Congenital High Airway Obstruction Syndrome (CHAOS): No Intervention, No Survival—A Case Report and Literature Review

Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening fetal anomaly which occurs as a result of either partial or complete obstruction of the upper fetal airway. Prenatal recognition is important for multidisciplinary team management. We present a case with CHAOS diagnosed antenatal imaging and the baby was delivered via ex utero intrapartum treatment (EXIT) procedure. The airway managed to be secured via tracheostomy while maintaining the fetus on feto-placental circulation. However, oxygen saturation started to decline despite successfully securing the upper airway. Unfortunately, we lost the baby despite active resuscitation possibly due to insufficient lung function secondary to chronic obstruction prenatally.

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“…Mostly, the cases are sporadic and the true incidence is unknown [2]. CHAOS has been documented in over 100 cases in the past [3].…”

Section: Discussionmentioning

confidence: 99%

Congenital High Airway Obstruction Syndrome (CHAOS): Prenatal Diagnosis and Perinatal Intervention – A Case Report

Baki

Draman

Ismail

et al. 2022

MJPCH

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“…While the true incidence of CHA-OS is unknown, the etiology of CHAOS includes a spectrum of anomalies thought to be secondary to failure of recanalization of the airway early in gestation, including tracheal atresia or stenosis, laryngeal atresia or stenosis, laryngeal webs, and laryngeal cysts [5]. In utero treatment to relieve the obstruction through a variety of fetoscopic and percutaneous techniques have been described in the literature in the presence of fetal hydrops to improve chances of survival for over 10 years [1][2][3]6]. The general consensus for management of prenatally diagnosed CHAOS has uniformly included ex utero intrapartum treatment (EXIT) to fetal airway regardless of fetoscopic or percutaneous treatment of obstructions involving cartilaginous structures with the ability to avoid this more invasive delivery modality described only in the case of successful decompression of a lesion limited to membranous web above normal laryngeal structures [7].…”

Section: Introductionmentioning

confidence: 99%

Fetoscopic Balloon Dilation and Stent Placement of Congenital High Airway Obstruction Syndrome Leading to Successful Cesarean Delivery

et al. 2022

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Introduction: Without fetal or perinatal intervention, congenital high airway obstruction syndrome (CHAOS) is a fatal anomaly. The ex utero intrapartum treatment (EXIT) procedure has been used to secure the fetal airway and minimize neonatal hypoxia, but is associated with increased maternal morbidity. Case Presentation: A 16-year-old woman (gravida 1, para 0) was referred to our hospital at 31 weeks gestation with fetal anomalies, including echogenic lungs, tracheobronchial dilation and flattened diaphragms. At 32 weeks, fetoscopic evaluation identified laryngeal stenosis, which was subsequently treated with balloon dilation and stent placement. The patient developed symptomatic and regular preterm contractions at post-operative day 7 with persistent sonographic signs of CHAOS, which prompted a repeat fetoscopy with confirmation of a patent fetal airway followed by Cesarean delivery under neuraxial anesthesia. Attempts to intubate through the tracheal stent were limited and resulted in removal of the stent. A neonatal airway was successfully established with rigid bronchoscopy. Direct laryngoscopy and bronchoscopy confirmed laryngeal stenosis with a small tracheoesophageal fistula immediately inferior to the laryngeal stenosis and significant tracheomalacia. A tracheostomy was then immediately performed for anticipated long term airway and pulmonary management. The procedures were well tolerated by both mom and baby. The baby demonstrated spontaneous healing of the tracheoesophageal fistula by day of life 7 with discharge home with ventilator support at three months of life. Conclusion: Use of repeated fetoscopy in order to relieve fetal upper airway obstruction offers the potential to minimize neonatal hypoxia, while concurrently decreasing maternal morbidity by avoiding an EXIT procedure. Use of the tracheal stent in CHAOS requires further investigation. The long-term reconstruction and respiratory support of children with CHAOS remain challenging

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“…though difficult, in order to plan for ex-utero intrapartum treatment (EXIT) [2][3][4][5]. Laryngeal atresia (LA) and tracheal agenesis (TA) are two of the rare causes of intrinsic airway obstruction in CHAOS [4,5]. TA is a rare congenital airway malformation, characterized by complete or partial absence of the trachea, frequently associated with carino-esophageal fistula, broncho-esophageal fistula, or tracheo-esophageal fistula (TEF).…”

Section: Introductionmentioning

confidence: 99%

“…CHAOS often results in stillbirths, while severe respiratory distress, hypoxia, absence of audible cry, and failure to intubate the airway are typical clinical features in newborn survivors, immediately after delivery. Therefore, prenatal diagnosis is desirable, even though difficult, in order to plan for ex-utero intrapartum treatment (EXIT) [ 2 , 3 , 4 , 5 ]. Laryngeal atresia (LA) and tracheal agenesis (TA) are two of the rare causes of intrinsic airway obstruction in CHAOS [ 4 , 5 ].…”

Section: Introductionmentioning

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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Congenital High Airway Obstruction Syndrome (CHAOS): No Intervention, No Survival—A Case Report and Literature Review

Cited by 7 publications

References 17 publications

Congenital High Airway Obstruction Syndrome (CHAOS): Prenatal Diagnosis and Perinatal Intervention – A Case Report

Congenital High Airway Obstruction Syndrome (CHAOS): Prenatal Diagnosis and Perinatal Intervention – A Case Report

Fetoscopic Balloon Dilation and Stent Placement of Congenital High Airway Obstruction Syndrome Leading to Successful Cesarean Delivery

Neonatal Respiratory Distress and Airway Emergency: Report of Two Cases

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