Congenital epignathus associated with a cleft palate: a case report

Abstract

Background Epignathus is a rare, benign, congenital teratoma of the hard palate with an estimated incidence of 1 in 200,000 live births. Epignathus frequently leads to a high mortality (80–100%) due to airway obstruction in the neonatal period. Case presentation We report a case of successful management of a rare oropharyngeal teratoma in a African newborn girl who was referred to our institute with a large protruding intraoral mass, combined with cleft palate, causing some respiratory compromise. The palatal mass was removed on the fifth day after birth, and a palatoplasty performed on day 30. Conclusion Epignathus is a life-threatening condition at the time of delivery. Appropriate management begins with securing the airway, followed by complete resection of the tumor.

Epignatus: manejo quirúrgico y multidisciplinar. Un caso clínico.

“…La cirugía de resección tumoral es el tratamiento de elección en caso de epignatus. Dicha resección debe ser completa, y posteriormente establecer un tratamiento sobre las secuelas derivadas del epignatus 3,9,10 . La resecabilidad del epignatus posee valor pronóstico, aumentando el riesgo de recidiva maligna en caso de ser incompleta 3 .…”

“…En cuanto al seguimiento del paciente, cabe destacar la presencia de 2 malformaciones mecánicas craneofaciales ocurridas en nuestro caso, derivadas directamente del efecto de masa que producía el teratoma. Una de ellas es la fisura palatina, que es la malformación mecánica más frecuente asociada a epignatus 10 . En nuestro caso afectaba únicamente a paladar blando posterior.…”

Palatal teratoma associated with cleft palate- A rare case report

“…Epignathus (fetus en fetu) are highly differentiated teratomas and have a high mortality rate. 10,11 Teratomas occur at the ratio of 1 in every 4000 births with a slight female predilection. These are mostly benign but malignant cases have also been reported.…”