Since 1989, 31 children presented with tracheobronchial and/or oesophageal compression secondary to vascular anomalies. Their median age was 7 months (range 3 weeks – 12 years).
Most important respiratory symptoms were stridor (14), recurrent airway infection (13) and moderate to severe respiratory distress (10). Four patients required preceding ventilatory support. Upper digestive complaints were present in 11 patients.
Investigation, mainly including bronchoscopy and angiography revealed several pathologic disorders as right aortic arch with aortic diverticulum and double aortic arch in 15 children, innominate artery compression in 7, arteria lusoria in 5 and pulmonary artery sling in 1. Five children developed tracheobronchial compression secondarily to previous repair of a complex cardiac malformation. Excluding these postsurgical cases, an associated intracardiac defect was present in 12 children, which had been corrected previously in 5 patients.
The often insidious clinical presentation resulted in a median delay between the onset of symptoms and time of surgery of 97 days (4 – 450).
Surgical relief of tracheobronchial compression was successful in 30 patients. A single baby with palliated Tetralogy of Fallot and pulmonary artery sling died because of intractable extensive tracheobronchial stenosis with complete rings.
Postoperative recovery was uneventful in 21 children. Pulmonary infection was the major complication. The median ventilation time was 18 hours and the median ICU stay 4 days.
During a follow-up period of 3 to 90 months, most patients were completely or nearly completely free of symptoms after a median delay of 6 months. One child required reoperation by aortopexia after 42 months. One child died late after a third correction of his cardiac anomaly.
In conclusion, tracheobronchial compression of vascular origin in children is uncommon and may be masked by indistinct and non-specific respiratory symptoms, resulting in an often delayed diagnosis. Besides bronchoscopy, angiography and eventually MRI are mandatory to delineate accurately the variant pathologic patterns. However, surgical repair is often straightforward and effective, in spite of the common presence of tracheobronchial malacia.