Esophageal atresia and tracheoesophageal fistula

The treatment of esophageal atresia and tracheoesophageal fistula, although still a challenge, represents one of the true successes of newborn surgery. Thomas Gibson first described the classic form of esophageal atresia with tracheoesophageal fistula in 1697 [1]. In 1888, Charles Steele attempted the first surgical repair of a pure esophageal atresia. He carried out a gastrotomy and attempted to pass a steel probe through the suspected esophageal membrane. At autopsy, the probe exited the lower esophageal pouch but the two ends of the esophagus were noted to end blindly with a gap between them of 1.5 inches with no connection that could be identified [2]. Attempted repair of esophageal atresia with a tracheoesophageal fistula employing fistula division and primary anastomosis was first reported by Robert Shaw in Dallas in 1938 [3] but first attempted by Thomas Lanman in Boston in 1936, who later reported it along with 4 other attempts and 27 additional cases of esophageal atresia in 1940 [4]. All of these attempts were unsuccessful, but not all because of technical issues. After 5 failed attempts, the first in 1939, Cameron Haight undertook the first successful primary repair of esophageal atresia in 1941 using a left extrapleural approach, fistula ligation, and a single-layer anastomosis [5]. Over the years, improvements in surgical technique and neonatal care have improved outcomes in the treatment of esophageal atresia and tracheoesophageal fistula. In 1994, Spitz et al. Created a system to stratify outcome based on birth weight (>1,500 g or <1,500 g) and the presence of a major cardiac anomaly [6]. In 2006, Spitz compared results in the present era to the 1994 results and noted that survival of babies with birth weight >1500 g and no cardiac anomalies was similar: 97% in 1994 versus 98.5% in 2006. Survival for babies that weighed <1,500 g at birth, or had a cardiac anomaly, improved from 59% to 82% during that interval. The babies with the worst outcomes, those that weighed <1,500 g at birth and had a cardiac anomaly, improved from 22% in the early era to 50% [7]. In 2009, Okamoto et al. Reviewed the Japanese experience, and proposed a modification to the Spitz classification depicted in Table 8.1 [8].