Extra Topic 1.8 -- Tracheoesophageal Fistula

Question 1

What are the different types of tracheoesophageal fistulae (TEF)? Which is most common?

(A 2-day-old, 3100 g, male infant born at 34 weeks gestation, presents for repair of a tracheoesophageal fistula. VS: RR = 25, HR = 170, BP = 74/40 mmHg, SaO2 = 98%)

Answer 1

Tracheoesophageal fistulae are typically classified into five different types, A-E.

According to the Gross and Vogt classification:

• Type A is esophageal atresia with no fistula;
• Type B is esophageal atresia with a communication between the upper segment and the trachea;
• Type C is esophageal atresia with a blind upper pouch and lower segment tracheal fistula;
• Type D is esophageal atresia with a proximal and distal fistula communicating with the trachea; and
• Type E (also called an H-type fistula) is a pure tracheoesophageal fistula without esophageal atresia.

Of the various types, Type C is the most common, representing 80% to 90% of cases.

Question 2

What are the associated congenital abnormalities?

(A 2-day-old, 3100 g, male infant born at 34 weeks gestation, presents for repair of a tracheoesophageal fistula. VS: RR = 25, HR = 170, BP = 74/40 mmHg, SaO2 = 98%)

Answer 2

50% of neonates with esophageal atresia and TEF have associated congenital anomalies, often described by the VACTERL association (formerly the VATER association).

The VACTERL association includes vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula, radial and renal dysplasia, and limb anomalies.

Importantly, 20% of these neonates have a major cardiovascular abnormality such as coarctation of the aorta, atrial or ventricular septal defect, and tetralogy of Fallot.

• In other words… In a patient with TEF, there is a 50% chance there is some form of anomaly in this VACTERL association.*
• There is a 20% chance of a significant cardiac anomaly.*

Question 3

What are your primary concerns during induction and intubation?

(A 2-day-old, 3100 g, male infant born at 34 weeks gestation, presents for repair of a tracheoesophageal fistula. VS: RR = 25, HR = 170, BP = 74/40 mmHg, SaO2 = 98%)

Answer 3

I have several concerns, including:

1. aspiration, due to the tracheoesophageal fistula (gastric contents through fistula, oral secretions from upper esophageal segment);
2. gastric distention, secondary to positive pressure ventilation or improper ETT placement;
3. difficult intubation, if intubating an awake and vigorous neonate;
4. hypotension, secondary to hypovolemia from an inability to feed; and
5. inadequate ventilation, secondary to decreased pulmonary compliance (i.e. prematurity, aspiration pneumonitis), gastric distention, or improper ETT placement.

Question 4

How will you stop air from entering the stomach during positive pressure ventilation?

(A 2-day-old, 3100 g, male infant born at 34 weeks gestation, presents for repair of a tracheoesophageal fistula. VS: RR = 25, HR = 170, BP = 74/40 mmHg, SaO2 = 98%)

Answer 4

Proper endotracheal tube placement would help to prevent airflow into the stomach.

Ideally the tip of the ETT should be placed distal to the fistula and proximal to the carina, allowing positive pressure ventilation of both lungs without excessive airflow through the fistula (this may be impossible if the fistula is too close to the carina).

One method of properly positioning the ETT is to advance the ETT into the right main-stem bronchus and then slowly withdraw it until breath sounds are heard through a stethoscope placed in the left axilla.

If excessive airflow through the fistula occurs due to poor pulmonary compliance or proximity of the fistula to the carina, consideration should be given to performing a gastrostomy to decompress the stomach.

If I felt I was unable to provide adequate positive pressure ventilation despite the placement of a gastrostomy tube (excessive flow through the fistula, usually due to decreased pulmonary compliance), I would consider repositioning the ETT or passing a Fogarty catheter retrograde through the gastrostomy to occlude the esophagus from below.