Respiratory morbidity in children with tracheoesophageal fistula

Tracheoesophageal fistula (TEF) is a common congenital anomaly of the respiratory tract that can be isolated or associated to malformations of the digestive tract. The defective lateral septation of the foregut during the embryonic period is the most commonly accepted pathogenic hypothesis. Respiratory morbidity may recognize multiple aetiologies in TEF affected children. Indeed, associated airway abnormalities, tracheomalacia and bronchomalacia, airways hyperreactivity, iatrogenic laryngeal nerve damage and gastroesophageal reflux (GER) contribute to the onset of respiratory complaints. Symptoms onset (cough and cyanosis during feeding, barky cough, wheeze, asthma on exertion, inhalation pneumonia) is variable and the diagnosis is often not straightforward. Combined flexible bronchoscopy and digestive endoscopy are the main diagnostic modalities, supplanted by radiographic modalities especially CT scans of the chest. Fistula ligation or endoscopic repair by using tissue or fibrin adhesive are commonly employed. Because of the association of TEF with abnormalities involving other organs and systems, a multidisciplinary approach should be considered in the management of children with TEF. Although respiratory problems are more frequent during childhood, they tend to persist into adulthood. Therefore, transition of care from paediatric to adult services is imperative.