8.5 Tracheoesophageal Fistula and Esophageal Atresia
Key Features
Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are the result of a congenital communication between the trachea and esophagus.
EA is also present in most cases of TEF.
These congenital anomalies present with respiratory and/or feeding difficulties in the newborn.
Congenital TEF and EA are common congenital anomalies that usually occur together. Most cases are diagnosed immediately following birth or during infancy due to the associated life-threatening complications. However, isolated TEF may escape diagnosis until later. Problems with growth and feeding, pulmonary complications, and gastroesophageal morbidity may result from the condition.
Epidemiology
TEF and EA in their various forms occur in ~ 1 in 3,000 live births, with a slight male predominance. In over 50% of cases, TEF and EA are linked with other defects, such as in the VACTERL (vertebral defects, imperforate anus, cardiac anomalies, TEF, renal dysplasia, limb defects) association, and with chromosomal anomalies.
Clinical
Signs and Symptoms
A sign of the condition, though nonspecific, is polyhydramnios on prenatal ultrasound. Symptoms of TEF include recurrent chest infections, cyanosis and choking on feeding, and abdominal distension. When EA is also present, the patient will not be able to swallow saliva or feed and will drool excessively.
Differential Diagnosis
Aspiration pneumonia
Laryngeal cleft
Tracheomalacia
Esophageal stricture
Esophageal diverticula
Vascular ring
Gastroesophageal reflux
Other feeding problems
TEF and EA develop as a result of incomplete separation of the respiratory and digestive divisions of the primitive foregut. Several variants of TEF and EA have been described, but five types predominate, as shown in Fig. 8.2 .