A rare form of congenital high airway obstruction syndrome and a literature review of ex utero intrapartum treatment

Abstract

Abstract Objective This is a rare presentation of a fetus with a right mainstem bronchus occlusion leading to congenital high airway obstruction syndrome (CHAOS). Methods A review of 15 articles reporting on the use of the ex utero intrapartum treatment (EXIT) procedure. Results A total of 22 reported cases of CHAOS had undergone the EXIT procedure from 1994 to 2016. Seventy-seven percent of fetuses that underwent EXIT were alive at the last point of follow-up, but only 36% were alive without any sequelae. Conclusion CHAOS is a rare malformation, but after the development of EXIT, survival is a viable option. It is associated with other malformations, but its etiology is entirely unknown.