54

or without feeding, respiratory distress, inability to swallow, and inability to pass a tube through the mouth or nose into the stomach. If a distal fistula is present, the abdomen distends as inspired air passes through the fistula into the stomach. Pulmonary compromise can be significant as gastric fluid passes upward through the TEF and spills into the trachea and lungs, leading to chemical pneumonitis. As the abdomen distends with air, the diaphragm elevates and the pulmonary status worsens. Aspiration of saliva from the upper pouch into the trachea further exacerbates the pulmonary compromise. The larger the distal TEF, the greater are the intestinal distention and subsequent respiratory compromise. In addition, if an associated distal congenital obstruction of the intestine is present, such as duodenal atresia or imperforate anus, then the proximal intestinal distention is even greater and the respiratory compromise is worse.

The diagnosis of EA can be confirmed by passing a firm catheter through the mouth into the esophagus to the point where resistance is met. A few milliliters of air can be injected through the tube and used as a contrast agent to distend the upper esophageal pouch as a frontal and lateral film is obtained. If necessary, 0.5 to 1.0 ml of diluted barium can be used as a contrast agent and injected into the upper pouch to confirm the diagnosis. Under carefully controlled fluorography, barium may also be used to detect a proximal TEF; however, under the usual circumstance of a portable film performed in the neonatal unit, barium identified in the tracheobronchial tree more likely represents contrast material aspirated through the larynx rather than through a proximal TEF. Nevertheless, a very small upper blind pouch suggests the presence of a proximal TEF. Air in the stomach and bowel confirms the presence of a distal TEF. The absence of air in the abdomen typically represents isolated EA without TEF ( Fig. 3-2 ). The diagnosis of TEF without EA is more difficult and requires a high index of suspicion based on clinical symptoms. The diagnosis can be made with barium esophagography with the patient in the prone position; however, bronchoscopy and esophagoscopy are often required to confirm the diagnosis.

Because the incidence of other congenital defects associated with EA is between 50 and 70%, clinical evidence of these other anomalies should be considered in the diagnostic evaluation for EA. In addition to a physical examination focused to evaluate for known associated defects, such as those of the VACTERL and CHARGE association, additional testing usually includes echocardiography, renal sonography, and chromosomal analysis. In fact, it is not unusual for the finding of an anorectal malformation, for instance, to proceed the clinical signs and symptoms of EA.

Preoperative Treatment

Pneumonitis is the most critical problem that requires attention in the immediate preoperative period, and it results from aspiration of the pharyngeal contents and from reflux of the gastric juice through the TEF into the tracheobronchial tree. Preoperative treatment involves both the prevention of further aspiration and reflux and

Figure 3-2 Gasless abdomen confirms the diagnosis of isolated esophageal atresia.

Figure 3-2 Gasless abdomen confirms the diagnosis of isolated esophageal atresia.

Figure 3-3 The infant is kept in the upright position until definitive surgery is carried out. A gastrostomy tube is not routinely placed preoperatively.

Figure 3-5 Wet cotton-tipped applicators aid in the extrapleural dissection.

Figure 3-6 The divided azygos vein with the tracheoesophageal fistula under it is seen in this diagram.

Figure 3-7 The tracheal end of the fistula is closed with interrupted 5-0 or 6-0 silk or Prolene.
Figure 3-8 The feasibility of the primary anastomosis between the two esophageal segments is assessed.

Figure 3-9 The anastomosis is performed as a single layer with the knots on the inside posteriorly (A) and on the outside anteriorly (B). The anterior row is performed over a tube (C).

Figure 3-11 The stomach is mobilized into the chest (A), a proximal esophagomyotomy is performed (B), and the anastomosis (C and D) is performed as described in Figure 3-14 A and B.

Figure 3-10 This diagram shows the use of a proximal esophagomyotomy to gain additional length.

Figure 3-11 The stomach is mobilized into the chest (A), a proximal esophagomyotomy is performed (B), and the anastomosis (C and D) is performed as described in Figure 3-14 A and B.

Figure 3-12 A cervical approach is used for an isolated tracheoesophageal fistula. This shows a left cervical approach, which can be used, although I prefer a right cervical incision.

Figure 3-13 The fistula is divided and both sides are closed with interrupted sutures of 5-0 or 6-0 silk.

Figure 3-14 Repair of a type III LTEC. A, Stabilization of a bifurcated endotracheal tube is performed at bronchoscopy using a loop passed through a tracheotomy, which draws the endotracheal tube forward. B, A cervical and thoracic approach allows retropleural exposure of the cleft. A longitudinal incision is made in the right tracheoesophageal groove below the tracheal rings. The incision is extended inferiorly and across the esophagus and up the left side, leaving approximately 1 cm of esophageal wall attached to the trachea to allow adequate tissue to close the trachea. C, The trachea has been closed with interrupted sutures, and the esophagus is closed in a running fashion up to the thoracic inlet. Closure of the laryngeal portion of the cleft and the lateral pharyngeal wall is not yet accomplished. (From Donahoe PK, and Gee PE:

Complete laryngotracheal cleft: management and repair. J Pediatr Surg 19:143, 1984. Used with permission.)

SlifM) in KocTieosicn*

Figure 3-14 Repair of a type III LTEC. A, Stabilization of a bifurcated endotracheal tube is performed at bronchoscopy using a loop passed through a tracheotomy, which draws the endotracheal tube forward. B, A cervical and thoracic approach allows retropleural exposure of the cleft. A longitudinal incision is made in the right tracheoesophageal groove below the tracheal rings. The incision is extended inferiorly and across the esophagus and up the left side, leaving approximately 1 cm of esophageal wall attached to the trachea to allow adequate tissue to close the trachea. C, The trachea has been closed with interrupted sutures, and the esophagus is closed in a running fashion up to the thoracic inlet. Closure of the laryngeal portion of the cleft and the lateral pharyngeal wall is not yet accomplished. (From Donahoe PK, and Gee PE:

Complete laryngotracheal cleft: management and repair. J Pediatr Surg 19:143, 1984. Used with permission.)

Tracheoesophageal Groove Anatomy
Figure 3-15 Typical anatomy in double aortic arch. Here, the anterior arch is larger, but in some Instances the posterior arch Is more important.
Figure 3-16 Typical anatomy in aberrant right subclavian artery.
Figure 3-17 Barium esophagogram reveals typical lateral indentations.
References

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