respiratory manifestations in children and adults born with oesophageal atresia (OA)/ tracheo - oesphageal fistula (TOF)

Last edited 08/2022 and last reviewed 09/2022

Respiratory manifestations in children and adults born with oesophageal atresia (OA)/ tracheo - oesphageal fistula (TOF)

Around 50% of adults born with OA/TOF have respiratory symptoms every year, and in early childhood this is much higher. (1)

Clinical features:

• chronic cough/TOF cough (a hoarse brassy cough due to tracheomalacia)
• coughing associated with eating
• difficulty clearing secretions
• recurrent chest infections (2)
• slow recovery from chest infections
• wheeze that is aggravated by or doesn’t respond to bronchodilators
• sleep difficulties (apnoea, nocturnal cough, nocturnal choking) (3)

Causes:

• tracheomalacia- 87% had this in one study (4,5)
• dysphagia and aspiration due to airway reflux (6)
• bronchiectasis (3)
• undiagnosed airway defects e.g. laryngeal cleft (7)
• restrictive lung disease due to surgery and associated anomalies(6)
• recurrent tracheo-oesophageal fistula (8,9)

Contributor:

• Dr Caroline Love (August 2022)
• Associate Specialist in Dermatology at York Hospitals Trust; Adult born with OA/TOF and representative of TOFS charity

Reference:

• Gatzinsky V, Jönsson L, Ekerljung L, Friberg LG, Wennergren G. Long-term respiratory symptoms following oesophageal atresia. Acta Paediatrica. 2011;100(9):122-5.
• Sistonen SJ, Koivusalo A, Nieminen U, Lindahl H, Lohi J, Kero M, et al. Esophageal morbidity and function in adults with repaired esophageal atresia with tracheoesophageal fistula: a population-based long-term follow-up. Ann Surg. 2010 Jun;251(6):1167-73.
• Kovesi T. Aspiration Risk and Respiratory Complications in Patients with Esophageal Atresia. Front Pediatr [Internet]. 2017 [cited 2021 Aug 2];0. Available from: https://www.frontiersin.org/articles/10.3389/fped.2017.00062/full
• Baxter KJ, Baxter LM, Landry AM, Wulkan ML, Bhatia AM. Structural airway abnormalities contribute to dysphagia in children with esophageal atresia and tracheoesophageal fistula. Journal of Pediatric Surgery. 2018 Sep;53(9):1655-9.
• Roberts K, Karpelowsky J, Fitzgerald DA, Soundappan SS. Outcomes of oesophageal atresia and tracheo-oesophageal fistula repair. J Paediatr Child Health. 2016 Jul;52(7):694-8.
• Sadreameli SC, McGrath-Morrow SA. Respiratory Care of Infants and Children with Congenital Tracheo-Oesophageal Fistula and Oesophageal Atresia. Paediatr Respir Rev. 2016 Jan 1;17:16-23.
• Shah AR, Lazar EL, Atlas AB. Tracheal diverticula after tracheoesophageal fistula repair: case series and review of the literature. J Pediatr Surg. 2009 Nov;44(11):2107-11.
• Lin JH, Deng L, Li X. Recurrent tracheoesophageal fistula in an adolescent without persistent symptoms: A case report. Medicine (Baltimore). 2017 Nov;96(46):e8668.
• Downey P, Middlesworth W, Bacchetta M, Sonett J. Recurrent and congenital tracheoesophageal fistula in adults. Eur J Cardiothorac Surg. 2017 Dec 1;52(6):1218-22.