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因喉闭锁导致的先天性高位气道梗阻综合征(CHAOS)成功实施产时宫外治疗(EXIT)手术。

Successful ex utero intrapartum treatment (EXIT) procedure for congenital high airway obstruction syndrome (CHAOS) owing to laryngeal atresia.

作者信息

DeCou J M, Jones D C, Jacobs H D, Touloukian R J

机构信息

Section of Pediatric Surgery, Yale University School of Medicine and Yale-New Haven Children's Hospital, CT 06520-8062, USA.

出版信息

J Pediatr Surg. 1998 Oct;33(10):1563-5. doi: 10.1016/s0022-3468(98)90500-0.

DOI:10.1016/s0022-3468(98)90500-0
PMID:9802816
Abstract

Congenital high airway obstruction syndrome (CHAOS) caused by laryngeal atresia was diagnosed by prenatal ultrasound in a male fetus at 18-weeks-gestation. Findings included enlarged lungs, inverted diaphragms, dilated trachea distal to the obstruction, and ascites. At 35 weeks' gestation, a planned ex utero intrapartum treatment (EXIT) procedure was performed, allowing bronchoscopic evaluation of the airway and placement of a tracheostomy. The postnatal course was marked by gradual recovery of diaphragmatic function with corresponding weaning from conventional ventilation to positive airway pressure. The sustained improvement in diaphragmatic and pulmonary function after the EXIT procedure for laryngeal atresia is one of the first reported for this entity and provides encouragement for future attempts.

摘要

一名妊娠18周的男性胎儿经产前超声诊断为先天性高气道梗阻综合征(CHAOS),由喉闭锁引起。检查结果包括肺增大、膈肌倒置、梗阻远端气管扩张和腹水。妊娠35周时,进行了计划性产时宫外治疗(EXIT)手术,以便对气道进行支气管镜评估并放置气管造口术。产后病程的特点是膈肌功能逐渐恢复,相应地从传统通气逐渐过渡到气道正压通气。EXIT手术后喉闭锁患者膈肌和肺功能的持续改善是该病例首次报道之一,为未来的尝试提供了鼓励。

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