Aim To raise awareness regarding undiagnosed tracheal atresia and associated high mortality in a preterm infant

Material and Method This is a unique case of an extremely preterm female infant who had an unexpected collapse post-extubation. She was born at 25+1 weeks gestation via normal vaginal delivery and was steroid mature. Her mum had sepsis and Preterm Prolonged Rupture of membranes. She got intubated at 15 minutes of life and received surfactant. On admission to the neonatal unit, she remained minimally ventilated for 12 hours and was noted suitable for extubation with good blood gases and adequate respiratory drive. Her CXR showed endotracheal tube at T2, good lung expansion, and NGT in stomach with abnormal vertebral bodies between T4-T6. Post extubation, she did not make a good respiratory effort and was noted desaturating. Several attempts at intubation were completed by various senior members. The ETT was seen to pass through the vocal cords but no chest wall movement was observed. An emergency airway team was called which included anaesthetics and ears, nose, and throat consultants.

Results Video laryngoscopy showed a large retrolarygeal posterior wall defect. Flexible scope and MLB identified a blind-ended trachea with a fistula into the oesophagus. An MDT discussion identified an un-survivable condition and a decision was made to re-orientate the care. The infant passed away peacefully in her mum’s arms. Her genetic report confirmed no abnormalities. Her post-mortem report confirmed chorioamnionitis with a possibility of VACTERL.

Conclusions Tracheal atresia as a part of Congenital High Airway Obstruction Syndrome(CHAOS) is a rare congenital anomaly that causes postnatal respiratory distress and makes intubation impossible or complicated. It is difficult to diagnose in the antenatal period, especially in extreme preterm gestations. It is almost always associated with high mortality within a few days of life.