mass in the inferomedial aspect of left orbit with a radiopaque, solid lesion attached to the inferior orbital rim in close proximity to the lacrimal sac fossa. The lesion was extending into the medial orbit with globe indentation and absent bony remodeling (shown in Fig. 1c, d).

With a revised clinicoradiological diagnosis of orbital foreign body granuloma or cyst, exploration of the lesion was performed. The cyst surface was incised with egress of turbid, brownish fluid, and an ectopic canine tooth attached to the underlying bone within the substance of the cyst was visualized. The entire cyst wall and ectopic tooth were eventually removed with a small tear to the adherent lacrimal sac wall (shown in Fig. 2a, b). Irrigation to confirm the patency was performed, and the tear in the lacrimal sac wall was sutured and bicanalicular stent intubation was performed. Histopathology showed ulcerated epithelial tissue with stromal inflammation, fibro-collagenous tissue, muscle fascicles, dense lymphomononuclear infiltrates, eosinophilic, and focal lymphoid aggregates (shown in Fig. 2c). Based on clinicopathological findings, the diagnosis of left-sided DC in the inferomedial orbit, arising from an ectopic tooth was confirmed. An orthopantomogram (OPG) to understand the status of the dentition and any missing or ectopic teeth revealed no abnormality (shown in Fig. 1e). Stent extubation was performed at 4 weeks, and lacrimal irrigation confirmed patent LDS. Complete resolution of the signs and symptoms was noted at 1-year follow-up with no evidence of recurrence (shown in Fig. 1f).

Fig. 2.

Intraoperative photograph showing extraction of the ectopic tooth through tear trough incision (a). Gross photograph of the extracted tooth revealing normal canine morphology (b). Histopathological examination revealed an ulcerated epithelium with stromal inflammation; hematoxylin and eosin stain; ×20 (c).

Fig. 2.

Intraoperative photograph showing extraction of the ectopic tooth through tear trough incision (a). Gross photograph of the extracted tooth revealing normal canine morphology (b). Histopathological examination revealed an ulcerated epithelium with stromal inflammation; hematoxylin and eosin stain; ×20 (c).

Close modal

DCs are developmental cysts lined by stratified non-keratinized squamous epithelium. Histopathological evidence has shown that these cysts develop from the remnants of reduced enamel epithelium adjacent to the crown of the unerupted tooth [10‒12]. Long-standing cysts can often become inflamed, rendering the epithelium hyperplastic and occasionally unidentifiable. Secondary infections or hemorrhage into the cyst can further complicate the presentation [13]. Although DCs are usually unilateral, it is imperative to carry out a comprehensive evaluation to rule out syndromic associations such as Gardner syndrome, basal cell nevus syndrome, and cleidocranial dysplasia, in case of bilaterality [14].

Non-dentate DCs have known to involve the maxillary sinus (most common), mandible and coronoid process, orbit, infratemporal fossa, hard palate, facial skin, and nasal cavity [15]. Orbital tooth in association with DC was first described in a 2-year-old child by Cousins [3] in 1887, who presented with an inferior orbital mass. It turned out to be an ectopic tooth with surrounding lacrimal sac upon excision biopsy.

Several mechanisms have been hypothesized for the development of these ectopic teeth in association with DC but is commonly believed to be secondary to the pathological dislocation of the tooth follicle beyond the primitive dental groove. Since the orbit is closely associated with the maxillary and gum area, the tooth can get impacted in the orbit, most commonly the inferior quadrant. Another mechanism is iatrogenic trauma, which can dislodge the tooth, inciting further inflammation [5]. However, there was no history of trauma elicited in our case and OPG confirmed no missing tooth or migration.

Clinical manifestations of DC associated with ectopic or missing tooth usually begin to arise between the second and third decade of life, similar to the present case, depending on the affected site and size [2]. Malar edema, facial pain, sinusitis, and SALDO can occur when the maxillary sinus is the affected site. When present intranasally, recurrent sinusitis and rhinitis are common [16]. Although there are numerous reports with DCs involving the LDS as well as the orbit, cases with isolated orbital involvement are rare, as highlighted in Table 1. Cases with concurrent sinonasal involvement were excluded from this literature review [3‒8, 17, 18].

Table 1.

Summary of reported cases of orbital tooth associated with DC [3‒8]

S. No.Author/yearAge, years/sexClinical featuresImagingDiagnosisManagementOutcome [follow-up]
Cousins [3] (1887) 2/NM Left inferior non-tender orbital mass NM Ectopic canine tooth Excision biopsy + tooth extraction NM 
Savundranayagam [4] (1972) 23/M Left inferomedial eyelid tissue loss and loss of vision NM Ectopic third molar with secondary osteomyelitis NM NM 
Odufuwa and Rose [5] (2001) 65/M Left inferomedial painless swelling CT – inferomedial cystic mass extending into the NLD DC with missing canine tooth in NLD Tooth extraction + DCR NM 
Naini [6] (2010) 14/F Incidental finding X-ray – tooth in left inferior orbit Ectopic third molar tooth NM NM 
Garde et al. [7] (2012) 19/M Right sided facial pain CT – impacted canine tooth seen just beneath the orbital floor Missing canine tooth Excision biopsy + tooth extraction NM 
Theiveegan et al. [8] (2023) 7/F Inferotemporal non-tender orbital mass with cutaneous sinus MRI – inferotemporal cystic lesion with internal solid component Ectopic molar tooth with associated cyst Excision biopsy + tooth extraction No recurrence [2 months] 
Our case 21/F Left inferomedial painful swelling CT – cystic lesion with tooth in inferomedial orbit DC with ectopic canine tooth Excision biopsy + tooth extraction No recurrence [6 months] 
S. No.Author/yearAge, years/sexClinical featuresImagingDiagnosisManagementOutcome [follow-up]
Cousins [3] (1887) 2/NM Left inferior non-tender orbital mass NM Ectopic canine tooth Excision biopsy + tooth extraction NM 
Savundranayagam [4] (1972) 23/M Left inferomedial eyelid tissue loss and loss of vision NM Ectopic third molar with secondary osteomyelitis NM NM 
Odufuwa and Rose [5] (2001) 65/M Left inferomedial painless swelling CT – inferomedial cystic mass extending into the NLD DC with missing canine tooth in NLD Tooth extraction + DCR NM 
Naini [6] (2010) 14/F Incidental finding X-ray – tooth in left inferior orbit Ectopic third molar tooth NM NM 
Garde et al. [7] (2012) 19/M Right sided facial pain CT – impacted canine tooth seen just beneath the orbital floor Missing canine tooth Excision biopsy + tooth extraction NM 
Theiveegan et al. [8] (2023) 7/F Inferotemporal non-tender orbital mass with cutaneous sinus MRI – inferotemporal cystic lesion with internal solid component Ectopic molar tooth with associated cyst Excision biopsy + tooth extraction No recurrence [2 months] 
Our case 21/F Left inferomedial painful swelling CT – cystic lesion with tooth in inferomedial orbit DC with ectopic canine tooth Excision biopsy + tooth extraction No recurrence [6 months] 

M, male; F, female; NLD, nasolacrimal duct; DCR, dacryocystorhinostomy; NM, not mentioned; CT, computed tomography; MRI, magnetic resonance imaging.

The present case manifested as a unilateral left-sided lacrimal diverticulitis with preseptal cellulitis at presentation. Antibiotics and anti-inflammatory medications reduced the symptomatology, but the inferomedial paralacrimal lesion persisted with patent LDS, raising a suspicion of lacrimal sac diverticulum. Progression in size further prompted the need for imaging. Lacrimal sac diverticulum usually arises from the inferolateral aspect of the sac wall, and may or may not be associated with features of inflammation. Although the clinical presentation is varied, its occurrence as a stand-alone inferomedial mass is known with anatomically patent LDS [17].

CT of the orbit and paranasal sinuses, with or without 3D reconstructed images, is the imaging modality of choice. Dacryocystogram may be added in cases where lacrimal involvement is suspected. The size, location, and relation of the ectopic tooth with surrounding structures are better delineated on the bone window as well as 3D-reconstructed images, along with an assessment of adjacent bone (thickness, presence of erosion, or remodeling). Concurrent sinus involvement or soft tissue edema is common [18]. The present report is unique as the orbital tooth associated with DC was entirely intra-orbital with no concurrent sinonasal involvement, clinically mimicked a lacrimal sac diverticulum, had well-defined borders, and was adherent to the orbital surface of the maxilla. The lacrimal sac could not be appreciated separate from the lesion. The hyperdense structure, on retrograde review, resembled the canine tooth. Odufuwa and Rose [5] in 2001 reported a similar case of an inferomedial orbital mass with dilated nasolacrimal duct (NLD) with minimal signs of inflammation, following trauma. Intraoperatively, the tooth was impacted within the NLD and, therefore, extracted along with dacryocystorhinostomy (DCR). An OPG in preoperative work-up may serve as an adjunct tool to rule out any missing tooth or abnormal dentition [11, 19].

An orbital teratoma can present with similar features clinically and radiologically and can be differentiated based on age at presentation and distinct histopathological features [20]. Secondary involvement of the orbit by maxillary or mandibular ameloblastoma is well-documented and differs distinctly on imaging and histopathology [21]. Odontogenic fibroma and myxoma can mimic a maxillary DC, but orbital involvement has not been documented yet [22].

Once inflamed or infected, the management of DCs become challenging [3]. Multiple episodes of cyst inflammation can lead to adherence of the cyst wall with the adjacent structures and lacrimal sac. Over the years, various surgical approaches have been employed to deal with DCs in and around the orbit. Intranasal cysts are best excised endoscopically, whereas DCs of the maxillary antrum can be either approached through an external route involving Caldwell-Luc or Weber Fergusson approach or an endoscopic route via a maxillectomy or Denker’s approach [10, 13, 23]. One must be cautious while performing antrostomy through the Caldwell-Luc approach to avoid damaging the NLD, otherwise leading to iatrogenic SALDO warranting a DCR [24]. Surgical enucleation of the cystic lesion with complete tooth extraction is the procedure of choice as it bears the least chance of recurrence [15]. Rarely, cyst marsupialization may be preferred in cases where adjacent bone thinning is present, as enucleation bears a risk of collateral damage. In this case, cyst excision with tooth extraction was performed using a tear trough incision, which provided ready access to the inferomedial orbit and better visualization of the relationship of the lesion with the LDS. Adhesions with the lacrimal sac wall were severed with minimal trauma to the lacrimal sac.

Ectopic tooth with DC must be included in the differential diagnosis of inferomedial orbital lesions presenting with or without signs of inflammation. A simultaneous OPG may be a useful adjunct to look for the origin and nature of the ectopic tooth and the dentition status [20]. Surgical excision of the cyst with tooth extraction remains the mainstay of treatment [21]. It might be interesting to explore the application of sclerosing agents in cases where the lesion may not be amenable to total excision or due to the proximity of delicate orbital tissues.

A written informed consent was obtained from patient for publication of the details of their medical case and any accompanying images. Ethical approval was not required for this study in accordance with the local as well as national guidelines.

The authors have no conflicts of interest to declare.

This study was supported by Hyderabad Eye Research Foundation. The funder had no role in the design, data collection, data analysis, and reporting of this study.

All authors met the authorship criteria. S.D. drafted the manuscript. N.B., M.J.A., and A.A. critically edited and revised the manuscript. A.A. and S.D. were involved in data and image acquisition. N.B. conceptualized the article and serves as the corresponding author. All authors were involved in the management of the patient and approved the final version of the article to be published.

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.

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