Questions
- 1.
What is a tracheoesophageal fistula?
- 2.
What is the typical presentation of a patient with a tracheoesophageal fistula?
- 3.
What are the preoperative concerns in patients with tracheoesophageal fistula?
- 4.
How are patients with tracheoesophageal fistula managed intraoperatively?
- 5.
What are the postoperative concerns in patients with tracheoesophageal fistula?
A 1-day-old, 3.3-kg neonate presented to the operating room for repair of tracheoesophageal fistula (TEF). The patient was born at 38 weeks gestation by normal vaginal delivery.
1
What is a tracheoesophageal fistula?
TEF is a congenital malformation involving esophageal atresia (EA) combined with a fistula connecting the esophagus to the trachea. These abnormalities can occur in various combinations. The most common classification system of TEFs is Gross’ classification ( Figure 61-1 ). Table 61-1 describes the various types of TEF and their approximate incidence. The most common type, EA with a distal fistula (Gross type C), accounts for approximately 80% of cases.
| Type | Incidence (%) | Description |
|---|---|---|
| A | 10 | EA without fistula |
| B | 1 | EA with proximal fistula |
| C | 80 | EA with distal fistula |
| D | 2 | “K” type, EA with proximal and distal fistula |
| E | 7 | “H” type, fistula without atresia |
The incidence of EA and TEF is approximately 1 in 3000 births. Embryologically, the trachea and esophagus both originate from the ventral diverticulum of the primitive foregut. They normally become separated by the esophagotracheal septum. Because the trachea is situated anterior to the esophagus, the fistula is located on the posterior aspect of the trachea and usually just proximal to the carina.
2
What is the typical presentation of a patient with a tracheoesophageal fistula?
In contrast to other congenital anomalies, it is difficult to diagnose TEF in utero. Polyhydramnios is often present because EA prevents the fetus from swallowing amniotic fluid. At delivery, an orogastric tube cannot be passed into the stomach. Typically, the orogastric tube passes only to a distance of approximately 10 cm from the gums. In most cases, the diagnosis is initially suspected at the first feeding, when the neonate presents with coughing, choking, and cyanosis (the “three Cs”). Excessive salivation and respiratory distress can also occur.
Confirmation of the diagnosis is made radiographically when a radiopaque orogastric catheter is seen curled in the proximal esophageal pouch. The presence of air in the stomach and intestines on radiography signifies the presence of a fistula between the trachea and distal esophagus (Gross types C and D). Absence of air in the stomach occurs with cases of EA without a distal fistula (Gross types A and B). The “H”-type fistula (TEF without atresia, Gross type E) usually manifests later in life, most commonly with choking during feedings and recurrent pneumonitis.
3
What are the preoperative concerns in patients with tracheoesophageal fistula?
There is a 30%–50% incidence of associated anomalies in infants with EA and TEF. Particular combinations have been described, termed VATER association or, more exactly, VACTERL association ( Table 61-2 ).
| V | Vertebral (vertebral malformations, hemivertebrae) |
| A | Anal (imperforate anus, also midgut malrotation, Meckel diverticulum) |
| C | Cardiac (VSD, PDA, TOF, ASD, right-sided aortic arch, coarctation of aorta) |
| T | Trachea (TEF) |
| E | Esophagus (EA) |
| R | Renal (renal agenesis, hydronephrosis, renal lobulation) |
| L | Limb (radial aplasia, polydactyly, wrist anomalies) |
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