Congenital laryngeal atresia, associated with congenital high airway obstruction syndrome (CHAOS) in neonates, presents as a rare and life-threatening anomaly requiring prompt surgical airway intervention. This case report elaborates the clinical
course of a preterm neonate born at 34+6 weeks with unremarkable prenatal ultrasound findings, encountered postnatal emergence of severe respiratory distress led to unsuccessful intubation attempts. Postmortem examination unveiled an atretic laryngeal inlet devoid of tracheal connection, and the presence of a pharyngoglottic duct extending from the pharynx to the trachea. Management include intervention with fetoscopy before birth or ex utero intrapartum treatment (EXIT) with tracheostomy if CHAOS suspected antenatally. If CHAOS impressed after delivery, immediate positive-pressure ventilation,intubation through a pharyngoglottic duct, and tracheostomy are advocated for advanced airway cases. Implementing this standardized protocol to establish airway is essential to effectively address diverse manifestations of congenital laryngeal atresia in the future. (J Taiwan Otolaryngol Head Neck Surg 2024; 59:23-27)
