Abstract
Purpose
We present an unusual case of a congenital lesion presenting with concomitant chronic dacryocystitis. The clinical presentation, examination, management, and histopathology are reviewed.
Observations
A healthy male infant born at 37 weeks gestation presented with an isolated painless 5mm congenital mass of the left medial lower eyelid. Parents also reported episodic epiphora and discharge from the left eye. A surgical excision of the mass revealed an underlying dacryocystitis and the presence of a formed tooth. A dacryocystorhinostomy was performed together with a repair of the soft tissue defect. Histopathology revealed components of disorganized epithelial and mesenchymal tissues including a tooth, skeletal muscle, fat, fibrous tissue, nonkeratinized epithelium, and myelinated nerves. A diagnosis of an odontogenic choristoma of the eyelid was made. Furthermore, a lacrimal sac culture was positive for oxacillin-susceptible Staphylococcus aureus with pathological evidence of chronic dacryocystitis.
Conclusions and Importance
Odontogenic choristoma is a very rare finding in the periocular region with only a few cases reported in the literature. Awareness of clinical findings from this case may allow for a more accurate clinical diagnosis and understanding of the embryologic mechanisms underpinning eyelid and nasolacrimal development. Timely management of this condition is critical to ensure normal oculofacial development and prevent future complications.
1
Introduction
Although eyelid masses are clinically common in the pediatric population, congenital masses of the palpebral region are rare. When located medially, these tumors may lead to obstruction of the lacrimal drainage system. Excluding hordeolums and chalazia, epidermal cysts and dermoid cysts have been found to be the most common pediatric eyelid tumors. However, the differential diagnosis may include meningoencephaloceles, capillary hemangiomas, sudoriferous cysts, nasal gliomas, dacryocystoceles, dacryocystitis, rhabdomyosarcomas, nodular fasciitis, and phakomatous choristomas.
Three cases of palpebral tumors and eight cases of orbital tumors containing teeth have been previously described, with only five in the English literature. , , Although these lesions were initially suggested to be teratomas, the pathologic classification of these tumors is not defined with certainty. We report a congenital, infra-nasal, palpebral mass containing a tooth with obstruction of the nasolacrimal system and dacryocystitis.
While tooth-bearing masses have been well-documented in the orbit and elsewhere, silent palpebral odontogenic choristomas concomitant with chronic dacryocystitis have not been previously described. This very unusual case can not only help clinicians recognize the clinical features of this condition but also allow for a better understanding of the embryological processes involved in orofacial development.
2
Case report
2.1
Patient history
The patient, a healthy male infant presented with a congenital mass of the left lower eyelid, noted upon delivery via cesarean section at 37 weeks of gestation. On initial physical exam, the lesion appeared as a 5 mm subcutaneous pedunculated mass inferior to the punctum of the left lower eyelid ( Fig. 1 ). Occasional mild epiphora and discharge were noted by the parents. The lesion extended 3 mm superiorly beyond the lid margin raising a concern of amblyopia by the referring pediatric ophthalmologist. A partially mucosalized demarcation line could be noted on the superior surface. The remainder of the eyelid structure appeared intact including the inferior punctum and medial canthus. Pupils were equal, round and reactive to light, and no afferent pupillary defect was noted. Fundus examination was unremarkable. The cornea was clear, sclera, and conjunctiva were intact. Extraocular movements were full and the orbits were symmetric. No abnormalities were noted in the contralateral eye. The intraoral exam was normal and the rest of the physical exam was not significant. Six weeks after the initial evaluation, the mass remained grossly unchanged although the parents reported episodic epiphora and discharge. To address the lacrimal system, improve aesthetics, and decrease the risk of amblyopia, the decision was made to excise the mass and explore the nasolacrimal passages.
2.2
Operative findings
After endotracheal intubation, dilation of the upper and lower puncta was performed. Irrigation of the nasolacrimal passages was performed revealing complete obstruction as well as purulent reflux. After injection of local anesthetic, the eyelid mass was excised at its base. Deep to the wound bed, a formed tooth was discovered and easily freed from the surrounding tissue ( Fig. 2 ). A purulent cavity was noted medial to the tooth and the surrounding tissue appeared to be fibrotic and thickened. Any visibly abnormal tissue was excised from the wound and sent for histopathology. At this point, a standard dacryocystorhinostomy was performed with mucosal anastomosis over bicanalicular silicone Crawford stents. No other anomalies were noted within the visible nasal passage. The eyelid defect was closed in layers without requiring any tissue advancement. The patient was discharged home the same day on oral antibiotics.
2.3
Post-operative course
At one week following surgery, the patient was healing well with no evidence of dacryocystitis or epiphora. The lacrimal sac culture was positive for oxacillin-susceptible Staphylococcus aureus with pathological evidence of chronic dacryocystitis. Pathologic examination of the mass revealed components of disorganized epithelial and mesenchymal tissues including a tooth, skeletal muscle, fat, fibrous tissue, nonkeratinized epithelium, and myelinated nerves ( Fig. 3 ). Based on the histological analysis of the tumor, a diagnosis of odontogenic choristoma was made. There was a 2-week and 2-month postoperative follow-up with the patient with no signs of recurrence, an unremarkable physical exam, and no complications ( Fig. 4 ).
