Chapter 120

1. Definition. Type C tracheoesophageal (TE) fistula is the most common type of esophageal atresia (85%). The esophagus ends blindly ~10–12 cm from the nares and the distal esophagus communicates with the posterior trachea (distal tracheoesophageal fistula [TEF]). Type A "pure" esophageal atresia has a similar presentation without the distal gastrointestinal air. This implies esophageal atresia without TEF (10% of cases).

2. Pathophysiology. Prime morbidity is pulmonary. Complete esophageal obstruction results in inability of the infant to handle his or her own secretions, producing "excess salivation" and aspiration of pharyngeal contents. More important, the direct communication between the stomach and the tracheobronchial tree via the distal TEF allows the crying newborn to greatly distend the stomach with air; impairment of diaphragmatic excursion promotes basilar atelectasis and subsequent pneumonia. Additionally, the distal TEF permits reflux of gastric secretions directly into the tracheobronchial tree, causing chemical pneumonitis, which may be complicated by bacterial pneumonia.

3. Clinical presentation. The pregnancy may have been complicated by polyhydramnios. After delivery, the infant typically is unable to swallow saliva, which drains from the corners of the mouth and requires frequent suctioning. Attempts at feeding result in prompt regurgitation, coughing, choking, and cyanosis.

4. Diagnosis. It is established by attempting to pass a nasogastric tube and meeting resistance at 10–12 cm from the nares followed by chest radiograph for confirmation. Chest radiograph will show the tube to end or coil in the region of the thoracic inlet. You can improve the sensitivity of the test by insufflating 20–30 mL of air into the tube as the radiograph is being taken. The radiograph should also be examined for possible skeletal anomalies, pulmonary infiltrates, cardiac size and shape, and abdominal bowel gas patterns. The tube in the proximal pouch with the presence of air in the gastrointestinal (GI) tract confirms diagnosis. Careful contrast radiograph of the proximal esophageal pouch can also be performed to delineate the precise length of the proximal pouch and to rule out the rare proximal TEF.

5. Management

1. Preoperative treatment should focus on protecting the lungs by evacuating the proximal esophageal pouch with an indwelling Repogle tube or frequent suctioning and by placing the infant in a relatively upright (45-degree) position to lessen the likelihood of reflux of gastric contents up the distal esophagus into the trachea. Broad-spectrum antibiotics should be administered.

1. Surgical therapy. The steps and timing of surgical therapy must be individualized. Some surgeons perform preliminary gastrostomy to decompress the stomach and provide additional protection against reflux. Single-step ligation of the TEF and esophageal anastomosis via a thoracotomy or thoracoscopy is the preferred intervention if the clinical status of the neonate allows single-stage closure.

1. Type A "pure" esophageal atresia. Surgical management differs somewhat because pure esophageal atresia is associated with a higher incidence of long gaps between the proximal and distal esophageal segments. Delayed surgical correction may be an option to allow growth of the segments and thus permit easier approximation.

1. Definition. Obstruction of the lumen of the duodenum may be complete ...

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