Congenital tracheoesophageal, umbilical, vascular and meningeal fistulas

Abstract

Esophageal atresia (EA) is a rare congenital anomaly (1:25004500 live births) which is most frequently associated with tracheoesophageal fistula (TEF). TEF and EA are two components of VACTERL association of congenital anomalies: “V” for vertebral or vascular anomalies, “A” for anal atresia, “C” for cardiac abnormalities, “T” for tracheoesophageal fistula, “E” for esophageal atresia, “R” for renal abnormalities, and “L” for limb anomalies.2,3 VACTERL is an association rather than a syndrome as its components are not pathologically caused but embryologically formed due to mesodermal defects.4 When at least two5 or three6,7 components of these congenital anomalies are identified in the same neonate, VACTERL association is diagnosed. Patients with EA most likely suffer from gastroesophageal reflux disease (GERD).8