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Virtual Pediatric Hospital: Paediapaedia: Esophageal Atresia and Tracheoesophageal Fistual (EA / TEF) Paediapaedia: Gastrointestinal Diseases

Esophageal Atresia and Tracheoesophageal Fistual (EA / TEF)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Prenatal ultrasound may show polyhydramnios. Soon after birth there is excessive drooling due to pooling of secretions in the blind proximal esophageal pouch, and aspiration of feedings with choking, coughing and cyanosis. Another presentation is failure to pass a nasogastric tube.

The H type TEF presents later in life than the pure EA. The H type's symptoms are choking, coughing attacks or cyanosis during feeding, and recurrent pneumonias.

Etiology/Pathophysiology:
Defective division of the embryonic foregut into the trachea and esophagus.

Types
1. Esophageal atresia without tracheoesophageal fistula (9% of cases)
2. Esophageal atresia with tracheoesophageal fistula. The fistula can be to the proximal esophagus, proximal and distal esophagus, or distal esophagus.
3. Tracheoesophageal fistula without esophageal atresia (H type tracheoesophageal fistula).

The most common type is esophageal atresia with a distal tracheoesophageal fistula.

The dilated proximal esophageal pouch compresses the trachea, which often leads to the development of tracheomalacia.

Five percent of these patients have duodenal atresia.

The H type TEF is most commonly located at the level of the upper thoracic vertebra.

The VACTER Association is a group of congenital anomalies occurring in variable combinations:
V - Vertebral body segmentation defects
A - Anal atresia
C - Cardiovascular: patent ductus arteriosus, ventricular septal defect
TE - Tracheoesophageal fistula
R - Radial ray hypoplasia, unilateral renal agenesis

Pathology:
Not applicable

Imaging Findings:
The esophageal atresia is visualized on the CXR as an lucent proximal pouch that can displace the trachea anteriorly on the lateral view. If there is no bowel gas present, you can make the diagnosis of esophageal atresia without tracheoesophageal fistula. If there is bowel gas present, then there must be a fistual to the distal esophagus.

If necessary, a small amount of air (preferred) or barium can be injected through the nasogastric tube, under fluoroscopy, to confirm the diagnosis.

The side the aortic arch is on needs to be determined because the surgeon wishes to do their thoracotomy on the side opposite the aortic arch. If the position of the aortic arch cannot be determined from the CXR, an echocardiogram needs to be performed.

DDX:

References:

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