Esophageal atresia, choanal atresia, and dysautonomia

Patients with esophageal atresia (EA) or choanal atresia (CA) manifest similar clinical and pathophysiological features. To determine the significance of this observation, the clinical records of 80 patients with EA and 57 with CA were reviewed. This survey showed that similarities between the two conditions included inspiratory and expiratory dyspnea, episodes of reflex apnea and/or bradycardia, oropharyngeal dysphagia, vomiting, convulsions, hyperhydrosis, hyperthermia, sialorrhea, and sudden death. After the second year of life most symptoms disappeared spontaneously. In both conditions, respiratory effort resulted in partial or complete obstruction affecting both the inspiratory and expiratory phases of the respiratory cycle. Support for this finding was obtained by studying the breathing pattern of 3 patients with EA and 3 with CA, before and during postural respiratory loading. The data suggest that patients with EA are similar to those with CA, having upper airway instability that may result in obstructive hypopnea or apnea associated with expiratory grunting. It is possible that this upper airway instability is a manifestation of more general maturational dysautonomia previously not recognised in patients with EA.