Salvage of a fetus with congenital high airway obstruction syndrome by ex utero intrapartum treatment (EXIT) procedure

Salvage of a fetus with congenital high airway obstruction syndrome by ex utero intrapartum treatment (EXIT) procedure
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DOI:
10.1159/000021022
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发表时间:
2000-09-01
影响因子:
2.2
通讯作者:
Adzick, NS
Adzick, NS
中科院分区:
医学3区
文献类型:
--
作者:
Crombleholme, TM;Sylvester, K;Adzick, NS

文献摘要

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一例胎儿因完全性气管闭锁而患有先天性高气道阻塞综合征(CHAOS),在妊娠31周,胎儿因大量气管积液12周后被转诊。胎儿在子宫外分娩时,在胎盘支持下有足够的时间进行支气管镜检查以确认气管闭锁并气管切开术以确保气道安全。他的出生过程是复杂的严重毛细血管渗漏综合征继发于积液,膈麻痹,气管支气管软化,并需要慢性通气支持。婴儿的气管支气管软化症在5个月大时消退,9个月时膈功能恢复正常,允许他脱离机械通气。他在17个月大时进行了气管重建。在32个月大的随访中,他的气道通畅,是第一位患有CHAOS的长期幸存者。版权所有(C) 2000 S. Karger AG,巴塞尔。
A fetus with congenital high airway obstruction syndrome (CHAOS) due to complete tracheal atresia was referred at 31 weeks of gestation after 12 weeks of massive hydrops, The fetus was delivered by the ex utero Intrapartum treatment procedure allowing sufficient time while on placental support for bronchoscopy to confirm tracheal atresia and tracheostomy to secure the airway. His postnatal course was complicated by severe capillary leak syndrome secondary to hydrops, diaphragmatic paralysis, tracheobronchial malacia, and the need for chronic ventilatory support. The infant's tracheobronchial malacia resolved by 5 months of age and normal diaphragmatic function was restored at 9 months allowing him to be weaned from mechanical ventilation. He underwent tracheal reconstruction at 17 months of age. At follow up at 32 months of age he has a patent airway and is the first long-term survivor with CHAOS. Copyright (C) 2000 S. Karger AG, Basel.