Long-Term Outcomes of Patients with Tracheoesophageal Fistula/Esophageal Atresia: Survey Results from Tracheoesophageal Fistula/Esophageal Atresia Online Communities.

Outcome studies of tracheoesophageal fistula (TEF) and/or esophageal atresia (EA) are limited to retrospective chart reviews. This study surveyed TEF/EA patients/parents engaged in social media communities to determine long-term outcomes.  A 50-point survey was designed to study presentation, interventions, and ongoing symptoms after repair in patients with TEF/EA. It was validated using a test population and made available on TEF/EA online communities.  In this study, 445 subjects completed the survey during a 2-month period. Mean age of patients when surveyed was 8.7 years (0-61 years) and 56% were male. Eighty-nine percent of surveys were completed by the parent of the patient. Sixty-two percent of patients underwent repair in the first 7 days of life. Standard open repair was most common (56%), followed by primary esophageal replacement (13%) and thoracoscopic repair (13%). Out of 405, 106 (26%) patients had postoperative leak. Postoperative leak was least likely in primary esophageal replacement (18%) and standard open repair (19%). Leak occurred in 32% of patients who had thoracoscopic repair; 31% (128/413) reported long-gap atresia, which was significantly associated with increased risk of postoperative leak (54/128, 42%) when compared with standard short-gap atresia (odds ratio, 3.5;  = 0.001). Out of 409, 221 (54%) patients reported dysphagia after repair, with only 77/221 (34.8%) reporting resolution by age 5. Out of 381, 290 (76%) patients reported symptoms of gastroesophageal reflux disease (GERD). There was no difference in dysphagia rates or GERD symptoms based on type of initial repair. Antireflux surgery was required in 63/290, 22% of patients with GERD (15% of all patients) and 27% of these patients who had surgery required more than one procedure antireflux procedure. The most common was Nissen fundoplication (73%), followed by partial wrap (14%). Reflux recurred in 32% of patients after antireflux surgery.  TEF/EA patients have long-term dysphagia and GERD that may be under reported. Retrospective studies of outcomes after TEF/EA repair may underestimate long-term esophageal dysmotility, dysphagia, GERD, and strictures that occur regardless of the type of repair and adversely affect quality of life. Fifteen percent of all TEF/EA patients surveyed required an antireflux procedure during childhood, and more than one-quarter of those required repeat surgery. These data demonstrate the need for long-term follow-up as pediatric patients transition to adult care.