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Restrictive airway disease in OA/TOF

Numerous research studies have consistently shown a restrictive pattern of pulmonary function impairment. Prevalence varies from 21% to 51.5%. (5,14,31,32) Lung volume is also reduced compared to matched non-TOF peers, as is exercise capacity. This is attributed to a number of factors, but mainly the impact of surgery on the developing lungs, with previous thoracotomy, scoliosis and vertebral anomalies reducing lung growth and thoracic movement. (14,15)

Even with these abnormalities evident on lung function testing, these often produce no symptoms in younger people, but those over 35 may develop shortness of breath, reduced exercise tolerance and increased respiratory infections as a result of this. (33)

Treatment is limited but it is important to keep the chest free of secretions to minimise risk of infection, eg by chest physiotherapy and secretion thinning agents.