Presentation

The neonate with EA typically has frothing and bubbling at the mouth and nose after birth as well as episodes of coughing, cyanosis, and respiratory distress. Feeding exacerbates these symptoms, causes regurgitation, and can precipitate aspiration. Aspiration of gastric contents via a distal fistula causes more damaging pneumonitis than aspiration of pharyngeal secretions from the blind upper pouch. The infant with an isolated TEF in the absence of EA (“H-type” fistula) might come to medical attention later in life with chronic respiratory problems, including refractory bronchospasm and recurrent pneumonias.

Diagnosis

In the setting of early-onset respiratory distress, the inability to pass a nasogastric or orogastric tube in the newborn suggests esophageal atresia. Maternal polyhydramnios might alert the physician to EA. Plain radiography in the evaluation of respiratory distress might reveal a coiled feeding tube in the esophageal pouch and/or an air-distended stomach, indicating the presence of a coexisting TEF (Fig. 311-2). Conversely, pure EA can manifest as an airless scaphoid abdomen. In isolated TEF (H type), an esophagogram with contrast medium injected under pressure can demonstrate the defect (Fig. 311-3). Alternatively, the orifice may be detected at bronchoscopy or when methylene blue dye injected into the endotracheal tube during endoscopy is observed in the esophagus during forced inspiration.

Figure 311-2 Tracheoesophageal fistula. Lateral radiograph demonstrating a nasogastric tube coiled (arrows) in the proximal segment of an atretic esophagus. The distal fistula is suggested by gaseous dilatation of the stomach (S) and small intestine. The arrowhead depicts vertebral fusion, whereas a heart murmur and cardiomegaly suggest the presence of a ventricular septal defect. This patient demonstrated elements of the VATER (vertebral, anorectal, tracheal, esophageal, renal, radial) anomalad.

(From Balfe D, Ling D, Siegel M: The esophagus. In Putman CE, Ravin CE, editors: Textbook of diagnostic imaging, Philadelphia, 1988, WB Saunders.)

Figure 311-3 H-type fistula (arrow) demonstrated in an infant after barium swallow on frontal-oblique chest x-ray. The tracheal aspect of the fistula is characteristically superior to the esophageal aspect. Barium is seen to outline the tracheobronchial tree.

(From Wyllie R, Hyams JS, editors: Pediatric gastrointestinal and liver disease, ed 3, Philadelphia, 2006, Saunders Elsevier, p 299.)

Management

Initially, maintaining a patent airway and preventing aspiration of secretions are paramount. Prone positioning minimizes movement of gastric secretions into a distal fistula, and esophageal suctioning minimizes aspiration from a blind pouch. Endotracheal intubation with mechanical ventilation is to be avoided if possible because it can worsen distention of abdominal viscera. Surgical ligation of the TEF and primary end-to-end anastomosis of the esophagus are performed when feasible. In the premature or otherwise complicated infant, a primary closure may be delayed by temporizing with fistula ligation and gastrostomy tube placement. If the gap between the atretic ends of the esophagus is >3-4 cm, primary repair cannot be done; options include using gastric, jejunal, or colonic segments interposed as a neo-esophagus. Careful search must be undertaken for the common associated cardiac and other anomalies. Thoracoscopic surgical repair is now considered feasible and associated with favorable long-term outcomes.

Outcome

The majority of children with EA and TEF grow up to lead normal lives, but complications are often challenging, particularly during the first 5 yr of life. Complications of surgery include anastomotic leak, refistulization, and anastomotic stricture. Gastroesophageal reflux disease (GERD), resulting from intrinsic abnormalities of esophageal function, often combined with delayed gastric emptying, contributes to management challenges in many cases. GERD contributes significantly to the respiratory disease (reactive airway disease) that often complicates EA and TEF and also worsens the frequent anastomotic strictures after repair of EA.

Many patients have an associated tracheomalacia that improves as the child grows.