An unusual variation of oesophageal atresia.

Congenital anomalies of the oesophagus were once thought to be relatively rare. Plass in 1919 reported one case and in an exhaustive review of the literature found 136 fully verified cases; he stated that there was some variation in the minor details of the anomaly, but the general picture was uniform -a blind upper pouch ending at a level between the lower border of the larynx and the tracheal bifurcation and a lower oesophageal segment opening into the trachea close to the bifurcation. Plass found, but did not discuss, 17 cases where there was an oesophageal atresia with upper and lower cul-de-sacs, but no fistula to the trachea; he also found one case with a diaphragmatic occlusion to the oesophagus. Since the work of Leven (1941) and Ladd (1944) on staged repairs and Haight and Towsley's (1943) first success with direct anastomosis there has been a great increase in the number of cases diagnosed and reported. The frequency of the variations of the anomaly remains largely unchanged; about 90% show a blind upper pouch, the distal oesophagus communicating with the trachea; about 8% have oesophageal atresia with no fistula and the remainder are the very unusual types-those with a fistula from the upper pouch to the trachea, with or without a fistula from the lower pouch, and those with a tracheo-oesophageal fistula but no atresia of the oesophagus. One further extremely rare variation has been reported by Goldenberg (1960) and Schwartz and Dale (1955); these were cases in which the deficiency in the oesophagus was in the nature of a web obstruction with an associated fistula passing downwards and backwards from the trachea to the oesophagus distal to the web. In Goldenberg's case there was a second tracheo-oesophageal fistula above the septum. Two further cases of this rare anomaly are reported.