Obstet Gynecol Sci.  2021 Jan;64(1):52-61. 10.5468/ogs.20266.

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

• Jeong SH
• Lee MY
• Kang OJ
• Kim R
• Chung JH
• Won HS
• Lee PR
• Jung E
• Lee BS
• Choi WJ
• Lee YS

• KMID: 2511565
• DOI: http://doi.org/10.5468/ogs.20266

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.

Keyword

Airway obstruction; Laryngeal disease; Prenatal diagnosis; Tracheostomy

Figure

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  Fig. 1 Prenatal ultrasonography showing symmetrically enlarged, hyperechoic, and homogeneous lungs with centrally positioned heart (A), flatted diaphragm (solid arrows in B), and associated ascites (open arrow in B).

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  Fig. 2 Magnetic resonance imaging showing an abrupt narrowing of T2 high-signal airway at the level of larynx (arrows in A and B) and symmetrically enlarged both lungs (B).

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  Fig. 3 Flow diagram of fetuses diagnosed with congenital high airway obstruction syndrome (CHAOS). EXIT, ex utero intrapartum treatment.

Reference

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