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


CONCLUSIONS
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.
Division 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
Payam
P
Saadai
Eric B
EB
Jelin
Amar
A
Nijagal
Samuel C
SC
Schecter
Shinjiro
S
Hirose
Tippi C
TC
MacKenzie
Larry
L
Rand
Ruth
R
Goldstein
Jody
J
Farrell
Michael
M
Harrison
Hanmin
H
Lee
D000015:Abnormalities, Multiple; D000025:Abortion, Eugenic; D000328:Adult; D000402:Airway Obstruction / Q000151:congenital / Q000196:embryology* / Q000601:surgery*; D001999:Bronchoscopy; D005260:Female; D005313:Fetal Death / Q000209:etiology; D046128:Fetal Therapies / Q000379:methods / Q000706:statistics & numerical data*; D005865:Gestational Age; D006801:Humans; D015160:Hydrops Fetalis; D007231:Infant, Newborn; D007830:Larynx / Q000002:abnormalities / Q000196:embryology; D008279:Magnetic Resonance Imaging; D008297:Male; D018579:Patient Selection; D011247:Pregnancy; D013577:Syndrome; D014132:Trachea / Q000002:abnormalities / Q000196:embryology / Q000601:surgery; D014139:Tracheostomy; D016896:Treatment Outcome; D055815:Young Adult