Tracheomalacia is associated with lower FEV₁ and Pseudomonas acquisition in children with CF

Abstract

BACKGROUND Tracheomalacia (TM) occurs in approximately 1 in 2,100 children. Because the trachea develops abnormally in animal models of cystic fibrosis (CF), we hypothesized this may also occur in children with CF, increasing their risk of TM. PURPOSE To examine the prevalence and clinical consequences of TM in children with CF. METHODS We studied children with CF born between 1995 and 2012. TM was defined as dynamic collapse of the trachea, and the severity was recorded as described in the chart. The effect of TM on patient outcomes, including FEV1, CT changes, and acquisition of CF pathogens, was assessed using a longitudinal patient dataset. RESULTS 89% of children with CF had at least one bronchoscopy (n = 97/109). 15% of these children had TM described in any bronchoscopy report (n= 15/97). Of the patients with TM, 8 had meconium ileus (p = 0.003) and all were pancreatic insufficient. Pseudomonas aeruginosa infection occurred 1.3 years earlier among children with TM (p = 0.01). Starting FEV1 values by age 8 were diminished by over 18% of predicted for patients with TM. Life-threatening episodes of airway obstruction occurred in 3 of 15 patients with CF and TM, including one leading to death. Gender, prematurity, and hepatic disease were not associated with TM. No difference was observed in the frequency of bronchiectasis. CONCLUSIONS TM is significantly more common in infants and children with CF than in the general population and is associated with airway obstruction and earlier Pseudomonas acquisition.

Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

“…Similar features have been reported in mice with CF and comparable abnormalities are observed in infants and young children with CF (15)(16)(17)(18)(19). Knowing that air trapping is present in humans with CF as early as weeks to months after birth and that loss of CFTR function leads to developmental airway abnormalities, we hypothesized that air trapping and airflow obstruction are present at birth and occur before the onset of airway infection, inflammation, and mucus accumulation.…”

Effect of Prenatal versus Postnatal Vitamin D Deficiency on Pulmonary Structure and Function in Mice

“…Thinner tracheal cartilage, therefore, may be a risk factor for tracheomalacia in the vitamin D-deficient mouse model. In humans, pliable tracheal cartilage is known to adversely affect flow dynamics during lower respiratory tract diseases (33,34).…”

Direct tracheobronchopexy to correct airway collapse due to severe tracheobronchomalacia: Short-term outcomes in a series of 20 patients

“…Furthermore, the adverse effects of TBM are heightened in such patient groups as esophageal atresia, congenital heart disease, and cystic fibrosis patients given the potential for airway obstruction, frequent infections, a potential for more limited physiological reserve, and mortality [2]. In the most severe cases, apparent life-threatening events (ALTE) or recurrent respiratory decompensation may require emergent intervention.…”

“…Coaptation of the airways with anterior and posterior collapse in spontaneously breathing patients indicates severe TBM [1][2][3][4][5][6][7][8][9]. TBM is commonly treated with aortopexy, which indirectly elevates the anterior wall of the trachea [1].…”

“…However, in our experience, anteriorly malformed tracheal cartilage and posterior tracheal membrane intrusion may limit the effectiveness of aortopexy in some cases and may require further interventions. Therefore, we reviewed a series of patients who underwent a more direct anterior and/or posterior tracheobronchopexy for severe TBM to determine the following: (1) the resolution of the symptoms that warranted the operative procedure; and, (2) to provide bronchoscopic evidence of the effectiveness of the direct tracheobronchopexy procedure. reviewed.…”