Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening fetal condition resulting from obstruction of the upper fetal airway which may be partial or complete. Prenatal diagnosis is crucial as it usually results in stillbirth or death after delivery if unrecognized. We report a case of CHAOS that was diagnosed prenatally due to characteristic ultrasound features. We also briefly review literature in light of current management options.

OBJECTIVE: To present antenatal management and use of ex utero intrapartum treatment (EXIT) in different fetal neck and high airway anomalies.
METHODS: We have presented four different cases of fetal neck or airway pathology which were indications for EXIT, at our department.
RESULTS: In three cases of fetal neck tumors, the primary precise antenatal diagnoses of tumors were confirmed after birth. The airways of all three fetuses were properly secured during EXIT by laryngologist. All these newborns survived. In the fourth case, a primary, antenatal diagnosis of congenital high airway obstruction syndrome due to severe trachea obstruction was not confirmed after birth. Finally, due to complete trachea dysgenesis, neither tracheoscopy nor tracheostomy was done during EXIT and the baby died.
CONCLUSIONS: Despite a failure of intrapartum treatment in the fourth case, we strongly recommend this procedure for deliveries of fetuses with a suspicion of airway obstruction.

OBJECTIVE: we aimed to report our experience about congenital high airway obstruction syndrome (CHAOS) that is a rare and fatal congenital anomaly; laryngeal atresia is the most frequent cause. Sonographic findings are enlarged echogenic lungs, dilated trachea, and ascites.
METHODS: we performed a single-center case series analysis collecting antenatally through ultrasound examination, and some of them confirmed by autopsy.
RESULTS: we report six cases of CHAOS diagnosed by antenatal ultrasonography between 2007 and 2013.
CONCLUSIONS: to date literature provides very few individual case reports. This work describes typical sonographic findings of this syndrome and it underlines the importance of early prenatal diagnosis to improve prognosis thought an ex utero intrapartum treatment, that seems to be the only chance of survival for the affected fetus.

Chromosome 22q11.2 deletion syndrome is common and presents with a range of clinical features from cardiac malformations to hypocalcemia. Laryngeal anomalies are not a common feature of this syndrome. We describe newly born twins who presented with unexpected severe birth depression secondary to severe type IV glottic webs requiring extensive resuscitation and emergency tracheostomy. They were diagnosed postnatally to have deletion of 22q11.2. The successful resuscitation of these infants at birth was only possible because they were born in a tertiary care hospital. This report shows the critical nature of prenatal diagnosis of 22q11.2 deletion syndrome.