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Respiratory problems

Nearly half of adults with OA/TOF will have respiratory symptoms each year. In one study, 30% had physician-diagnosed asthma, 44% reported wheezing in the last year, 28% recurrent wheeze, 30% long-standing cough, 25% cough and sputum. (4)  This is also reflected in pulmonary function test results, where 21% demonstrate a restrictive pattern, 21% obstructive and 36% both. (5)

Causes

  1. Dysphagia and aspiration.
    This is multifactorial in cause, and varies between individuals. Factors include unsafe swallow, oesophageal dysmotility and reflux, and pooling of upper airway secretions due to dysmotility, airway reflux, strictures and tracheal pouches. (6) The Hull Airway Reflux Questionnaire is 95% sensitive in diagnosing airway reflux in chronic cough.
  2. Tracheomalacia (TM) and/or bronchomalacia (BM). In a prospective study, 87% had TM. (7) TM can significantly impair secretion clearance, impair cough and increase risk of infection, and in turn delays recovery from infections. Tracheobronchomaacia (TBM) can also aggravate GORD and airway reflux. (8)
  3. Abnormal airway anatomy.
    Some patients have a tracheal posterior wall diverticulum from the repaired fistula site. These pouches can allow pooling of oral secretions and recurrent pneumonia. (9) These may not be discovered until bronchoscopy in adulthood.
  4. Restrictive lung disease.
    This is again multifactorial, including congenital vertebral and chest wall anomalies, surgical trauma, aspiration and recurrent infection. (6)
References

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