Heimann Konrad, Bartz Clemens, Naami Amjad, Peschgens Thomas, Merz Ulrich, Hörnchen Helmut
Department for Neonatal and Conservative Pediatric Intensive Care, University Hospital, Medical Faculty RWTH, 52074 Aachen, Germany.
Eur J Pediatr. 2007 Jan;166(1):79-82. doi: 10.1007/s00431-006-0210-4. Epub 2006 Aug 3.
Congenital absence of the trachea is a rare anomaly that might confront the obstetrician or neonatologist with an unexpected emergency. These patients present with cyanosis, severe respiratory distress, insufficient gas exchange, absence of audible crying and difficult or impossible endotracheal intubation. In more than 90% it is associated with further congenital malformations. Adequate oxygenation depends on the existence of a tracheo- or bronchooesphageal fistula and the length of the proximal trachea. We present the cases of three neonates with tracheal agenesis with tracheooesophageal fistula. Two of the neonates died within the first hour of life because endotracheal intubation was impossible and oxygenation through an oesophageally placed tube was insufficient. The third infant could be oxygenated through a tracheooesophageal fistula. The ventilation was at least insufficient and no surgical intervention was made. The diagnosis of a congenital absence of the trachea usually is made after birth because of the clinical signs and the course within the first minutes of life. The only way that the diagnosis can be made prenatally is by magnetic resonance imaging (MRI). The knowledge of this clinical picture helps to make decisions in an unexpected emergency in the immediate postpartum period and also in patients whose ventilation is very difficult right from the start.
先天性气管缺如是一种罕见的异常情况，可能会使产科医生或新生儿科医生面临意想不到的紧急情况。这些患者表现为紫绀、严重呼吸窘迫、气体交换不足、无哭声且气管插管困难或无法插管。超过90%的病例伴有其他先天性畸形。充分的氧合取决于是否存在气管或支气管食管瘘以及近端气管的长度。我们报告了三例患有气管食管瘘的先天性气管发育不全的新生儿病例。其中两名新生儿在出生后第一小时内死亡，因为无法进行气管插管，通过食管放置的导管进行氧合也不足。第三名婴儿可通过气管食管瘘进行氧合。通气至少不足，且未进行手术干预。先天性气管缺如的诊断通常在出生后根据临床症状和出生后几分钟内的病情发展做出。产前做出诊断的唯一方法是磁共振成像（MRI）。了解这种临床表现有助于在产后立即出现的意外紧急情况下以及从一开始通气就非常困难的患者中做出决策。
