Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management
Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management
复制标题
完全气管发育不全导致的先天性高气道阻塞综合征：一次自然事故，提供气管发育的线索和管理经验教训
DOI：
10.1159/000242454
复制
发表时间：
2009
影响因子：
2.2
通讯作者：
T. Crombleholme
中科院分区：
文献类型：
--
作者：
S. Vaikunth;Lee M. Morris;W. Polzin;W. Gottliebson;F. Lim;Beth Kline;T. Crombleholme
先天性高位呼吸道阻塞综合征(CHOS)是一种危及生命的疾病，其特征是胎儿呼吸道完全阻塞并伴有积水。我们提出一例由于罕见的完全性气管发育不全引起的混乱。采用宫外产内治疗(EXIT)策略，允许颈部和纵隔探查。胸腺切除可以解剖隆突水平，显示扩张的主干支气管汇合，但没有气管，也没有与食道的连接。将2.5支气管插管插入右侧主干支气管处，并固定于左侧锁骨。在3个月大时，由于气管造口管和鼻胃管之间的摩擦导致食道侵蚀，婴儿死于肠杆菌纵隔炎引起的脓毒症。完全性气管发育不全，如本例所见，与前肠发育的新理论所提示的正常气管伸长失败相一致。这个病例说明了最严重的气管闭锁形式，在出生时通过排气程序挽救了混乱。随后的产后课程强调了在这种特别具有挑战性的混乱形式中早期气管置换的必要性。
Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition characterized by complete blockage of the fetal airways associated with hydrops. We present a case of CHAOS due to the rare cause of complete tracheal agenesis. The ex utero intrapartum therapy (EXIT) strategy was employed to allow for neck and mediastinal exploration. Thymectomy allowed dissection to the level of the carina demonstrating the confluence of dilated mainstem bronchi but no trachea and no connection to the esophagus. A 2.5 endotracheal tube was inserted into the right mainstem bronchus and secured to the left clavicle. At 3 months of age, the infant succumbed to sepsis from Enterobacter mediastinitis due to friction between the tracheostomy tube and the nasogastric tube resulting in erosion of the esophagus. Complete tracheal agenesis, as seen in this case, is consistent with the failure of normal tracheal elongation as suggested by newer theories of foregut development. This case illustrates the most severe form of tracheal atresia causing CHAOS ever salvaged by the EXIT procedure at birth. The subsequent postnatal course highlights the need for early tracheal replacement in this particularly challenging form of CHAOS.