Extreme Airway Difficulty During EXIT in Severe Fetal Agnathia: Failed Conventional Intubation and Successful Emergency Tracheostomy—A Case Report
Abstract
Background Severe mandibular hypoplasia, particularly agnathia, is a rare congenital condition that may cause critical neonatal airway obstruction. The Ex Utero Intrapartum Treatment (EXIT) procedure allows airway control while maintaining uteroplacental circulation; overall airway establishment success rates for EXIT exceed 90%, yet conventional endotracheal intubation fails in a meaningful subset of cases with extreme anatomic distortion, necessitating surgical airway access. In the most severe forms of agnathia, primary tracheostomy under placental support may represent the most appropriate airway strategy from the outset. Case Presentation A 36‐year‐old gravida 5 para 4 woman was referred after prenatal ultrasound detected severe fetal facial abnormalities. Serial imaging demonstrated progressive micrognathia evolving toward agnathia. Prenatal ultrasound demonstrated a markedly abnormal inferior facial angle (IFA) of 11° (normal mean 65° ± 16°) and a fetal nasomental angle of 92.87° (normal approximately 147°), indicating profound mandibular hypoplasia. Fetal MRI performed at 35 weeks and 3 days confirmed these findings, demonstrating an IFA of 40° and a nasomental angle of 116°, both below normal thresholds. The pregnancy was complicated by severe polyhydramnios. Fetal magnetic resonance imaging confirmed near‐complete absence of the mandible with glossoptosis and severe narrowing of the oropharynx and hypopharynx, while the distal trachea and bronchi appeared normal.…
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