Congenital high airway obstruction syndrome is a rare malformation whose prognosis is very poor resulting in foetal or perinatal death if no perinatal intervention is performed. However, ultrasound and magnetic resonance enable an accurate prenatal diagnosis and optimal choice of interventional foetal medicine techniques (transtracheal puncture, fetoscopy). These approaches reduce foetal side effects related to the pathological process, and avoid the mother ex utero intrapartal treatment and its deleterious effects. If it becomes indispensable ex utero intrapartal treatment allows optimal management of the new born by securing the foetal airways while minimizing risk for hypoxic damage through the maintenance of maternal-foetal circulation. We present the story of a couple with a suspected case of CHAOS at 21 weeks of amenorrhea, the diagnostic and the management until the birth of the child.
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