Clinical characteristics of children with tracheobronchial anomalies.

Tracheobronchial anomalies (TBA) originate between the third and sixteenth week of gestation, and they primarily affect the main bronchi. The reported incidence varies between 1-3% of pediatric endoscopic studies. The objective of our review was to describe the clinical characteristics of patients with TBA diagnosed by flexible bronchoscopy (FB). During the period from March 1993-May 2001, we performed 580 FB at the Pediatric Services of the Catholic University Hospital. During this study period, 52 patients (9.65%) had a TBA (32 males, or 61.5%), with a mean age of 21.2 months (range, 7 days to 6 years). In 39 cases (75%), the diagnosis was made under age 2 years. Thirty-six patients with symptoms related to TBA were symptomatic (70%), and in 16 cases (30%) the TBA was an accidental finding during FB. The main clinical manifestations were recurrent atelectasis of the right upper lobe (RUL), recurrent pneumonia, or congenital stridor. Most of the TBA were localized at the RUL bronchus (47%) and were characterized by an anomaly at its origin, either agenesis or a supernumerary bronchus. Also, we found patients with tracheal bronchus and congenital tracheal stenosis. In 6 patients (12%), we had more than one TBA, the most frequent being the association between RUL and RML anomalies. In 12 cases we found another congenital airway anomaly, mainly laryngomalacia. Patients had associated diseases, such as genetic disorders in 16 cases (particularly Down syndrome), and congenital cardiopathies. In summary, in our clinical experience, TBA are more frequent than previously reported. The majority of young patients have symptoms or signs, and most are localized to the RUL. We found patients with more than one TBA, laryngomalacia associated with TBA, and at a high frequency related to Down syndrome. Copyright 2003 Wiley-Liss, Inc.