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Epignathus: Congenital Teratoid Syndrome

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Epignathus is a congenital teratoid tumor that protrudes through the mouth. It is rare and often fatal. It originates from the Rathke pouch (pharynx and or bones of the base of the skull) and hard palate. It can occupy the entire oropharyngeal cavity and externalize through the mouth. Depending on the size, Epignathus can cause asphyxia and severe obstructive respiratory insufficiency in the neonate, usually fatal. Prenatal diagnosis can be performed by prenatal ultrasonography and magnetic resonance imaging. With this, it is possible to plan the delivery in the best possible way, with a multidisciplinary team to provide specialized care and allow the beginning of the treatment of the neonate still in the delivery room with a view to the survival

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