Congenital tooth-bearing tumor of the eyelid leading to lacrimal system obstruction

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.