Epignathus: two cases.
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.
Freitas
Renato da Silva
Rda S
Plastic and Reconstructive Surgery Unit, Federal University of Paraná (UFPR), Assistance Center for Cleft Lip and Palate (CAIF), Curitiba/PR, Brazil. dr.renato.freitas@gmail.com
Alonso
Nivaldo
N
Azzolini
Thais de Freitas
Tde F
Gianini-Romano
Giovanna
G
Tolazzi
André Ricardo Dall'Oglio
AR
Busato
Luciano
L
Grande
César Vinicius
CV
D002972:Cleft Palate / Q000150:complications; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009303:Nasopharyngeal Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D013101:Sphenoid Sinus / Q000473:pathology / Q000601:surgery; D013724:Teratoma / Q000150:complications / Q000473:pathology* / Q000601:surgery