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Esophageal atresia with pulmonary agenesis

R J Brereton, A M Rickwood

    Journal of Pediatric Surgery
    |October 1, 1983
    PubMed

    Insights

    Infants with esophageal atresia and lung agenesis or severe pulmonary hypoplasia have a fatal outcome. Congenital heart disease, prematurity, and other anomalies contribute to mortality in these rare neonatal cases.

    Area of Science:

    • Neonatal medicine
    • Pediatric surgery
    • Developmental biology

    Background:

    • Esophageal atresia (EA) is a congenital anomaly of the foregut.
    • Agenesis of the lung is a rare congenital anomaly.
    • Pulmonary hypoplasia is characterized by incomplete lung development.

    Observation:

    • Two neonates presented with esophageal atresia and agenesis of the right lung.
    • A literature review identified 11 additional cases of esophageal atresia with severe pulmonary hypoplasia.

    Findings:

    • All infants (13 total cases) with esophageal atresia and severe pulmonary hypoplasia or lung agenesis died.
    • Congenital heart disease accounted for 50% of the mortality.
    • The remaining deaths were attributed to prematurity and other severe congenital anomalies, alongside respiratory complications.

    Implications:

    • This condition represents a severe spectrum of congenital anomalies with universally fatal outcomes.
    • Early identification and management strategies for associated anomalies are critical.
    • Further research into the genetic and environmental factors underlying these complex malformations is warranted.

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