Congenital High Airway Obstruction Syndrome and Airway Reconstruction

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Abstract
CONGENITAL HIGH airway obstruction syndrome (CHAOS) was defined by Hedrick et al1 in 1994 as upper airway obstruction that is diagnosed in utero by ultrasound, with concomitant findings of large echogenic lungs, flattened or inverted diaphragms, dilated airways distal to the obstruction, and fetal ascites or hydrops. Of the 4 children with CHAOS who were described by Hedrick and colleagues, none survived past childbirth. Since 1994, there have been several reports of in utero diagnosis of CHAOS, with survival enabled by means of the EXIT (ex utero intrapartum treatment) procedure.2 The EXIT procedure was originally designed to treat children with large cervical masses (eg, hemangiomas and teratomas) that were diagnosed in utero and that produced airway obstruction.3,4 Of key importance to this type of procedure is the multidisciplinary involvement of the obstetrician, anesthesiologist, otolaryngologist, pediatric surgeon, and neonatologist.