Craniofacial Dermoids: An Embryological Theory Unifying Nasal Dermoid Sinus Cysts

• Published in: The Cleft palate-craniofacial journal 2005-01, Vol.42 (1), p.51-57
• Main Authors: Charrier, Jean-Baptiste, Rouillon, Isabelle, Roger, Gilles, Denoyelle, Françoise, Josset, Patrice, Garabedian, Eréa Noël
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dentistry; Dermoid Cyst - congenital; Dermoid Cyst - embryology; Ectoderm; Humans; Infant; Male; Malformations of the nervous system; Medical sciences; Neural Crest; Neurology; Nose Neoplasms - congenital; Nose Neoplasms - embryology; Otorhinolaryngology. Stomatology
• ISSN: 1055-6656; 1545-1569
• DOI: 10.1597/03-106.1

Objective The nasal dermoid sinus cyst (NDSC) is an uncommon congenital lesion presenting as a large panel of midline craniofacial anomalies. The objective of this study was to review and reanalyze embryological hypotheses concerning NDSCs and to propose an embryological theory unifying the various anatomical characteristics of these lesions. The first case of frontal localization of a NDSC extending within the diploetic bone in a 9-month-old boy, presenting as a median frontal fistula with recurrent frontal swelling, 6 months after a mild frontal trauma is presented. Results Complete surgical removal was performed, and there was no evidence of either persistent or recurrent disease 2 years after his surgery. The embryological and anatomical origins of NDSCs are reviewed. This article reexamines and discusses major embryological theories on NDSC pathogenesis and proposes to refute the “prenasal space” theory of Grünwald and rehabilitate a forgotten embryological hypothesis, which unifies the main various clinical presentations of NDSCs.