How is esophageal atresia managed in patients with tracheoesophageal fistula (TEF)?

Updated: Nov 07, 2018
  • Author: Sat Sharma, MD, FRCPC; Chief Editor: Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS  more...
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Esophageal atresia with tracheoesophageal fistula is a relatively common congenital anomaly. Research with rodent models is contributing to the scientific understanding of the condition. Advances in surgical care and neonatal management have improved survival to approximately 90%. Long-gap and isolated esophageal atresia present significant management challenges. Post-operative and long-term complications including esophageal stricture, gastro-esophageal reflux, and respiratory compromise remain relatively common and continue to present a challenge for ongoing patient management. [15]

Repair of esophageal atresia and tracheoesophageal fistula has traditionally been performed via thoracotomy. Recent attempts at thoracoscopic repair have shown that such repair is feasible but technically challenging. [16] However, more data are needed for further evaluation of this approach, particularly in long-gap defects that require more extensive dissection and difficult anastomosis.

Wang and colleagues examined national outcomes in newborn patients with esophageal atresia and tracheoesophageal fistula (EA/TEF) in the United Sates, using the Kids' Inpatient Database (KID). Investigators analyzed inpatient admissions for pediatric patients with EA/TEF. They identified 4168 cases with a diagnosis of EA/TEF. The overall in-hospital mortality was 9%. Univariate analysis revealed lower survival in patients with associated acute respiratory distress syndrome, ventricular septal defect (VSD), birth weight (BW) < 1500 g, gestational age (GA), time of operation within 24 h of admission, coexisting renal anomaly, imperforate anus, African American race, and lowest economic status. Multivariate logistic regression identified BW < 1500 g, operation within 24 h, GA 17</ref>

In a different study of 268 infants with EA/TEF, 8 (3%) were extremely low birth weight (ELBW, <1000 g) and had high morbidity and mortality mainly associated with complications not related to EA/TEF repair. [18]

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