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1840 TRACHEOBRONCHOMALACIA - NEW CAUSES AND TREATMENT

We describe 7 infants (6 males, 1 female) seen over the last 3 yrs. initially diagnosed with bronchopulmonary dysplasia (BPD) or chronic lung disease and subsequently found to have tracheobronchomalacia (TBM). None had congenital anatomical abnormalities such as tracheoesophageal fistula or vascular rings. 5 were premature babies who had been ventilated for hyaline membrane disease, 2 presented with viral infections. All 7 had required intermittent ventilation, with 5/7 ventilated for most of their lives (9 mo.- 38 mo.). The diagnosis was suspected in all 7 because they had either cyanotic spells (“BPD” or “blue” spells) requiring manual ventilation, or wheezing unresponsive to usual therapy (bronchodilators, steroids). The diagnosis was confirmed by either bronchoscopy or fluoroscopy showing>75% collapse of the central airways during expiration or Valsalva. The mean time from onset of symptoms to diagnosis was 3-20 mos. Some associated factors include Pseudomonas sp. or Staphylococcus aureus tracheitis in 6/7, gastroesophageal reflux in 3/7, patent ductus in 4/7, and pneumothoraces in 4/7. Management was facilitated by finding optimal positive end-expiratory pressure (PEEP) during fluoroscopy. 6/7 require PEEP of at least 10-15cmH2O pressure for at least 3 mos., and 5/7 are still ventilated. All patients responded clinically with decreased cyanotic spells and improved ventilation. Bronchodilators, diuretics and sedation were all used with varying success. Tracheostomy without PEEP was not helpful. Thus TBM is a treatable, diffuse process which requires long term therapy, the incidence of TBM may be higher than suspected, and the therapy of choice is long term optimal distending PEEP.