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Key Features

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  • Presents in the first hours of life

  • Polyhydramnios

  • Excessive drooling and secretions; choking with attempted feeding

  • Unable to pass an orogastric tube to the stomach

  • Characterized by a blind esophageal pouch with or without a fistulous connection between the proximal or distal esophagus and the trachea

  • In 85% of infants, the fistula is between the distal esophagus and the airway

  • Polyhydramnios is common because of high GI obstruction

  • Incidence is approximately 1 in 3000 births

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Clinical Findings

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  • Copious secretions

  • Choking

  • Cyanosis

  • Respiratory distress

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Diagnosis

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  • Confirmed with chest radiograph after careful placement of a nasogastric (NG) tube to the point at which resistance is met

  • The tube will be seen radiographically in the blind pouch

  • If a tracheoesophageal fistula is present to the distal esophagus, gas will be present in the bowel

  • In esophageal atresia without tracheoesophageal fistula, no gas is seen in the bowel

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Treatment

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  • The NG tube in the proximal pouch should be placed on low intermittent suction to drain secretions and prevent aspiration

  • The head of the bed should be elevated to prevent reflux of gastric contents through the distal fistula into the lungs

  • Intravenous glucose and fluids should be provided and oxygen administered as needed

  • Surgery is definitive treatment

    • If the distance is short, the fistula can be ligated and the ends of the esophagus anastomosed

    • If the ends of the esophagus cannot be brought together, the initial surgery is fistula ligation and a feeding gastrostomy

  • Echocardiography should be performed prior to surgery to rule out a right-sided aortic arch (for which a left-sided thoracotomy would be preferred)

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