Prenatally Diagnosed Congenital High Airway Obstruction Syndrome: Perinatal Management and Outcome—A Single Tertiary Care Center Experience

Abstract

To report our experience with the management of prenatally diagnosed cases of congenital high airway obstruction syndrome (CHAOS) and the postnatal outcome of those who underwent an ex utero intrapartum treatment (EXIT) procedure. This is a single center, retrospective observational study of prenatally diagnosed CHAOS cases using two-dimensional ultrasound from December 2017 to December 2022 in a tertiary care facility. Of the total nine fetuses prenatally diagnosed with CHAOS, three (33.3%) were associated with multiple congenital anomalies, seven out of nine (77.8%) developed ascites, and one had fetal hydrops. Five (55.6%) underwent medical termination of pregnancy and two were lost to follow-up (22.2%). The remaining two continued pregnancy and required EXIT tracheostomy at the time of delivery (22.2%). Microarray was performed in both which was normal. Postnatally, both infants are tracheostomy dependent with one requiring frequent ventilator support. CHAOS even when isolated generally has poor prognosis without intervention. Performing an EXIT procedure at birth can significantly improve postnatal survival by minimizing hypoxic damage. However, the long-term medical and surgical challenges for survivors remain numerous especially speech disorders, even after lifesaving fetal intervention and surgical correction. Therefore, an accurate prenatal diagnosis is necessary to give the couple an option of continuing pregnancy after realistic counseling regarding the prognosis and postnatal outcome.

Keywords

CHAOS - prenatal diagnosis - EXIT

Consent to Participate and Consent to Publish

Written informed consent was obtained from the patients for participation and publication of this study.

Ethical Approval

This retrospective study was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards. The institutional ethics committee approved this study.

Authors' Contribution

All authors contributed to the study's conception and design. All authors have read and approved the final manuscript.

Publication History

Article published online:
24 May 2024

• References

• 1 Hedrick MH, Ferro MM, Filly RA, Flake AW, Harrison MR, Adzick NS. Congenital high airway obstruction syndrome (CHAOS): a potential for perinatal intervention. J Pediatr Surg 1994; 29 (02) 271-274
• 2 Hartnick CJ, Rutter M, Lang F, Willging JP, Cotton RT. Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm. Arch Otolaryngol Head Neck Surg 2002; 128 (05) 567-570
• 3 Courtier J, Poder L, Wang ZJ, Westphalen AC, Yeh BM, Coakley FV. Fetal tracheolaryngeal airway obstruction: prenatal evaluation by sonography and MRI. Pediatr Radiol 2010; 40 (11) 1800-1805
• 4 Yedururi S, Guillerman RP, Chung T. et al. Multimodality imaging of tracheobronchial disorders in children. Radiographics 2008; 28 (03) e29
• 5 Roybal JL, Liechty KW, Hedrick HL. et al. Predicting the severity of congenital high airway obstruction syndrome. J Pediatr Surg 2010; 45 (08) 1633-1639
• 6 Saadai P, Jelin EB, Nijagal A. et al. Long-term outcomes after fetal therapy for congenital high airway obstructive syndrome. J Pediatr Surg 2012; 47 (06) 1095-1100
• 7 Joshi P, Satija L, George R, Chatterjee S, D'Souza J, Raheem A. Congenital high airway obstruction syndrome-antenatal diagnosis of a rare case of airway obstruction using multimodality imaging. Med J Armed Forces India 2012; 68 (01) 78-80
• 8 Kuwashima S, Kitajima K, Kaji Y, Watanabe H, Watabe Y, Suzumura H. MR imaging appearance of laryngeal atresia (congenital high airway obstruction syndrome): unique course in a fetus. Pediatr Radiol 2008; 38 (03) 344-347
• 9 Garg MK. Case report: Antenatal diagnosis of congenital high airway obstruction syndrome - laryngeal atresia. Indian J Radiol Imaging 2008; 18 (04) 350-351
• 10 Coakley FV, Hricak H, Filly RA, Barkovich AJ, Harrison MR. Complex fetal disorders: effect of MR imaging on management–preliminary clinical experience. Radiology 1999; 213 (03) 691-696
• 11 Guimaraes CV, Linam LE, Kline-Fath BM. et al. Prenatal MRI findings of fetuses with congenital high airway obstruction sequence. Korean J Radiol 2009; 10 (02) 129-134
• 12 Mong A, Johnson AM, Kramer SS. et al. Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome. Pediatr Radiol 2008; 38 (11) 1171-1179
• 13 Lim FY, Crombleholme TM, Hedrick HL. et al. Congenital high airway obstruction syndrome: natural history and management. J Pediatr Surg 2003; 38 (06) 940-945
• 14 Nolan HR, Gurria J, Peiro JL. et al. Congenital high airway obstruction syndrome (CHAOS): Natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center. J Pediatr Surg 2019; 54 (06) 1153-1158
• 15 Kohl T, Hering R, Bauriedel G. et al. Fetoscopic and ultrasound-guided decompression of the fetal trachea in a human fetus with Fraser syndrome and congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia. Ultrasound Obstet Gynecol 2006; 27 (01) 84-88 , discussion 88
• 16 Kohl T, Van de Vondel P, Stressig R. et al. Percutaneous fetoscopic laser decompression of congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia via a single trocar–current technical constraints and potential solutions for future interventions. Fetal Diagn Ther 2009; 25 (01) 67-71
• 17 Martínez JM, Castañón M, Gómez O. et al. Evaluation of fetal vocal cords to select candidates for successful fetoscopic treatment of congenital high airway obstruction syndrome: preliminary case series. Fetal Diagn Ther 2013; 34 (02) 77-84
• 18 Peiro JL, Nolan HR, Alhajjat A. et al. A technical look at fetoscopic laser ablation for fetal laryngeal surgical recanalization in congenital high airway obstruction syndrome. J Laparoendosc Adv Surg Tech A 2020; 30 (06) 695-700
• 19 Nicolas CT, Lynch-Salamon D, Bendel-Stenzel E. et al. Fetoscopy-assisted percutaneous decompression of the distal trachea and lungs reverses hydrops fetalis and fetal distress in a fetus with laryngeal atresia. Fetal Diagn Ther 2019; 46 (01) 75-80
• 20 Jeong SH, Lee MY, Kang OJ. et al. Perinatal outcome of fetuses with congenital high airway obstruction syndrome: a single-center experience. Obstet Gynecol Sci 2021; 64 (01) 52-61
• 21 Masahata K, Soh H, Tachibana K. et al. Clinical outcomes of ex utero intrapartum treatment for fetal airway obstruction. Pediatr Surg Int 2019; 35 (08) 835-843