O3296: Revisiting Prognostic Scoring of Esophageal Atresia in the Current Era

Background: In 1994, Spitz determined birth weight less than 1500g and congenital heart defects (CHD) had the greatest influence on survival in tracheoesophageal atresia. This classification system remains widely used in prognostication. Neonatal intensive care and anesthesia have improved the past few decades. It is uncertain how these advances have affected the prognostic utility of Spitz’s classification. Our study aimed to evaluate the outcomes of patients with esophageal atresia based on Spitz classification.

Methods: A retrospective cohort study was performed using TriNetX– an international, deidentified database of electronic health record data. The database was queried for patients less than 1 year diagnosed with tracheoesophageal atresia who underwent operation between January 1, 2003 – January 1, 2023. Patients grouped based on Spitz classification: (I) no CHDs and birth weight >1500g, (II) CHD present or birth weight < 1500g, (III) CHD present and birth weight < 1500g. Demographics, mortality rates, types of congenital heart defects were collected. Spitz class correlation with mortality and complications was analyzed with logistic regression.

Results: 2,988 subjects were identified over the study period: (I) 1174, (II) 1772, (III) 42. More subjects were white (1,719, 58%), non-Hispanic (1,841, 62%), and male (1,520, 51%). Mortality rates were (I) 1.7% (20/1174), (II) 3% (53/1772), and (III) 19% (8/42). Increasing Spitz class was positively associated with mortality (OR 2.574, CI 1.652 - 4.069, p<.0001), postoperative sepsis (OR 5, CI 3.401 - 7.466, p<.0001), GERD (OR 1.969, CI 1.696 - 2.290, p<.0001), failure to thrive (OR 2.776, CI 2.217 - 3.498, p<.0001), tracheomalacia (OR 2.776, CI 2.217 - 3.498, p<.0001), and pneumonia (OR 2.776, CI 2.217 - 3.498, p<.0001). Subanalysis was performed of subjects who died within 1 year of operative repair (n=63) compared to those who survived (n=2,343). Congenital cardiac defects (46/63 [73%] vs 1,306/2,343[54%], p=0.002), premature birth (23/63 [37%] vs 563/2,343 [23%], p=0.013), and extremely low birthweight < 1000g (10/23 [16%] vs 173/2,343 [7%], p=0.008) were significantly more common in the group who died within one year. The most common congenital heart anomalies occurring in subjects who died within one year included septal defects (59%), conotruncal abnormalities (46%), congential defects of the cardiac chambers (30%) and aortic or mitral valves abnormalities (22%).

Conclusion: Mortality rates in tracheoesophageal atresia have declined in the last twenty years. Despite this, patients with Spitz higher classifications continue to demonstrate poorer postoperative outcomes. Those who die within 1 year of operative repair continue to demonstrate higher rates of congenital heart defects with lower birth weights than previously described in addition to prematurity. The Spitz classification can be a useful tool in determining postoperative prognosis for patients with tracheoesophageal atresia, but deeper evaluation should be made on impact of specific congenital heart defects.