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

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• 作者：Payam Saadai ^{payam.saadai@ucsfmedctr.org} ; Eric B. Jelin ; Amar Nijagal ; Samuel C. Schecter ; Shinjiro Hirose ; Tippi C. MacKenzie ; Larry Rand ; Ruth Goldstein ; Jody Farrell ; Michael Harrison ; Hanmin Lee ; ^{hanmin.lee@ucsfmedctr.org}
• 关键词：CHAOS ; EXIT ; Ex utero intrapartum treatment ; Fetal surgery ; Hydrops fetalis
• 刊名：Journal of Pediatric Surgery
• 出版年：2012
• 期刊代码：190_00223468
• 类别：med
• 出版时间：June, 2012
• 卷：47
• 期：6
• 页码：1095-1100
• 文件大小：285 K

摘要

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.