Esophageal atresia and tracheoesophageal fistula

Atresia of the esophagus with tracheoesophageal fistula is a congenital anomalies of trachea and esophagus. Esophageal atresia (EA) is caused by a failure of the esophagus to develop as a continuous passage from the throat to the stomach, resulting in obstruction of the infant's normal swallowing route. Tracheoesophageal fistula (TEF) is an abnormal connection between the trachea and the esophagus, often congenital but some time acquired, causing breathing and feeding problems in newborn baby.

The condition causes oral intake to enter the lungs or a large amount of air to enter the stomach, presenting a risk of coughing and choking; severe abdominal distention can occur in newborn baby. Aspiration pneumonia and severe respiratory distress may develop, and death is likely to occur without surgical intervention.

Causes

During normal embryologic development, the gut and respiratory tract originate as a single tube. During the fourth and fifth weeks of gestation, this tube lengthens and separates longitudinally. Two parallel tubes are formed, both of which are joined at the pharynx. Atresia occurs because of the defective growth of endodermal cells. When there is incomplete fusion of the lateral walls of the foregut, there is incomplete closure of the laryngotracheal tube and a fistula is formed, generally at the level of tracheal bifurcation.

Sign and symptoms

• Frothy saliva in the mouth and nose or excessive drooling.
• The "3 C's"- coughing, choking during feeding and unexplained cyanosis.
• Abdominal distention.
• Regurgitation and vomiting
• Increased respiratory distress during and after feeding.

Types of esophageal atresia and tracheoesophageal fistula

There are five types of congenital atresia of esophagus and tracheoesophageal fistula-

1. Type A: Upper and lower segments of esophagus end in blind sac. No fistula present.
2. Type B: Upper segment of esophagus ends in atresia and connects to trachea by fistulous tract and lower esophagus is a blind sac.
3. Type C: Upper segment of esophagus ends in blind pouch; lower segments connects with trachea by small fistulous tract. Most common form occurring in 80% to 95% of infants.
4. Type D: Both upper and lower segments of esophagus connect by fistulous tract to trachea. Infant may aspirate with first feeding.
5. Type E: Esophagus is continuous, but connects by fistulous tract to trachea-known as H-type.

Diagnosis

To confirm the esophageal atresia and tracheoesophageal fistula by taking X-rays of their chest and abdomen by the health care provider.

Management and Treatment

Treatment includes maintenance of a patients airway, prevention of aspiration pneumonia, gastric or blind pouch decompression, supportive therapy and surgical repair of defect.

Preoperative interventions

• The infant may be placed in an incubator or radiant warmer in which humidified oxygen is administered.
• After diagnosis is made, the infant neonate receives no oral feeding (NPO).
• Maintain IV fluids as prescribed.
• Monitor respiratory status closely.
• Suction accumulated secretions from the mouth and pharynx.
• Maintain in a supine upright position, at least 30 degrees upright to facilitate drainage and prevent aspiration of gastric secretions.
• Keep the blind pouch empty of secretions by intermittent or continuous suction as prescribed; monitor its patency closely because clogging from mucus can easily occur.
• If a gastrostomy tube is inserted, it may be left open so that air entering the stomach through the fistula can escape, minimizing the risk of regurgitation of gastric contents into the trachea.
• Broad spectrum antibiotics may be prescribed because of the high risk for aspiration pneumonia.

Postoperative interventions

• Monitor vital signs and respiratory status.
• Inspect the surgical site for signs and symptoms of infection.
• Monitor for anastomotic leaks as evidenced by purulent drainage from the chest tube, increased temperature and increased white blood cell count.
• If a gastrostomy tube is present, it is usually attached to gravity drainage until the infant can tolerated feedings and the anastomosis is healed; then feedings are prescribed.
• Instruct the parents in the techniques of suctioning, gastrostomy tube care and feedings, and skin site care as appropriate.
• Instruct the parents to identify behaviors that indicate the need for suctioning, signs of respiratory distress, and signs of a constricted esophagus.