Long-term outcomes after fetal therapy for congenital high airway obstructive syndrome

Payam Saadai, Eric B. Jelin, Amar Nijagal, Samuel C. Schecter, Shinjiro Hirose, Tippi C. MacKenzie, Larry Rand, Ruth Goldstein, Jody Farrell, Michael Harrison, Hanmin Lee

Research output: Contribution to journalArticlepeer-review

28 Scopus citations

Abstract

Background/Purpose: Congenital high airway obstructive syndrome (CHAOS) is a rare and devastating condition that is uniformly fatal without fetal intervention. We sought to describe fetal treatment and long-term outcomes of CHAOS at a single referral center. Methods: The medical records of patients with fetal CHAOS evaluated at our center between 1993 and 2011 were reviewed. Maternal history, radiographic findings, antenatal management, and postnatal outcomes were compared. Results: Twelve fetuses with CHAOS were identified. Eleven had concomitant hydrops at diagnosis. Six were electively terminated, and 2 had intra- or peripartum demise. Four patients underwent fetal intervention. Two underwent delivery via ex utero intrapartum treatment (EXIT) procedure with tracheostomy placement only, and 2 underwent fetal bronchoscopy with attempted wire tracheoplasty followed by EXIT with tracheostomy at delivery. All 4 patients who underwent EXIT were alive at last follow-up. One patient was ventilator and tracheostomy free and feeding by mouth. Conclusion: Long-term and tracheostomy-free survival is possible with appropriate fetal intervention even in the presence of hydrops. Fetal intervention earlier in pregnancy may improve long-term outcomes, but patient selection for intervention remains challenging. Magnetic resonance imaging may help select those patients for whom fetal intervention before EXIT delivery may be beneficial.

Original languageEnglish (US)
Pages (from-to)1095-1100
Number of pages6
JournalJournal of Pediatric Surgery
Volume47
Issue number6
DOIs
StatePublished - Jun 1 2012

Keywords

  • CHAOS
  • Ex utero intrapartum treatment
  • EXIT
  • Fetal surgery
  • Hydrops fetalis

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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