Mohammed H, West K, Bewick J, Wickstead M
ENT Department,Norfolk and Norwich University Hospital,Norwich,UK.
J Laryngol Otol. 2016 Mar;130(3):314-7. doi: 10.1017/S0022215115003515. Epub 2016 Jan 7.
This paper discusses three cases of tracheal agenesis that presented within a six-week period to the Norfolk and Norwich University Hospital. By reviewing the available literature on tracheal agenesis, the report aims to outline a protocol for future prenatal and postnatal management.
A case series and a literature review.
Three cases of tracheal agenesis presented in the classical manner, with respiratory distress and unsuccessful intubation following delivery. A literature review confirmed that prenatal diagnosis requires future innovation; survival is rare and is predominately reliant on intubation of the oesophagus when a patent tracheoesophageal fistula is present. In most cases, tracheal agenesis represents part of the 'VATER' association: vertebral defects, anal atresia, tracheoesophageal fistula with oesophageal atresia, and radial or renal dysplasia. Complex, multiple-stage surgical procedures have been described; however, no survival to adolescence is documented.
There is a call for improved prenatal diagnosis to allow both adequate counselling of parents and preparation for multi-specialty management at delivery. In addition, these cases highlight the ongoing need for improved congenital anomaly data within the UK, with currently only 49 per cent of England's births being registered.
本文讨论了在六周内就诊于诺福克和诺维奇大学医院的三例气管发育不全病例。通过回顾现有的关于气管发育不全的文献，本报告旨在概述未来产前和产后管理的方案。
病例系列研究和文献综述。
三例气管发育不全病例均以典型方式呈现，即出生后出现呼吸窘迫且插管失败。文献综述证实，产前诊断需要未来的创新；存活率很低，主要依赖于存在开放性气管食管瘘时对食管进行插管。在大多数情况下，气管发育不全是“VATER”综合征的一部分：椎体缺陷、肛门闭锁、伴有食管闭锁的气管食管瘘以及桡骨或肾脏发育异常。已经描述了复杂的多阶段手术程序；然而，尚无存活至青春期的记录。
需要改进产前诊断，以便为父母提供充分的咨询，并为分娩时的多专科管理做好准备。此外，这些病例凸显了英国持续需要改善先天性异常数据的情况，目前英格兰仅有49%的出生信息进行了登记。
