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Congenital absence of the trachea

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Congenital absence of the trachea is a rare cause of severe neonatal respiratory distress. Experimental studies show that it is probably caused by disorders in a system of folds in the tracheo-oesophageal space rather than abnormalities of a tracheo-oesophageal septum. A literature review disclosed 82 cases of tracheal agenesis, which showed male predominance and an association with prematurity and polyhydramnios. In 90% of cases associated congenital malformations were present, most frequently affecting the cardiovascular or gastro-intestinal systems and the genito-urinary tract. Clinical signs of tracheal agenesis or atresia are respiratory insufficiency, often with severe respiratory distress, absence of audible crying and difficult or impossible endotracheal intubation. At present, curative repair and survival are impossible unless there is enough proximal or distal trachea to create a tracheostoma. Important ongoing research is aimed at tissue-engineered cartilage for surgical repair of tracheal defects.

Conclusion Tracheal agenesis, although very rare, can be recognised from a characteristic clinical pattern. Mostly associated congenital malformations are present. Research into tissue-engineering might lead to possibilities for definitive surgical repair of tracheal agenesis or atresia; however, until curative repair becomes possible, prolonged ventilation via the oesophageal tube does not seem to be worthwhile.

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Received: 25 June 1998 / Accepted: 19 February 1999

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van Veenendaal, M., Liem, K. & Marres, H. Congenital absence of the trachea. Eur J Pediatr 159, 8–13 (2000).

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