Recurrent tracheo-oesophageal fistula in adulthood

Recurrent TOF is a known complication of surgical repair of OA in infancy, but can also occur, very rarely, in adulthood. There are a small number of case reports of this occurring, including multiple in the same adult patient. Symptoms include severe coughing symptoms and recurrent aspiration pneumonia. The case reports identified the fistulae using bronchoscopy and were repaired surgically. (34,35)

Recommended long-term management of patients with repaired OA/TOF and respiratory morbidity (15)

Mild

Defined as SpO2 at rest from 90% to 93% and/or normal-to-slightly abnormal chest radiography and/or FEV1 ≥ 70% predicted and/or FVC ≥ 70% predicted:

• Primary care follow-up
• Prompt aggressive treatment of infection
• Functional assessment once a year
• Consider referral to respiratory physician (preferably one experienced with OA/TOF) if clinical deterioration
• Annual influenza vaccination is recommended in people born with OA/TOF
• Pneumococcal vaccination is recommended
• Covid booster is recommended as those with OA/TOF are a vulnerable group

Moderate/severe

Defined as SpO2 at rest < 90% and/or relevant abnormalities at chest radiography and/or FEV1 < 70% predicted and/or FVC < 70% predicted

• Regular respiratory physician follow-up (preferably one experienced with OA/TOF)
• Advanced lung imaging at least at baseline
• Endoscopy
• Annual influenza vaccination is recommended in people born with OA/TOF
• Pneumococcal vaccination is recommended
• Covid booster is recommended as those with OA/TOF are a vulnerable group