Fetal epignathus: the case of an early EXIT (ex utero intrapartum treatment).

BACKGROUND: Fetal epignathus, a teratoma arising from the oropharynx that may be lethal, can be diagnosed prenatally. CASE: A 29-year-old woman, gravida 1, was evaluated for an elevated alpha-fetoprotein level. Imaging evaluation revealed a fetal epignathus without intracranial extension. Preterm labor necessitated delivery at 27 5/7 weeks of gestation with ex utero intrapartum treatment (EXIT) procedure using a classical incision. The neonate's small size and short umbilical cord required complete exteriorization to secure the airway. Pathology revealed an immature teratoma. CONCLUSION: Prenatal diagnosis of fetal epignathus is imperative so that all options can be discussed. An EXIT procedure may be necessary for airway management at birth. If preterm delivery is necessary, choice of uterine incision and fetal size are important factors to consider for a successful outcome.