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先天性喉黏液囊肿:CHAOS的罕见病因。

Congenital laryngomucocoele: a rare cause for CHAOS.

作者信息

Cunha Manuel Sousa, Janeiro Patrícia, Fernandes Rosário, Carreiro Helena, Laurini Ricardo

机构信息

Hospital Fernando Fonseca, Pediatrics, IC 19, Amadora, 2720-276, Portugal.

出版信息

BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.07.2008.0595. Epub 2009 Feb 27.

Abstract

Congenital high airway obstruction syndrome (CHAOS) is a rare but life-threatening condition that results from the obstruction of the upper airways.We describe a female newborn, from a Grávida II, Para 0, 36-year-old woman, with a routine ultrasound at 30 weeks' gestation that showed polyhydramnios. She delivered a live-born female baby at 36 weeks without any dismorphic features but with respiratory distress. Attempts at endotracheal intubation were unsuccessful due to the presence of a mass obstructing the larynx. The reanimation process was stopped after 20 minutes. Post-mortem examination demonstrated the presence of a total occlusion of the larynx by a laryngomucocoele. Laryngocele, a congenital cyst of the larynx, occurs rarely and hardly ever as a cause of CHAOS. What is more, laryngomucocoele has not been previously reported as a cause of CHAOS. These conditions represent a neonatal emergency with reserved prognosis unless diagnosed antenatally allowing for a programmed ex utero intrapartum treatment (EXIT) by performing tracheostomy while maintaining the placental circulation.

摘要

先天性高气道梗阻综合征(CHAOS)是一种罕见但危及生命的疾病,由上呼吸道梗阻引起。我们描述了一名女性新生儿,其母亲为36岁经产妇,孕2产0,在妊娠30周时常规超声检查显示羊水过多。她在36周时分娩出一名活产女婴,该女婴无任何畸形特征,但有呼吸窘迫症状。由于存在阻塞喉部的肿物,气管插管尝试未成功。复苏过程在20分钟后停止。尸检显示喉黏液囊肿导致喉部完全阻塞。喉囊肿是一种先天性喉部囊肿,很少发生,几乎从未作为CHAOS的病因。此外,此前尚未有喉黏液囊肿作为CHAOS病因的报道。这些情况代表了一种新生儿急症,预后不佳,除非产前诊断,以便通过在维持胎盘循环的同时进行气管切开术进行计划性产时宫外治疗(EXIT)。

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