Tracheoesophageal Fistula
Michael A. Lopez
Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are congenital malformations that occur approximately in 1:3500 live-born infants.1
It is defined as an interruption in the continuity of the esophagus.
TEFs may or may not be present in patients with EA.
This abnormality is usually due to improper separation of the trachea and esophagus during the 4th week of gestation when the primitive foregut is developed.1
The first successful primary repair of EA was accomplished in 1941 by Cameron Haight.
The high mortality rates associated with EA and TEFs have decreased, thanks to early diagnosis, preoperative management/preparation, and clever surgical techniques.
EPIDEMIOLOGY AND ETIOLOGY
Most cases are sporadic.
35% to 60% of infants with EA will also have other congenital abnormalities.2
˜25% of patients trisomy 18 will be born with EA/TEF.2
Once the diagnosis is made, a thorough physical examination must be performed to determine if any other abnormalities are present.
Approximately 10% to 20% occur with VACTERL or CHARGE (although not criteria for diagnosis).9
Associated abnormalities:2
Cardiovascular (ventricular septal defect, atrial septal defect, tetralogy of fallot) → 20% to 30%
Gastrointestinal (GI) (imperforate anus, duodenal atresia, Meckel diverticulum) → 15% to 25%
Genitourinary → 10% to 20%
Musculoskeletal → 10% to 15%
Craniofacial/Central nervous system → 5% to 10%
Chromosomal abnormalities → 3% to 5%
CLINICAL PRESENTATION
EA may be suspected when absence of gastric bubble, polyhydramnios (earliest symptom, found during second half of pregnancy), and distension of upper esophagus are seen on prenatal ultrasound.
Fetal MRI can confirm diagnosis of atresia or TEF when suspected.5
After birth, the child may present with excessive salivation with choking and cyanotic spells.
DIAGNOSIS
If clinical presentation suggests EA, the diagnosis can be made inserting 10- or 12-F Replogle into the esophagus.
Inability to advance tube further than 10 cm is highly suggestive of EA.
The tube can then be left in place to aspirate secretions, preventing aspiration.
Once the tube is in place, AP and lateral chest radiographs are performed while injecting a small amount of air into the tube.
This will provide the exact location of the atretic esophagus and demonstrate whether or not a TEF is present (Figure 32.1).
If abdominal gas is seen, it is very likely that a distal or H-type TEF is present.6
No air in the GI tract suggests pure atresia.
Injecting barium into the Replogle tube can be performed to confirm diagnosis but is rarely needed.8
The most commonly used systems to classify this disease process are described by Gross (Figure 32.2).
The most frequent type of EA is the blind-ending esophageal pouch with a fistula between the trachea and lower esophagus, classified as C (Gross).
Approximately 85% of patients diagnosed with EA will fall under this classification.7
Type A, pure atresia with TEF, is seen in approximately 7% of cases.
Type B, EA with a TEF between the trachea and upper esophageal pouch, is seen in approximately 2% of cases.
Type D, where both upper and lower esophageal segments communicate with the trachea, is seen in approximately 3% of cases.
Type E, also known as H-type TEF, is a different entity because no EA is present (Figure 32.3).
 Figure 32.1 Esophageal atresia with distal tracheoesophageal fistula. A, Frontal radiograph of the chest and abdomen demonstrates a nasogastric tube within the upper esophageal pouch (arrow). The air in the bowel indicates a distal tracheoesophageal communication. B, Lateral view in another patient shows a dilated upper esophageal pouch (arrows) containing a nasogastric tube. (Reprinted with permission from Siegel MJ, Coley BD. Core Curriculum: Pediatric Imaging. Philadelphia, PA: Lippincott Williams & Wilkins; 2005.) |
MEDICAL AND SURGICAL MANAGEMENT
If no other life-threatening issues are present, surgery should not be delayed.
Although placement of the Replogle tube minimizes secretions, these patients are still at high risk for aspiration.
In premature infants with respiratory distress, early operative intervention is still preferred, as it can be difficult to adequately aerate noncompliant lungs when a majority of air will enter the esophagus in those with TEFs.3
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