ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL FISTULA
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 implies esophageal atresia without TEF (10% of cases). It has a similar presentation without the distal gastrointestinal air.
Because of the esophageal obstruction, the infant is unable to handle secretions, with subsequent “excess salivation” and aspiration of pharyngeal contents. Communication between the tracheobronchial tree and the distal fistula allows the crying newborn to greatly distend the stomach with air. This impairment of diaphragmatic excursion can promote basilar atelectasis and respiratory distress. Additionally, the distal TEF permits reflux of gastric secretions directly into the tracheobronchial tree, causing chemical pneumonitis or pneumonia.
III. CLINICAL PRESENTATION
Pregnancy can be complicated by polyhydramnios. Typically, the newborn is unable to manage oral secretions and requires frequent suctioning. Attempted feedings result in prompt regurgitation, coughing, choking, and cyanosis.
A nasogastric tube cannot be passed beyond 10–12 cm from the nares. Chest radiograph shows the tube ending in the region of the thoracic inlet. The upper pouch can be better visualized by insufflating 20–30 mL of air into the tube as the radiograph is being taken. Air in the gastrointestinal tract confirms presence of a distal fistula. The radiograph should also be examined for skeletal anomalies, pulmonary infiltrates, cardiac size and shape, and abdominal bowel gas patterns. Appropriate evaluation for VATER/VACTERL association is necessary
Preoperative treatment should focus on protecting the lungs by evacuating the proximal esophageal pouch with an indwelling Replogle tube or frequent suctioning. Placing the baby in an upright (45-degree) position lessens the likelihood of reflux of gastric contents up the distal esophagus into the trachea. Broad-spectrum antibiotics should be administered. An echocardiogram should be obtained to assess for cardiac and aortic arch anomalies.
Surgical therapy. The steps and timing of surgical therapy must be individualized to the baby's anatomy. Some surgeons perform preliminary gastrostomy to decompress the stomach and provide additional protection against reflux. Single-stage TEF ligation and esophageal anastomosis via thoracotomy or thoracoscopy is the preferred intervention if allowed by the neonate's clinical status.
Type A esophageal atresia is associated with a higher incidence of a long gap between the proximal and distal esophageal segments. Delayed surgical correction allows growth of the segments and permits primary anastomosis. Enteral nutrition is provided via gastrostomy.
Obstruction of the lumen of the duodenum may be complete, partial, pre- or postampullary, and caused by either intrinsic or extrinsic problems.