Background: Oesophageal atresia (OA) and tracheoeosophageal fistula (TOF) are congenital malformations that can affect lung development and subsequent respiratory health.
Aim: To define respiratory problems, their investigation and management as experienced by children and young people with OA/TOF seen in our regional paediatric centre.
Methods: Search criteria for those with a diagnosis of oesophageal atresia/tracheoesophageal fistula (OA-TOF) attending hospital from 01/01/2001 to 31/12/2020 were used to identify patients. Electronic health records were used to gain retrospective data.
Results: We identified 223 patients with a diagnosis of OA-TOF. Approximately 50% are managed in our joint respiratory/surgical OA-TOF clinic for respiratory symptoms.
49 patients (22%) had documented spirometry, with 13 (6%) having more detailed lung function. 21 (9%) had reduced lung function with FEV1 <80% predicted, whilst 13 (6%) had markedly reduced lung function (FEV1 <66% predicted). The most common pattern found was a restrictive pattern. Those with more detailed lung function were generally found to have normal gas transfer.
Of those with reduced FEV1, 11 had undergone CT chest. This demonstrated compression of the trachea by vasculature in two patients and reduced lung volume related to oesophageal replacement in two patients. Lung parenchyma was found to be normal in all patients. No one had a diagnosis of bronchiectasis.
Conclusion: We present a 20 year experience of OA-TOF and associated respiratory problems in these patients. Respiratory symptoms are common in these children and a small number have significantly reduced lung function.