Long term pulmonary morbidity after esophageal atresia and tracheoesophageal fistula repair


  • Sathyaprasad Burjonrappa Rutgers University




Esophageal atresia, Pulmonary morbidity, GERD


Background: Respiratory morbidity can influence a patient's quality of life after successful EA/TEF (Esophageal Atresia with and without Tracheo-Esophageal Fistula) repair. Multidisciplinary clinics have made it easier to manage comorbidities in patients with complex congenital malformations. The aim of this study was to evaluate the impact of respiratory issues after EA/TEF repair.  Secondarily we sought to evaluate the contribution of respiratory symptoms by Broncho-Pulmonary Dysplasia (BPD) and Gastro-Esophageal-Reflux-Disease (GERD) in patients with EA/TEF.

Methods:  Retrospective review of the medical record of 50 patients, who underwent EA/TEF repair, needing hospital readmission for pulmonary morbidity, and subsequently followed up in a surgical clinic was performed. The data collected included patient demographics, presence and nature of significant respiratory comorbidity, findings on imaging studies and bronchoscopy, and results of pulmonary function tests (PFT).

Results: Respiratory issues were identified in 75% of the patients. Congenital malformations and tracheomalacia were found in n=7 (14%) of cases. Prematurity associated BPD and Gastro-Esophageal Reflux were not the major cause of respiratory symptoms. Respiratory morbidity in this population included recurrent pneumonia n=18 (36%), reactive airway disease n=16 (32%), bronchiolitis n=4 (8%), bronchiectasis n=2 (4%), laryngitis n=2 (4%) and empyema n=1 (2%).

Conclusions: Pulmonary complications significantly impact the quality of life in terms of respiratory events, after successful EA/TEF repair. While GERD is common in surgically repaired EA/TEF patients, its exact role in precipitating pulmonary morbidity needs further study. Tracheomalacia can be managed conservatively without resorting to aortopexy.


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Rasheed A, Tindall S, Cueny DL, Klein MD, Delaney-Black V. Neurodevelopmental outcome after congenital diaphragmatic hernia: Extracorporeal membrane oxygenation before and after surgery. J Pediatr Surg. 2001; 36:539-44.

Muratore CS, Kharasch V, Lund DP, Sheils C, Friedman S, Utter S, et al. Pulmonary morbidity in 100 survivors of congenital diaphragmatic hernia monitored in a multidisciplinary clinic. J Pediatr Surg. 2001; 36:133-40.

Jancelewicz T, Vu LT, Keller RL, Bratton B, Lee H, Farmer D, et al. Long-term surgical outcomes in congenital diaphragmatic hernia: observations from a single institution. J Pediatr Surg. 2010; 45:155-60.

Holland AJ, Ron O, Pierro A, Drake D, Curry JI, Kiely EM, et al. Surgical outcomes of esophageal atresia without fistula for 24 years at a single institution. J Pediatr Surg. 2009; 44:1928-32.

Gratadour P, Védrinne JM, Guillaume C, Gagnieu MC, Motin J. Intra-macrophage lipid particles collected by bronchoalveolar lavage: incidence and diagnostic value. Ann Fr Anesth Reanim. 1993; 12:462-68.

Adams R, Ruffin R, Campbell D. The value of the lipid-laden macrophage index in the assessment of aspiration pneumonia. Aust N Z J Med. 1997; 27:550-3.

Sistonen S, Malmberg P, Malmstrom K, Haahtela T, Sarna S, Rintala RJ, et al. Repaired esophageal atresia: Respiratory morbidity and pulmonary function in adults in a population-based long-term follow-up study. Eur Respir J. 2010; 36:1106-12.

Malmstrom K, Lohi J, Lindahl H, Pelkonen A, Kajosaari M, Sarna S, et al. Longitudinal follow-up of bronchial inflammation, respiratory symptoms, and pulmonary function in adolescents after repair of esophageal atresia with tracheo esophageal fistula. J Pediatr. 2008; 46:396-401.

Patria MF, Ghislanzoni S, Macchini F, Lelii M, Mori A, Leva E, et al. Respiratory morbidity in children with repaired congenital esophageal atresia with or without tracheoesophageal fistula. Int J Environ Res Public Health. 2017; 14:1136.

Rothenberg S, Cowles R. The effect of laparoscopic Nissen fundoplication on patients with severe gastroesophageal reflux disease and steroid dependent asthma. J Pediatr Surg. 2012; 47:1101-4.

Fayon M, Donato L. Tracheomalacia (TM) or bronchomalacia (BM) in children: conservative or invasive therapy. Arch Pediatr. 2010; 17:97-104.

Duerloo JA, Ekkelkamp S, Schoorl M, Heij HA, Aronson DC. Esophageal atresia: historical evolution of management and results in 371 patients. Ann Thorac Surg. 2002; 73:267-72.

Perger L, Kim HB, Jaksic T, Jennings RW, Linden BC. Thoracoscopic aortopexy for treatment of tracheomalacia in infants and children. J Laparoendosc Adv Surg Tech A. 2009; 19:S249-54.

Etesami M, Ashwath R, Kanne J, Gilkeson RC, Rajiah P. Computed tomography in the evaluation of vascular rings and slings. Insights Imaging. 2014; 5:507-21.

Sadreameli SC, McGrath-Morrow SA. Respiratory care of infants and children with tracheo-esophageal fistula and esophageal atresia. Pediatr Respir Rev. 2016; 17:16-23.

Cozzi DA, Zani A, Conforti A, Colarizi P, Moretti C, Cozzi F. Pathogenesis of apparent life-threatening events in infants with esophageal atresia. Pediatr Pulmonol. 2006; 41:488-93.



How to Cite

Burjonrappa S. Long term pulmonary morbidity after esophageal atresia and tracheoesophageal fistula repair. J Neonatal Surg [Internet]. 2021Feb.4 [cited 2021Sep.25];10:10. Available from: https://www.jneonatalsurg.com/ojs/index.php/jns/article/view/914



Original Article