[Clinical and embryological approaches to nasal dermoid sinus cysts]

Nasal dermoid sinus cyst (NDSC) is an uncommon congenital lesion presenting as a large panel of midline craniofacial anomalies. Thirty children with nasal midline masses and/or sinus ostia were surgically treated in the pediatric ENT and cervicofacial surgery department of Trousseau's children's hospital (Paris-France) between 1995 and 2002. All our patients underwent radiological evaluation including CT scan (including axial and coronal planes) and/or MRI (in all three planes) in search of intracranial extension. Thirteen of our patients presented with a midline cyst only, ten had nasal pit only, and seven had combined anomalies. Preoperative radiology and surgery showed an intracranial extension in 3 patients, which exhibited contact of the cyst with the dura. Recurrence being expected if any dermal tissues were left in place, "one-time-excision management" was the rule. CT and MR imaging features were reviewed. The external rhinoplasty procedure resulted in a wide surgical approach, low recurrence and good esthetic results. The embryological and anatomical origins of NDSCs are reviewed. Development of NDSC during embryological development implicates two necessary and sufficient conditions: competence of the ectodermal and mesectodermal cells to form dermoid tissue with epithelial-mesenchymal interactions, and a topographical site of ectodermal inclusion, which fits well with the various clinical presentations of NDSCs. We propose to distinguish NDSCs of anterior topography, located at the anterior skull base level from the basal NDSCs, located at the middle skull base level. We reviewed the various localizations of NDSCs, revisiting a forgotten embryological theory, which unify the various clinical localizations of anterior NDSCs.