Unilateral lung agenesis associated with esophageal atresia & tracheoesophageal fistula: Report of two cases & review of literature

The association of lung agenesis with esophageal atresia (EA) & tracheoesophageal fistula (TEF) is exceedingly rare with a lethal outcome. We describe two neonates with this association who underwent primary repair shortly after birth. One of these neonates also had gross hydrocephalus, bilateral microtia and right thumb deformity. The goal in the management of these patients is early protection and preservation of respiratory units which is best accomplished by primary repair.