Abstract
Esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) occurs in 1 out of every 3000 births. Current survival approaches 95%, and research is therefore focused on morbidity and health-related quality of life issues. Up to 50% of neonates with EA ± TEF have one or more additional malformations including those of the respiratory tract that occur in a relatively high proportion of them and particularly of those with vertebral, anal, cardiac, tracheoesophageal, renal, and limb association. Additionally, a significant proportion of survivors suffer abnormal pulmonary function and chronic respiratory tract disease. The present review summarizes the current knowledge about the nature of these symptoms in patients treated for EA ± TEF, and explores the hypothesis that disturbed development and maturation of the respiratory tract could contribute to their pathogenesis.
Highlights
Progress in surgical techniques and perinatal care allowed dramatic improvement of survival in the decades elapsed since the first successful primary repair of esophageal atresia with tracheoesophageal fistula (EA ± TEF) in 1941 [1]
Respiratory morbidity associated with EA ± TEF may be due to numerous players intrinsically related to the malformation itself or the surgical treatment, and has been often ascribed to associated disorders like tracheomalacia and gastroesophageal reflux (GER)
Because of the scarcity of pathologic material and the difficulties for carrying out embryonic studies in humans, the mutant mice and toxicologic rodent models became invaluable tools in the research of the pathophysiologic processes involved in the disturbed morphogenesis
Summary
Progress in surgical techniques and perinatal care allowed dramatic improvement of survival in the decades elapsed since the first successful primary repair of esophageal atresia with tracheoesophageal fistula (EA ± TEF) in 1941 [1]. AND CONCLUSION Respiratory morbidity did not improve and is still highly prevalent in EA ± TEF survivors despite the improvement of perinatal and surgical care, the greater awareness, and more aggressive therapeutic strategies concerning associated morbidity (in particular, GER disease and surgical treatment of tracheomalacia).
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