A Case of Congenital High Airway Obstruction Syndrome Managed by Ex Utero Intrapartum Treatment: Case Report and Review of the Literature

ABSTRACT

Congenital high airway obstruction syndrome (CHAOS) has been reported to be fatal. Ten cases of CHAOS that underwent ex utero intrapartum treatment (EXIT) procedure to secure the fetal airway have been reported. A 36-year-old woman (gravida 3, para 2) was referred to our hospital at 22 weeks of gestation. Sonography revealed large echogenic lungs, flattened diaphragm, and marked hydrops. Magnetic resonance imaging confirmed the diagnosis of CHAOS. Polyhydramnios and fetal skin edema were improved and the fetal ascitic fluid was regressed gradually. At 36 weeks of gestation, an EXIT procedure was undertaken. Fetal laryngoscopy and bronchoscopy showed complete laryngeal obstruction, and a tracheostomy was performed immediately. The infant was discharged from hospital at 6 weeks of age. Thereafter, he developed well both physically and mentally. A laryngoplasty was performed at 20 months of age using silicon sheet as a patent airway. The child has a tracheostomy, is able to phonate but does not speak, and is awaiting decannulation. Use of the EXIT procedure in CHAOS cases offers the potential for salvage and excellent long-term outcome of these fetuses that otherwise would not survive. However, management of the airway, particularly with regard to long-term reconstruction in children with CHAOS, remains challenging.

CHAOS - EXIT - hydrops fetalis - laryngotracheoplasty

REFERENCES

• 1 Hedrick M H, Ferro M M, Filly R A, Flake A W, Harrison M R, Adzick N S. Congenital high airway obstruction syndrome (CHAOS): a potential for perinatal intervention.  J Pediatr Surg. 1994;  29 271-274
• 2 DeCou J M, Jones D C, Jacobs H D, Touloukian R J. Successful ex utero intrapartum treatment (EXIT) procedure for congenital high airway obstruction syndrome (CHAOS) owing to laryngeal atresia.  J Pediatr Surg. 1998;  33 1563-1565
• 3 Crombleholme T M, Sylvester K, Flake A W, Adzick N S. Salvage of a fetus with congenital high airway obstruction syndrome by ex utero intrapartum treatment (EXIT) procedure.  Fetal Diagn Ther. 2000;  15 280-282
• 4 Bui T H, Grunewald C, Frenckner B et al.. Successful EXIT (ex utero intrapartum treatment) procedure in a fetus diagnosed prenatally with congenital high-airway obstruction syndrome due to laryngeal atresia.  Eur J Pediatr Surg. 2000;  10 328-333
• 5 Paek B W, Callen P W, Kitterman J et al.. Successful fetal intervention for congenital high airway obstruction syndrome.  Fetal Diagn Ther. 2002;  17 272-276
• 6 Hirose S, Farmer D L, Lee H, Nobuhara K K, Harrison M R. The ex utero intrapartum treatment procedure: looking back at the EXIT.  J Pediatr Surg. 2004;  39 375-380
• 7 Lim F Y, Crombleholme T M, Hedrick H L et al.. Congenital high airway obstruction syndrome: natural history and management.  J Pediatr Surg. 2003;  38 940-945
• 8 Oepkes D, Teunissen A K, Van De Velde M, Devlieger H, Delaere P, Deprest J. Congenital high airway obstruction syndrome successfully managed with ex-utero intrapartum treatment.  Ultrasound Obstet Gynecol. 2003;  22 437-439
• 9 Kanamori Y, Kitano Y, Hashizume K et al.. A case of laryngeal atresia (congenital high airway obstruction syndrome) with chromosome 5p deletion syndrome rescued by ex utero intrapartum treatment.  J Pediatr Surg. 2004;  39 E25-E28
• 10 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 84-88
• 11 Hirose S, Harrison M R. The ex utero intrapartum treatment (EXIT) procedure.  Semin Neonatol. 2003;  8 207-214
• 12 Liechty K W, Crombleholme T M. Management of fetal airway obstruction.  Semin Perinatol. 1999;  23 496-506
• 13 Fraser G R. Our genetic “load”: a review of some aspects of genetic variation.  Ann Hum Genet. 1962;  25 387-415
• 14 Hartnick C J, Rutter M, Lang F, Willging J P, Cotton R T. Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm.  Arch Otolaryngol Head Neck Surg. 2002;  128 567-570