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 language | English (US) |
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Pages (from-to) | 1095-1100 |
Number of pages | 6 |
Journal | Journal of pediatric surgery |
Volume | 47 |
Issue number | 6 |
DOIs | |
State | Published - Jun 2012 |
Externally published | Yes |
Keywords
- CHAOS
- EXIT
- Ex utero intrapartum treatment
- Fetal surgery
- Hydrops fetalis
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Surgery