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J Pediatr Surg. 2012 Jun;47(6):1095-100. doi: 10.1016/j.jpedsurg.2012.03.015.

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

Author information

  • 1Division of Pediatric Surgery and Fetal Treatment Center, Department of Surgery, University of California,Box 0570, San Francisco, CA 94143-0570, USA. payam.saadai@ucsfmedctr.org

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.

Copyright © 2012 Elsevier Inc. All rights reserved.

PMID:
22703776
[PubMed - indexed for MEDLINE]
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