Long-term esophageal and respiratory outcomes in children with esophageal atresia and tracheoesophageal fistula

OBJECTIVES: Few studies have evaluated the long-term complications and outcomes of esophageal atresia with or without tracheoesophageal fistula (EA/TEF) beyond childhood. The aim of our study was to characterize the esophageal and respiratory morbidity of EA/TEF through evaluation of clinical symptoms, diagnostic testing and therapeutic intervention at a tertiary care center. METHODS: Patients with congenital EA/TEF evaluated from 2011 to 2014 were included. Demographic characteristics, type and mode of repair of EA/TEF, clinical symptoms, radiographic, endoscopic, bronchoscopic and medication use data were obtained. RESULTS: A total of 43 patients were identified. The median age of this predominantly Caucasian population was 8 years (interquartile range: 3, 20). Twenty (62.5%) had type C (EA with distal TEF) abnormality. Twenty-one (48.8%) patients had heartburn, 19 (44.1%) had acid regurgitation, and 31 (72.1%) had dysphagia to solids. Barium swallow in 26 patients revealed strictures in 17 (65.4%), dysmotility in 20 (76.9%) and recurrent fistulas in four patients (15.4%). Thirty patients underwent upper endoscopy, of which 21 (70.0%) had a stricture, and six (20.0%) had recurrent fistula requiring surgical intervention. Eight (18.6%) patients underwent fundoplication. Pulmonary evaluation showed cough and choking in 31 (72.1%) patients and dyspnea and wheezing in 32 (53.4%) patients. Recurrent respiratory infections were reported in 19 (44.2%). PATIENTS: Other findings included tracheomalacia in 86.7% and restrictive lung disease in 54.5% of patients. CONCLUSION: There is a high burden of residual esophageal and pulmonary pathology in patients with EA/TEF. Ongoing follow-up is required to monitor both the clinical symptoms and treatment responses.