Congenital high airway obstruction syndrome (CHAOS): Natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center

Nolan, Heather R.; Gurria, Juan P.; Peiró, José Luis; Tabbah, Sammy; Diaz-Primera, Ramiro; Polzin, William; Habli, Mounira; Lim, Foong‐Yen

doi:10.1016/j.jpedsurg.2019.02.034

Cited by 26 publications

(20 citation statements)

References 17 publications

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“…Several fetal interventions, including airway decompression via fetoscopy, have been attempted to improve the associated fetal hydrops [12,13]. These interventions can affect the fetus in a manner similar to a tracheoesophageal fistula.…”

Section: Discussionmentioning

confidence: 99%

Perinatal outcome of fetuses with congenital high airway obstruction syndrome: a single-center experience

et al. 2021

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Objective To report our experience with management of fetuses with congenital high airway obstruction syndrome (CHAOS). Methods We retrospectively reviewed the cases of fetuses who were prenatally diagnosed and postnatally confirmed with CHAOS between 2010 and 2019 at Asan Medical Center, Seoul, Korea. Results Of 13 fetuses prenatally diagnosed with CHAOS, 7 were lost to follow-up and 6 were postnatally confirmed as having CHAOS. All fetuses, except one were delivered via cesarean section with an ex utero intrapartum treatment (EXIT) procedure. Two patients had coexisting congenital heart diseases requiring several cardiac surgeries following birth. Both of these patients demonstrated developmental delay; however, the remaining 4 had a normal development except for expressive language. Two infants died of respiratory complications, and the remaining 4 were alive at the end of the follow-up period. All 4 live patients underwent tracheostomy with planned reconstruction surgery. Three children are now able to phonate, and 1 can maintain a conservation. Conclusion The proper management of CHAOS using the EXIT procedure results in high survival and low hypoxemia-induced complication rates. Therefore, an accurate prenatal diagnosis is necessary for an appropriate perinatal management.

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Section: Discussionmentioning

confidence: 99%

Perinatal outcome of fetuses with congenital high airway obstruction syndrome: a single-center experience

et al. 2021

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“…However, a TEF is frequently associated with TA and LA, making US diagnosis challenging as polyhydramnios is the single most common, though non-specific, prenatal finding [3,10,19,21]. Once other causes of polyhydramnios have been excluded and fetal airway obstruction is suspected, fetal MRI can confirm the diagnosis and locate the site of obstruction, and complete genetic evaluation is essential to rule out genetic anomalies and syndromes [3,4,19,22]. In case of prenatal diagnosis, pregnancy counselling is mandatory, especially in case of genetic anomalies or near complete TA which are incompatible with survival despite treatment [22].…”

Section: Discussionmentioning

confidence: 99%

“…Once other causes of polyhydramnios have been excluded and fetal airway obstruction is suspected, fetal MRI can confirm the diagnosis and locate the site of obstruction, and complete genetic evaluation is essential to rule out genetic anomalies and syndromes [3,4,19,22]. In case of prenatal diagnosis, pregnancy counselling is mandatory, especially in case of genetic anomalies or near complete TA which are incompatible with survival despite treatment [22]. When pregnancy is continued, and the cause of airway obstruction can be successfully surgically managed, fetal surgery can be attempted under specific circumstances.…”

Section: Discussionmentioning

confidence: 99%

“…When pregnancy is continued, and the cause of airway obstruction can be successfully surgically managed, fetal surgery can be attempted under specific circumstances. Otherwise, an EXIT can be planned to secure the airway to allow for further evaluation and delay surgical correction of the obstruction [4,22].…”

Section: Discussionmentioning

confidence: 99%

“…While LA seldom allows for fetal intervention and tracheostomy at birth, TA is almost universally fatal even in highly specialized centers, and survivors are exceptional [10,22]. In a systematic review, Smith et al reported a mortality rate of 87.2% within the first week of life and 92.6% at 1 year; 41.3% of patients did not survive beyond the first 24 h of life [10].…”

Section: Discussionmentioning

confidence: 99%

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Neonatal Respiratory Distress and Airway Emergency: Report of Two Cases

et al. 2021

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We discuss two cases of congenital airway malformations seen in our neonatal intensive care unit (NICU). The aim is to report extremely rare events characterized by immediate respiratory distress after delivery and the impossibility to ventilate and intubate the airway. The first case is a male twin born at 34 weeks by emergency caesarean section. Immediately after delivery, the newborn was cyanotic and showed severe respiratory distress. Bag-valve-mask ventilation did not relieve the respiratory distress but allowed for temporary oxygenation during subsequent unsuccessful oral-tracheal intubation (OTI) attempts. Flexible laryngoscopy revealed complete subglottic obstruction. Postmortem analysis revealed a poly-malformative syndrome, unilateral multicystic renal dysplasia with a complete subglottic diaphragm, and a tracheo-esophageal fistula (TEF). The second case is a male patient that was vaginally born at 35 weeks. Antenatally, an ultrasound (US) arose suspicion for a VACTERL association (vertebral defects, anal atresia, TEF with esophageal atresia and radial or renal dysplasia, plus cardiovascular and limb defects) and a TEF, and thus, fetal magnetic resonance (MRI) was scheduled. Spontaneous labor started shortly thereafter, before imaging could be performed. Respiratory distress, cyanosis, and absence of an audible cry was observed immediately at delivery. Attempts at OTI were unsuccessful, whereas bag-valve-mask ventilation and esophageal intubation allowed for sufficient oxygenation. An emergency tracheostomy was attempted, although no trachea could be found on cervical exploration. Postmortem analysis revealed tracheal agenesis (TA), renal dysplasia, anal atresia, and a single umbilical artery. Clinicians need to be aware of congenital airway malformations and subsequent difficulties upon endotracheal intubation and must plan for multidisciplinary management of the airway at delivery, including emergency esophageal intubation and tracheostomy.

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