Prevalence of tracheobronchomalacia is higher than previously reported in children with cystic fibrosis.

Background: Tracheobronchomalacia (TBM) is estimated to be present in 1 in 2100 children. Previous reports suggest the prevalence is higher in children with cystic fibrosis (CF). This has clinical implications with potential to influence airway clearance and lung health.

Aim: To determine the prevalence and clinical associations of TBM in Western Australian children with CF.

Methods: Children with CF born between 2001 and 2016 were included. Operation reports from bronchoscopies performed until the age of 4 were retrospectively reviewed. Data were collected on the presence, persistence defined as a repeat diagnosis, and severity of TBM. Data on genotype, pancreatic status, and symptoms at CF diagnosis were extracted from the medical record. Associations between categorical variables were compared using χ and Fisher's exact test.

Results: Of 167 children (79 male), 68 (41%) were diagnosed with TBM at least once, with TBM persistent in 37 (22%) and severe in 31 (19%). TBM was significantly associated with pancreatic insufficiency (χ  = 7.874, p < 0.05, odds ratio [OR] 3.4), delta F508 gene mutation (χ  = 6.489, p < 0.05, OR 2.3), and a presentation of meconium ileus (χ  = 8.615, p < 0.05, OR 5.0). Severe malacia was less likley in females (χ = 4.523, p < 0.05, OR 0.42) . No significant relationship was found with respiratory symptoms at the time of CF diagnosis (χ  = 0.742, p = 0.39).

Conclusions: TBM was common in this group of children under the age of 4 with CF. A high index of suspicion for airway malacia should be considered in children with CF, particularly those who present with meconium ileus and have gastrointestinal symptoms at diagnosis.