Abstract
Agnathia-otocephaly complex has been regarded as a lethal condition, although less severe and non-lethal variations of the isolated type have been documented. Ensuring airway patency during the acute phase of this condition is paramount, and some cases with indications for Ex-Utero Intrapartum Treatment（EXIT）have been previously reported. In this report, a 37-year-old primiparous woman was referred to our hospital at 27 weeks of gestation and was diagnosed with an isolated type of agnathia-otocephaly with no other abnormalities. Magnetic resonance imaging and microbubble tests revealed delayed fetal lung maturation but not entirely immature. With the patient’s consent, an emergency cesarean section with EXIT was performed owing to clinical chorioamnionitis at 35 weeks of gestation; however, the neonate died 12 hours after delivery because of hypoplastic lungs. The methods for evaluating lung maturation are inconsistent; thus, the indication for invasive EXIT procedure warrants careful consideration, and the procedure necessitated robust teamwork. As our hospital only has few physicians experienced in performing EXIT, the present case marked our inaugural execution of the procedure. Consequently, we accomplished the prenatal diagnosis and execution of the EXIT procedure through coordinated medical care by multidisciplinary and interinstitutional teams.
