Esophageal atresia and tracheoesophageal fistula (TEF) are often association with other anomalies. The most common congenital abnormality is the VACTERL association (vertebral defects, imperforate anus, cardiac defects, TE fistula, radial aplasia, limb anomalies). A thorough evaluation for these additional defects, especially cardiac, should be undertaken in these infants. The severity of illness can be mild (e.g., feeding difficulties in a full-term neonate with no respiratory distress), but some patients are critically ill. Severe respiratory failure can result from continuous aspiration of gastric contents via the distal TEF, exacerbated by respiratory distress syndrome as well as massive abdominal distention from filling of the stomach with gas from the TEF.
Esophageal atresia and TEF are seen in five different anatomic configurations (Figure 139-1). The most common type is IIIB (esophageal atresia with a distal TEF). Affected neonates usually present with excessive oral secretions, choking, and cyanosis when attempting oral feeds, and respiratory distress exacerbated by feedings. Diagnosis is made when the chest and abdominal radiograph reveal inability to pass an orogastric tube, which lodges in the blind esophageal pouch, the pouch demonstrated by air contrast or radiopaque dye and radiographic studies and the presence of gas-filled intestines from the distal TEF.
Types of tracheoesophagel fistulas. (Reproduced with permission from Butterworth JF, Mackey DC, Wasnick JD, eds. Morgan & Mikhail’s Clinical Anesthesiology. 5th ed. New York, NY: McGraw-Hill Education, Inc.; 2013: Fig. 42-3.)
Neonates with TEF should be nursed preoperatively prone or in lateral position at an incline of 30° to decrease the risk of pulmonary aspiration. A suction catheter may be placed in the esophageal pouch preoperatively to decrease the accumulation of saliva and reduce the risk of aspiration. It is not uncommon for the neonate to undergo staged repairs of the lesions. A gastrostomy tube to vent the stomach and a central line for total parenteral nutrition may be placed under sedation with local anesthesia or general anesthesia. Thus, allowing the infant to receive long-term nutrition and continue to grow so that the esophageal anastomosis can be performed when the infant is older. When the infant is older, the distance between the esophageal pouch and stomach is decreased.
In the patient whose trachea is not intubated, awake tracheal intubation was classically considered to be the best technique. Infants with TEF may undergo intubation while they are awake with topical anesthesia of the airway. If they are medically stable, either intravenous or inhaled induction of anesthesia, with muscle relaxation, can be achieved after suctioning the upper esophageal pouch and administration of oxygen. Then, an endotracheal tube is passed into the distal trachea, and gentle positive-pressure ventilation is accomplished with careful assessment of effectiveness of ...