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  • Title: Epignathus: two cases.
    Author: Freitas Rda S, Alonso N, Azzolini Tde F, Gianini-Romano G, Tolazzi AR, Busato L, Grande CV.
    Journal: Br J Oral Maxillofac Surg; 2008 Jun; 46(4):317-9. PubMed ID: 17716791.
    Abstract:
    Epignathus is a rare congenital nasopharyngeal tumour that is derived from the upper jaw, palate, and sphenoid bone. It usually protrudes through the mouth, leading to an appreciable risk of obstruction of the upper airway and death soon after birth. We report two cases of unusual presentations that illustrated some uncommon and similar characteristics. Although these tumours are not consistent in origin, number, and differentiation of tissues, both contained structures that were derived from all three layers of germ cells, including different anomalous tissues internally (dental bud, fat and muscular tissues, mucosal epithelium). Externally, they contained normal epidermis (skin with fine hairs). Imaging studies and operative findings showed that both tumours originated from the anterior portion of the sphenoid bone causing a sphenoidal sinus cleft without intracranial communication.
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