UBP 1.7 (Short Form): Pediatrics – Tracheoesophageal Fistula

Question 1

What are you going to do to aid in this resuscitation?

(A pregnant woman with polyhydramnios delivers a 33 weeks gestational age infant, weighing 2,500 grams, with APGAR scores of 6 at 1 minute, and 8 at 5 minutes. During resuscitation, the infant exhibits copious oral secretions and progressive gastric distention as the nurse provides positive pressure mask ventilation. The resuscitation team unsuccessfully attempts to advance an orogastric tube more than 7 centimeters, and ventilation is becoming more difficult.)

Answer 1

Copious oral secretions, the inability to pass an orogastric tube into the stomach, and progressive gastric distention with positive pressure ventilation

are all consistent with the presence of esophageal atresia and a tracheoesophageal fistula (TEF).

1. Therefore, I would first – tell the nurse to discontinue positive pressure ventilation to avoid further gastric distention, which could lead to impaired ventilation, reduced venous return (leading to reduced cardiac preload), and cardiopulmonary arrest.
2. If the gastric distention remained significant, ventilation of the neonate did not improve, and/or the baby developed cardiovascular instability, I would – call for a surgeon to perform gastrostomy to decompress the stomach.
3. In the interim, I would consider – placing an ETT beyond the fistula
   
   • (may need to advance into the mainstem bronchus) so that positive pressure ventilation could be delivered while minimizing further gastric distention.
4. I would also place the neonate in the head-up position and initiate continuous suction of the upper esophageal pouch to prevent aspiration of pharyngeal secretions.
5. Finally, I would – consider a chest x-ray to identify coiling of the orogastric tube in the upper esophageal pouch, which confirms the diagnosis
   
   • (air in the stomach is another associated radiographic finding)

Question 2

Following gastrostomy, the endotracheal tube is accidentally removed. Would you replace it?

(A pregnant woman with polyhydramnios delivers a 33 weeks gestational age infant, weighing 2,500 grams, with APGAR scores of 6 at 1 minute, and 8 at 5 minutes. During resuscitation, the infant exhibits copious oral secretions and progressive gastric distention as the nurse provides positive pressure mask ventilation. The resuscitation team unsuccessfully attempts to advance an orogastric tube more than 7 centimeters, and ventilation is becoming more difficult.)

Answer 2

If following decompression of the stomach, the neonate exhibited no signs of respiratory distress syndrome (tachypnea, tachycardia, intercostal retractions, bilateral rales, and cyanosis due to inadequate surfactant) and I believed she could manage adequate spontaneous ventilation,

I would NOT replace the endotracheal tube.

If possible, endotracheal intubation and positive-pressure mask ventilation are avoided in these patients to prevent exacerbating gastric distention.

However, if assisted ventilation became necessary (atelectasis, aspiration, and prematurity can lead to poor lung compliance), I would mask ventilate with as little positive pressure as possible, and use the gastrostomy to decompress the stomach as necessary.

If ventilation were still inadequate due to preferential flow through the fistula (the path of least resistance), I would consider re-intubation or passing a Fogarty catheter retrograde through the gastrostomy to occlude the esophagus from below.

Question 3

After decompression of the stomach, the neonate is stabilized and breathing spontaneously.

What are the types of tracheoesophageal fistula (TEF) and which one is the most common?

(A pregnant woman with polyhydramnios delivers a 33 weeks gestational age infant, weighing 2,500 grams, with APGAR scores of 6 at 1 minute, and 8 at 5 minutes. During resuscitation, the infant exhibits copious oral secretions and progressive gastric distention as the nurse provides positive pressure mask ventilation. The resuscitation team unsuccessfully attempts to advance an orogastric tube more than 7 centimeters, and ventilation is becoming more difficult.)

Answer 3

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

According to the Gross and Vogt classification:

Type A is esophoageal 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 4

Does her prematurity concern you?

(A pregnant woman with polyhydramnios delivers a 33 weeks gestational age infant, weighing 2,500 grams, with APGAR scores of 6 at 1 minute, and 8 at 5 minutes. During resuscitation, the infant exhibits copious oral secretions and progressive gastric distention as the nurse provides positive pressure mask ventilation. The resuscitation team unsuccessfully attempts to advance an orogastric tube more than 7 centimeters, and ventilation is becoming more difficult.)

Answer 4

It does concern me because prematurity is associated with multiple medical problems that may complicate perioperative care, such as –

• respiratory distress syndrome (hyaline membrane disease),
• persistent pulmonary hypertension of the newborn (PPHN),
• apneic spells,
• bronchopulmonary dysplasia,
• necrotizing enterocolitis,
• retinopathy of prematurity, and
• intraventricular hemorrhage.

In addition, her physiologic prematurity is associated with –

• reduced renal function,
• impaired glucose regulation,
• immature hepatic function, and
• increased susceptibility to hypothermia.

These issues are added to my concerns that are specifically associated with esophageal atresia and TEF, such as –

• aspiration and
• associated congenital abnormalities, in particular the VACTERL association.

Extra Q: Explain to me why neonates develop persistent pulmonary hypertension (PPHN). – Good discussion at ~13:45.

Question 5

What are the congenital abnormalities associated with esophageal atresia (EA) and TEF?

(A pregnant woman with polyhydramnios delivers a 33 weeks gestational age infant, weighing 2,500 grams, with APGAR scores of 6 at 1 minute, and 8 at 5 minutes. During resuscitation, the infant exhibits copious oral secretions and progressive gastric distention as the nurse provides positive pressure mask ventilation. The resuscitation team unsuccessfully attempts to advance an orogastric tube more than 7 centimeters, and ventilation is becoming more difficult.)

Answer 5

50% of neonates with esopahgeal 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.

Question 6

How would you evaluate this patient preoperatively?

(A pregnant woman with polyhydramnios delivers a 33 weeks gestational age infant, weighing 2,500 grams, with APGAR scores of 6 at 1 minute, and 8 at 5 minutes. During resuscitation, the infant exhibits copious oral secretions and progressive gastric distention as the nurse provides positive pressure mask ventilation. The resuscitation team unsuccessfully attempts to advance an orogastric tube more than 7 centimeters, and ventilation is becoming more difficult.)

Answer 6

I would perform a history and physical to identify the presence and severity of –

• aspiration pneumonia;
• respiratory distress syndrome;
• hypovolemia from the lack of communication between upper esophagus and stomach; and
• renal, cardiac, and vertebral anomalies.

To this same end, I would order –

• a CXR and ABG to determine the extent of any pulmonary disease (atelectasis, infiltrates, pleural effusion);
• an echocardiogram to identify and evaluate any significant cardiac defects;
• renal ultrasound to identify any renal abnormalities;
• spinal films to identify any vertebral anomalies; and
• electrolyte, hemoglobin, and hematocrit levels.

My primary goals are –

• to optimize the neonate’s pulmonary and cardiac function;
• avoid aspiration;
• ensure adequate hydration;
• establish a plan for intubation, ventilation, and extubation; and
• anticipate perioperative complications.