Medicine and the Law

Fetal intervention (EXIT procedure) for a rare fetal abnormality in a public sector hospital in South Africa – exploring what is possible

Authors

  • C J M Stewart Department of Obstetrics and Gynaecology, University of Cape Town, and Groote Schuur Hospital, Cape Town, South Africa
  • S Peer Division of Otolaryngology, University of Cape Town and Red Cross War Memorial Children’s Hospital, Cape Town, South Africa
  • A Numanoglu Division of Paediatric Surgery, University of Cape Town and Red Cross War Memorial Children’s Hospital, Cape Town, South Africa
  • K Fieggen Division of Human Genetics, Department of Medicine, University of Cape Town and Groote Schuur Hospital, Cape Town, South Africa
  • D van Dyk Department of Anaesthetics, University of Cape Town and Groote Schuur Hospital, Cape Town, South Africa
  • K Bester Division of Paediatric Anaesthesia, Department of Anaesthesia and Perioperative Medicine, University of Cape Town and Red Cross War Memorial Children’s Hospital, Cape Town, South Africa

DOI:

https://doi.org/10.7196/SAMJ.2022.v112i10.16471

Keywords:

Fetal intervention, EXIT procedure

Abstract

Congenital high airway obstruction syndrome (CHAOS) is a rare condition that can be diagnosed antenatally by ultrasound. It is usually lethal without immediate intervention at delivery. A 24-year-old woman was diagnosed with fetal CHAOS at 27 weeks’ gestation. The couple declined termination of pregnancy. A multidisciplinary team including obstetricians, geneticists, paediatric surgeons, neonatologists and anaesthetists was constituted to plan an ex utero intrapartum treatment (EXIT) procedure. After several simulations, a caesarean section was performed at 38 weeks’ gestation under deep inhalational anaesthesia. The fetus was fully delivered with placenta remaining in utero to maintain perfusion. A surgical airway was established via tracheostomy in approximately 5 minutes. The operation was then completed with no maternal complications. The child remains well at 3 years of age. To our knowledge, this is the first EXIT procedure performed for CHAOS in the public sector. This procedure can be lifesaving and is possible with proper planning.

References

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(2):271- 274. https://doi.org/10.1016/0022-3468(94)90331-x

Saadai P, Jelin EB, Nijagal A, et al. Long-term outcomes after fetal therapy for congenital high airway obstructive syndromse. J Pediatr Surg 2012;47(6):1095-1100. https://doi.org/10.1016/j. jpedsurg.2012.03.015

Nicolas CT, Lynch-Salamon ED, Bendel-Stenzal E, et al. Fetoscopically-assisted percutaneous decompression of the distal trachea and lung reverses fetal hydrops and fetal distress in a fetus with laryngeal atresia. Fetal Diagn Ther 2019;46(1):75-80. https://doi.org/10.1159/000500455

Catalano PJ, Urken ML, Alvarez M, et al. New approach to the management of airway obstruction in ‘high-risk’ neonates. Arch Otolaryngol Head Neck Surg 1992;108(3):306-309. https://doi.org/10.1001/ archotol.1992.01880030094019

Bouchard S, Johnson HP, Flake AW, Howell LJ, Myers LB, Adzick NS. The EXIT procedure: Experience and outcome in 31 cases. J Pediatr Surg 2002;37(3):418-426. https://doi.org/10.1053/jpsu.2002.30839

HiroseS,HarrisonMR.Theexuterointrapartum(EXIT)procedure.SeminNeonatol2003;8(3):207-214.

Shimabukuro F, Sakumoto K, Masamoto H, et al. A case of congenital high airway obstruction syndrome managed by ex utero intrapartum treatment: Case report and review of the literature. Am J Perinatol 2007;24(3):197-201. https://doi.org/10.1055/s-2007-972928

Crombleholme TM, Sylvester K, Flake AW, Adzick NS. Salvage of a fetus with congenital high airway obstruction syndrome by ex utero intrapartum (EXIT) procedure. Fetal Diagn Ther 2000;15(5):280- 282. https://doi.org/10.1159/000021022

Barthod G, Teissier T, Bellarbi N, et al. Fetal airway management on placental support: Limitations and ethical considerations in seven cases. J Obstet Gynaecol 2013;33:787-794. https://doi.org/10.31 09/01443615.2013.823924

Paek BW, Callen PW, Kitterman J, et al. Successful intervention for congenital high airway obstructions syndrome. Fetal Diagn Ther 2002;17(5):272-276. https://doi.org/10.1159/000063179

Yaneza MM, Cameron A, Clement WA, et al. An interventional airway delivery service for congenital high airway obstruction. J Laryngol Otol 2015:129(8):795-800. https://doi.org/10.1017/s0022215115000900

Elliott R, Vallera C, Heitmiller ES, et al. Ex utero intrapartum treatment procedure for management of high airway obstruction syndrome in a vertex/ breech twin gestation. Int J Pediatr Otorhinolaryngol 2012;77:439-442. https://doi.org/10.1016/j.ijporl.2012.11.023

Botto HA, Boailchuk ID, Garcia C, Decaro M, Aiello H, Copich J. Ex utero intrapartum treatment- management of neonatal congenital high airway obstruction syndrome: A case report. Arch Argent Pediatr 2010;108(4):e92-95. https://doi.org/10.1590/s0325-00752010000400013

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. Neonat Fetal Cond 2019;54(6):1153-1158. https://doi.org/10.1016/j.jpedsurg.2019.02.034

Downloads

Published

2022-10-05

Issue

Section

In Practice

How to Cite

1.
Stewart CJM, Peer S, Numanoglu A, Fieggen K, van Dyk D, Bester K. Medicine and the Law: Fetal intervention (EXIT procedure) for a rare fetal abnormality in a public sector hospital in South Africa – exploring what is possible. S Afr Med J [Internet]. 2022 Oct. 5 [cited 2025 Oct. 12];112(10):791-4. Available from: https://samajournals.co.za/index.php/samj/article/view/257

Most read articles by the same author(s)