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Congenital high airway obstruction syndrome (CHAOS): Natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center.
J Pediatr Surg 2019; 54(6):1153-1158JP

Abstract

PURPOSE

Congenital high airway obstruction syndrome (CHAOS) is a devastating fetal condition of complete airway discontinuity resulting in significant hydrops and extreme lung hyperplasia. It is universally fatal with survival reported only in the rare spontaneous fistulization or EXIT intervention (Ex Utero Intrapartum Treatment). Even in these cases, mortality remains high, and current investigations are targeting prenatal interventions. This report describes our experience with management and fetal interventions for CHAOS, including laser laryngotomy.

METHODS

We retrospectively reviewed all patients diagnosed with CHAOS at a single academic institution between 2006 and 2017.

RESULTS

Fifteen patients were identified. Eight had obstruction at the trachea and seven at the larynx. In the laryngeal obstructions, three expired shortly after birth, and one survived after spontaneous fistulization and subsequent EXIT to tracheostomy. The remaining three underwent in-utero treatment with laser laryngotomy. One had preterm premature rupture of membranes (PPROM), delivered 3 days post-operatively, and died. Two underwent EXIT to tracheostomy with one surviving to discharge and is currently 2 years old.

CONCLUSION

Our study demonstrates the outcomes of a large series of patients diagnosed with CHAOS. While mortality remains high, options for fetal intervention are being explored to allow alterations in the prenatal natural history and improve postnatal outcomes.

TYPE OF STUDY

Retrospective Treatment Study.

LEVEL OF EVIDENCE

Level IV.

Authors+Show Affiliations

Cincinnati Fetal Center, Cincinnati, OH; Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Electronic address: heather.nolan@cchmc.org.Cincinnati Children's Hospital Medical Center, Cincinnati, OH.Cincinnati Fetal Center, Cincinnati, OH; Cincinnati Children's Hospital Medical Center, Cincinnati, OH; University of Cincinnati College of Medicine, Cincinnati, OH.Cincinnati Fetal Center, Cincinnati, OH; Cincinnati Children's Hospital Medical Center, Cincinnati, OH; University of Cincinnati College of Medicine, Cincinnati, OH.Cincinnati Fetal Center, Cincinnati, OH; Cincinnati Children's Hospital Medical Center, Cincinnati, OH.Cincinnati Fetal Center, Cincinnati, OH; Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Good Samaritan Hospital, Cincinnati, OH.Cincinnati Fetal Center, Cincinnati, OH; Cincinnati Children's Hospital Medical Center, Cincinnati, OH; University of Cincinnati College of Medicine, Cincinnati, OH; Good Samaritan Hospital, Cincinnati, OH.Cincinnati Fetal Center, Cincinnati, OH; Cincinnati Children's Hospital Medical Center, Cincinnati, OH; University of Cincinnati College of Medicine, Cincinnati, OH.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

30890267

Citation

Nolan, Heather R., et al. "Congenital High Airway Obstruction Syndrome (CHAOS): Natural History, Prenatal Management Strategies, and Outcomes at a Single Comprehensive Fetal Center." Journal of Pediatric Surgery, vol. 54, no. 6, 2019, pp. 1153-1158.
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(6):1153-1158.
Nolan, H. R., Gurria, J., Peiro, J. L., Tabbah, S., Diaz-Primera, R., Polzin, W., ... Lim, F. Y. (2019). Congenital high airway obstruction syndrome (CHAOS): Natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center. Journal of Pediatric Surgery, 54(6), pp. 1153-1158. doi:10.1016/j.jpedsurg.2019.02.034.
Nolan HR, 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(6):1153-1158. PubMed PMID: 30890267.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Congenital high airway obstruction syndrome (CHAOS): Natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center. AU - Nolan,Heather R, AU - Gurria,Juan, AU - Peiro,Jose L, AU - Tabbah,Sammy, AU - Diaz-Primera,Ramiro, AU - Polzin,William, AU - Habli,Mounira, AU - Lim,Foong-Yen, Y1 - 2019/03/01/ PY - 2019/02/09/received PY - 2019/02/21/accepted PY - 2019/3/21/pubmed PY - 2019/8/27/medline PY - 2019/3/21/entrez KW - Congenital high airway obstruction syndrome (CHAOS) KW - Fetal interventions KW - Laser laryngotomy KW - Prenatal diagnosis SP - 1153 EP - 1158 JF - Journal of pediatric surgery JO - J. Pediatr. Surg. VL - 54 IS - 6 N2 - PURPOSE: Congenital high airway obstruction syndrome (CHAOS) is a devastating fetal condition of complete airway discontinuity resulting in significant hydrops and extreme lung hyperplasia. It is universally fatal with survival reported only in the rare spontaneous fistulization or EXIT intervention (Ex Utero Intrapartum Treatment). Even in these cases, mortality remains high, and current investigations are targeting prenatal interventions. This report describes our experience with management and fetal interventions for CHAOS, including laser laryngotomy. METHODS: We retrospectively reviewed all patients diagnosed with CHAOS at a single academic institution between 2006 and 2017. RESULTS: Fifteen patients were identified. Eight had obstruction at the trachea and seven at the larynx. In the laryngeal obstructions, three expired shortly after birth, and one survived after spontaneous fistulization and subsequent EXIT to tracheostomy. The remaining three underwent in-utero treatment with laser laryngotomy. One had preterm premature rupture of membranes (PPROM), delivered 3 days post-operatively, and died. Two underwent EXIT to tracheostomy with one surviving to discharge and is currently 2 years old. CONCLUSION: Our study demonstrates the outcomes of a large series of patients diagnosed with CHAOS. While mortality remains high, options for fetal intervention are being explored to allow alterations in the prenatal natural history and improve postnatal outcomes. TYPE OF STUDY: Retrospective Treatment Study. LEVEL OF EVIDENCE: Level IV. SN - 1531-5037 UR - https://www.unboundmedicine.com/medline/citation/30890267/Congenital_high_airway_obstruction_syndrome_(CHAOS):_Natural_history,_prenatal_management_strategies,_and_outcomes_at_a_single_comprehensive_fetal_center L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022-3468(19)30175-7 DB - PRIME DP - Unbound Medicine ER -