Partial absence of the trachea with live birth.

Although anomalies of the trachea and oesophagus are not uncommon in paediatric practice, the case to be described is quite rare and no similar anomaly has been previously recorded at the Royal Hospital for Sick Children, Glasgow. Briefly, the condition is one of atresia of the larynx, partial absence of the trachea and a fistulous connexion between the oesophagus and the trachea. A point of considerable interest is that life was sustained for a short time and partial expansion of the lungs was achieved; but for the presence of much mucus in the lower respiratory passages life might have been prolonged further. A separate anomaly, namely extreme hypoplasia of one kidney, was also present. The tracheal anomaly has already been recorded in the German literature by Fritz (1933) and similar but not identical cases have been described by Beneke (1905), Walcher (1928) and Kessel and Smith (1953). That described by Fritz is essentially similar to our case and occurred in a 43-cm. male infant who survived for about 12 minutes but failed to cry. Necropsy showed that a portion of the trachea 3 cm. in length was entirely absent, that the larynx was fully formed but completely atretic distally and that a small fistula passed downwards connecting the oesophagus, from a point 3-2 cm. below the incisura interarytenoidea, with the upper extremity of the formed trachea. The lungs were poorly expanded. Fritz discusses briefly the anomalies of the trachea and oesophagus, comprising (a) simple oesophageal atresia, (b) oesophageal atresia with the distal portion in continuity with the carina, (c) tracheo-oesophageal fistula, (d) atresia of the larynx, (e) atresia of the larynx, partial absence of the trachea, the upper end of which is in continuity with the upper oesophagus while the lower part of the oesophagus takes origin at the carina, and (f) atresia of the larynx, partial absence of the trachea and oesophago-tracheal fistula. The present case and that described by Fritz belong to this last category. A similar but not identical case was described by Beneke (1905) in which the larynx was small and atretic, the trachea entirely absent, and the bifurcation of the major bronchi opened into the oesophagus. Walcher (1928) also describes a neonatal absence of the trachea in a female infant of body length 45 cm. and body weight 1,990 g. who survived for four days. Here a 2-5 cm. slit-like opening in the anterior wall …