Congenital airway abnormalities requiring tracheotomy: a profile of 56 patients and their diagnoses over a 9 year period.

We reviewed the 9 year experience at the Children's Hospital of Philadelphia with patients requiring tracheotomy for a diagnosis of congenital airway abnormalities. Of the 56 patients, 28 (50%) had cardiovascular, or chromosomal abnormalities, neurologic conditions, or congenital syndromes, 24 (43%) were born prematurely, and 13 (23%) were found to have gastroesophageal reflux. Only 18 (32%) went on to eventual decannulation of their tracheotomy with a mean tracheotomy duration of 1.75 years. The majority of patients (75%) had multiple presenting signs. Stridor was the most common (54%), followed by accessory respiratory effort (39%), cyanosis (30%), apnea (29%), and failure to thrive (23%). Twenty eight patients (50%) had multiple airway abnormalities contributing to their need of a tracheotomy for airway protection orr ventilator dependence. Laryngeal abnormalities were found in 71% of patients, tracheal abnormalities in 48% of patients, bronchial abnormalities in 11%, and upper airway obstruction in 14%. Of the laryngeal abnormalities, laryngomalacia was the most common, followed by subglottic stenosis, glottic web, and vocal cord paralysis. Tracheomalacia was the most common tracheal abnormality. The relatively large percentage of patients with cardiovascular or other major malformations, and prematurity, accounts for comorbid factors in the need for prolonged tracheotomy (and low early decannulation rate). Although gastroesophageal reflux was found in a recognizable portion off the patients, it is unclear whether this represents a comorbid condition.