Fig. 1
Types of esophageal atresia and tracheoesophageal fistula. (a) Pure esophageal atresia without fistula; (b) proximal tracheoesophageal fistula, blind distal pouch; (c) distal tracheoesophageal fistula, blind proximal pouch; (d) proximal and distal tracheoesophageal fistula; (e) intact continuous esophagus with H-type tracheoesophageal fistula (Source: Bryan Walters)
(i)
Esophageal atresia with no fistula, 7.8 % (pure esophageal atresia).
(ii)
B: Esophageal atresia and a proximal fistula, 0.8 %.
(iii)
C: Esophageal atresia and a distal fistula, 85.8 % (most common type).
(iv)
D: Esophageal atresia with both a proximal and a distal fistula, 1.4 %.
(v)
Fistula to the trachea with no esophageal atresia 4.2 % (H-type fistula).
(b)
Embryology:
(i)
The esophagus and the trachea appear at three weeks gestation and develop into separate structures up to the level of the larynx by the 4th week of gestation. The defect occurs by the 20th week of gestation with rapid growth of the trachea. A vascular defect accounts for pure esophageal atresia.
(c)
Incidence: 1 in 4,000–5,000 live births.
(d)
Associated anomalies:
(i)
Maternal polyhydramnios: 60 % of patients.
(ii)
Cardiac: 30 % with patent ductus arteriosum, ventricular septal defect, or atrial septal defect.
(iii)
Gastrointestinal: 12 % have imperforate anus, duodenal atresia or pyloric stenosis.
(iv)
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VACTERL constellation of anomalies: 20–30 % cases.
1.
V: Vertebral.
2.
A: Anorectal.
3.
C: Cardiac.
4.
TE: Tracheal-esophageal.
