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

Abstract

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.

Prenatal Diagnosis and Outcome of Tracheal Agenesis as Part of Congenital High Airway Obstruction Syndrome. Case Presentation and Literature Review

“…A notable case of laryngeal atresia was described by Witters et al, who reported a case associated with pulmonary atresia, thymus hypoplasia and Fallot tetralogy, constellation consistent with the diagnosis of DiGeorge defect. Other issues presented with an increased number of alveoli, an aspect suggestive for the diagnosis of polyalveolar lungs while some authors report a subglotic stenosis, due to the hyperplasia of the fibrous connective tissue from the submucosa [2,7,18]. As we can see in Table 1, CHAOS is a second-trimester diagnosis.…”

Mixed Heterotopic Gastrointestinal/Respiratory Oral Cysts in Newborns: From Prenatal Diagnosis to Histopathological and Therapeutic Management: A Case Report and Literature Review

“…A prenatal ultrasound/MRI evaluation can identify the possible malposition of the fetal head, with the limitation of the flexion, an enlarged diameter of the trachea, the anterior displacement of the heart, a flattened diaphragm, or enlarged lungs with altered echogenicity. These factors can establish a possible degree of airway obstruction before birth, defining congenital high airway obstruction syndrome (CHAOS) [52]. An alternative in the therapeutic management of these cases is either the intrapartum ex utero (EXIT) technique [53,54] with the intubation/tracheostomy of the newborn with the maintenance of fetal circulation or the OOPS procedure with a placental vascular approach [55].…”

The silent storm: a case report of missed antenatal diagnosis leading to fatal respiratory distress and death in a rare case of congenital high airway obstruction syndrome

“…Another study by Lee et al reported on 13 prenatally diagnosed CHAOS cases, with 7 lost to follow-up and 6 postnatally confirmed. 17 Except for one, all fetuses were delivered via caesarean section with an EXIT procedure.…”