Esophageal Atresia

Esophageal atresia (EA) is a rare congenital condition (present at birth) in which the esophagus fails to connect to the stomach as it should and ends in a blind pouch instead. Some babies have a hole between the trachea and esophagus, called trachehoesophageal fistula, or TEF. These conditions can occur separately or together and may vary in severity, but babies with EA, TEF, or EA-TEF must undergo surgical repair as soon as possible upon birth. Without treatment, attempting to eat could cause babies to aspirate milk and stomach acid into the trachea and lungs.

Learn more about treatment for EA in the Spring 2014 issue of Healthpoints.

Causes

Causes of esophageal atresia are not fully understood. Some children are born with EA as part of a larger syndrome of heart and or digestive tract disorders, while others have EA without these additional problems.

Signs and Symptoms

Babies born with esophageal atresia have symptoms including:

  • Cyanosis – bluish tint to the skin – during attempted feeding
  • Coughing and choking with attempted feeding
  • Drooling
  • Poor feeding

Diagnosis

Suspected esophageal atresia may be diagnosed through several steps. 

During pregnancy, excess amniotic fluid appearing during ultrasound may signal EA. After birth, EA may be suspected if the baby chokes, coughs, and turns blue when attempting to eat. The baby’s physician will attempt to pass a feeding tube through the baby’s mouth or nose. X-ray imaging will show whether the tube reaches the stomach or if it is coiled in the upper esophagus, indicating EA.

Treatment

Babies with EA, TEF, or EA-TEF must undergo surgical repair, often very soon after birth. Without treatment, attempting to eat could cause babies to aspirate milk and stomach acid into the trachea and lungs. Surgery is successful in the vast majority of children.

The Esophageal Atresia Clinic at NewYork-Presbyterian/Columbia uses a minimally invasive surgical approach for EA repair, using three small incisions in the chest, whenever possible. After surgery, the multidisciplinary team provides long term follow-up in order to prevent and treat acid reflux, which affects many children after EA surgery, and to ensure optimal care as children mature into adulthood.