Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management

Abstract

Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition characterized by complete blockage of the fetal airways associated with hydrops. We present a case of CHAOS due to the rare cause of complete tracheal agenesis. The ex utero intrapartum therapy (EXIT) strategy was employed to allow for neck and mediastinal exploration. Thymectomy allowed dissection to the level of the carina demonstrating the confluence of dilated mainstem bronchi but no trachea and no connection to the esophagus. A 2.5 endotracheal tube was inserted into the right mainstem bronchus and secured to the left clavicle. At 3 months of age, the infant succumbed to sepsis from Enterobacter mediastinitis due to friction between the tracheostomy tube and the nasogastric tube resulting in erosion of the esophagus. Complete tracheal agenesis, as seen in this case, is consistent with the failure of normal tracheal elongation as suggested by newer theories of foregut development. This case illustrates the most severe form of tracheal atresia causing CHAOS ever salvaged by the EXIT procedure at birth. The subsequent postnatal course highlights the need for early tracheal replacement in this particularly challenging form of CHAOS.

EXIT procedure for fetal neck masses

“…The EXIT procedure has since been applied to other conditions in which advanced perinatal resuscitation is required. It is being used for controlled airway access in fetuses with large neck masses [2,3 && ], congenital high airway obstruction [4][5][6] or severe micrognathia (EXIT for airway) [7], as a bridge for fetuses with anticipated immediate need for extracorporeal support (EXIT-to-ECMO) [8,9], or for resection of large intrathoracic masses that may impair fetal resuscitation (EXIT-to-resection) [10,11 & ]. This article reviews indications, technique and outcomes following EXIT procedure for fetal neck masses.…”

Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature

“…Forty-nine cases of TA, including our case, were found [1, 2, 4–7, 9–11, 14, 17, 18, 20, 23, 24, 26, 29–33, 35–38, 40, 42]. The perinatal characteristics, diagnostic investigations, and management are summarized (Table 1) .…”

Tracheal agenesis in a new born: lessons learnt

“…When prenatal diagnosis is suspected, a planned EXIT procedure can be done for an airway control during the initial assessment 10. For immediate resuscitation, oesophageal intubation is a temporary solution.…”