Craniofacial anomalies with toothy nose

Abstract

A simple subcutaneous lesion such as an epidermoid cyst can present as an aesthetic challenge when located in the nasal tip. Usually the nasal dermoid presents as swelling over the dorsum of nose extending from the nasal tip to the nasal bridge with or without intracranial extensions. In some cases, the overlying skin gets rupture and underlying abnormal structures developed from pluripotent cells of dermoid become visible with variable discharges such as cerebrospinal fluid or sequestrum. Origin of nasal dermoid sinus cyst (NDSC) during embryological development requires two necessary and sufficient conditions. One is competence of the ectodermal and meso-ectodermal cells to form dermoid tissue with epithelial-mesenchymal interactions and second, presence of a topographical site of ectodermal inclusion, which fits well with the various clinical presentations of NDSCs. The case presented here has naso-ethmoidal dermoid cyst with ectopic teeth at the tip of the nose. The patient had a cranial defect with bulging of tissue in naso-ethmoidal region that was in line of 1-13 facial cleft of Tessier. Computerized tomography revealed a bony gap in the region of right fronto-ethmoidal region and brain cyst with craniosynostosis in left frontoparietal suture. Transcranial approach was used to manage the case and all the teeth at the tip of the nose were removed.