caa a22 4500 475786351 CHVBK 20180406123659.0 cr unu---uuuuu 170329e20000101xx s 000 0 eng 10.1007/s004310050002 doi (NATIONALLICENCE)springer-10.1007/s004310050002 Congenital absence of the trachea [Elektronische Daten] [M. B. van Veenendaal, K. D. Liem, H. A. M. Marres] 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. Springer-Verlag Berlin Heidelberg, 2000 Key words Tracheal agenesis nationallicence Neonates nationallicence Tracheo-oesophageal fistula nationallicence Malformation association nationallicence van Veenendaal M. B. Department of Paediatrics, University Hospital Nijmegen, Nijmegen, The Netherlands, NL aut Liem K. D. Department of Paediatrics, University Hospital Nijmegen, Nijmegen, The Netherlands, NL aut Marres H. A. M. University Hospital Nijmegen, Department of Otorhinolaryngology, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands, Tel.:+ 31-24-3614450, Fax: + 31 24 3540251, NL aut https://doi.org/10.1007/s004310050002 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s004310050002 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- van Veenendaal M. B. Department of Paediatrics, University Hospital Nijmegen, Nijmegen, The Netherlands, NL aut NATIONALLICENCE 700 1- Liem K. D. Department of Paediatrics, University Hospital Nijmegen, Nijmegen, The Netherlands, NL aut NATIONALLICENCE 700 1- Marres H. A. M. University Hospital Nijmegen, Department of Otorhinolaryngology, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands, Tel.:+ 31-24-3614450, Fax: + 31 24 3540251, NL aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer