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

Abstract

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.

Impaired VEGF Signaling in Lungs with Hypoplastic Esophageal Atresia and Effects on Branching Morphogenesis

“…Survivors of EA-TEF often suffer from chronic respiratory tract disease characterized by hoarse cough, repeated pneumonia or bronchiectasis and chronic bronchitis [20][21][22]. Peptic bronchitis, gastro-esophageal reflux or fistula stump account in part for these sequelae, but defects in the airway or lung parenchyme, or lung hypoplasia might also play a role [12,23,24].…”

Aspiration Risk and Respiratory Complications in Patients with Esophageal Atresia

“…Using CT scanning, rates of bronchiectasis in EA/TEF survivors may be as high as 27% (4, 55). While neither DeBoer et al (4) nor Cartabuke et al (60) examined potential associations with bronchiectasis (59), bronchiectasis in this population has generally been associated with massive aspiration, including patients with gastric or colonic interposition in a selected referral population (22), longstanding GERD (61), massive TEF pouch secretions, trisomy 21 (62), undiagnosed TEF (63), or broncho-esophageal fistula (64, 65). …”

Low-Power and Low-Cost Stiffness-Variable Oesophageal Tissue Phantom

“…The robotic implant aims to lengthen oesophageal tissue in vivo for a duration of weeks in order to reconstruct missing oesophageal tissue in the treatment of longgap oesophageal atresia (LGEA) [10,11]. During the in vivo performance, the oesophageal tissue experiences not only a change in length as a result of physiological factors, e.g., growth, peristaltic motion [13], but also a change in stiffness, as a consequence of inflammatory responses that are expected in any surgical intervention or implantation over time [6,7]. It is desired to have an oesophageal phantom capable of mimicking these responses realistically, as a benchtop testing platform for the robotic implant before in vivo evaluation.…”