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Babies born with esophageal atresia (EA) have an esophagus that is divided into two sections and is not connected all the way from the mouth to the stomach.
Sometimes babies with esophageal atresia have another anatomical difference: the upper or lower portion of the esophagus is connected to the trachea in one or two places. (In a normal anatomy, the trachea and the esophagus are two separate tubes.) This condition is called tracheoesophageal fistula (TEF).
When these two conditions are combined, it is common to see the upper part of the esophagus end as a disconnected pouch and the lower part of the esophagus connect to the trachea rather than the stomach.
Currently, researchers are exploring possible genetic links to this congenital (before birth) condition. At one time, it was considered a morbid condition, but most babies today lead a normal life with only modest impact on lifelong health, usually resulting from chronic esophageal reflux. It is a rare condition, seen in one out of 2,500 to 4,500 newborns. The Fetal Center at Riley at IU Health treats approximately 10 to 20 patients a year with this condition.
Although it is usually discovered within the first few hours of life, sometimes it can be predicted by a prenatal ultrasound or magnetic resonance imaging (MRI) that shows polyhydramnios—an excess of amniotic fluid in a mother. When it is not detected until birth, a baby’s difficulty with feeding is one of the first signs of esophageal atresia.
A baby may turn blue, cough or choke while feeding or spit up all or some of his/her formula. Breathing may also be difficult. If there is a connection between the lower esophagus and the trachea, the belly can appear hard and distended from inhaling air into the abdominal cavity.
Diagnosis begins by gently running a tube into a baby’s mouth and down through the stomach. If the tube does not glide smoothly, it suggests the baby may be affected by esophageal atresia with a possibility of tracheoesophageal fistula, as well. Before treatment, doctors verify EA by X-ray and by placing a scope down the patient’s airway to confirm what X-rays show.
Babies affected by esophageal atresia may have other serious problems with the heart, kidneys, anal-rectal anomalies and the anatomy of the arms, vertebrae and ribs. For this reason, doctors evaluate these newborns at birth to rule out the possibility of other conditions.
If other anomalies exist, your child’s multidisciplinary team of specialists will appraise them and decide the order in which they need to be addressed for optimal health. The first years of life can be challenging for children with multiple conditions, and they may be in the hospital for weeks or months.
If an evaluation reveals no other serious conditions, doctors perform surgery within the first 24 to 48 hours of a baby’s life to repair the anatomy. During this time, babies are given intravenous fluids (IV) until they can be fed with breast milk or formula following their recovery from surgery.
Surgery removes the fistula between the trachea and the esophagus and restores a normal anatomy by reconnecting the top of the esophagus to the throat and the bottom of the esophagus to the stomach. When a child’s esophagus has a significant distance between two pieces/ends, surgeons can bring the two ends together, but it is a more complicated process that may involve multiple operations and longer hospital stays.
Parents of children affected by esophageal atresia can expect a hospital stay of 10 days or more, depending on a baby’s progress after surgery and other physical conditions that require treatment or observation.
If your child needs surgery to correct this condition, it will be performed at Riley at IU Health by an expert pediatric surgeon who has experience with these procedures. Most children are successfully treated with one surgery.
Surgery can be performed either by opening the chest or, if a child meets certain criteria for health and size, through a minimally-invasive technique that inserts surgical instruments through small incisions. A temporary feeding tube is often inserted at the end of the surgery to allow nutrition during recovery.
Most children need a breathing tube for several days after the operation and a drainage tube from the chest until at least a week after surgery, when doctors can be sure there are no leaks from the new connection in the esophagus. If things go well, babies may begin to feed by mouth about a week after surgery. They may stay on reflux medicines for at least the first year of life or longer to prevent irritation in the esophagus. Some children take these medicines all through life to control acid reflux.
The most common complication after surgery is a narrowing of the new connection in the esophagus. Forty percent of children encounter this problem, but it can be repaired surgically through an outpatient dilation procedure that requires no new incisions. Twenty percent of children may develop a small leak in the connection, which typically heals on its own over time.
Some infants have difficulty with swallowing during the first few months of life. These babies may be discharged from the hospital with a nasogastric feeding tube that delivers food and medicine to the stomach through the nose, or a gastrostomy tube inserted across the abdominal wall and into the stomach. This is only a temporary solution until a baby acquires the coordination to swallow properly.
In severe cases, the esophagus may later need replaced. Surgeons use pieces of the stomach, colon or small intestine to build a new esophagus.
If your baby is treated for EA, he/she will very likely lead a normal life, but will need routine follow-up care as a child, adolescent and adult to deal with gastroesophageal reflux—a back flow of the stomach’s contents into the esophagus. Lifelong acid reflux makes these children more prone to esophageal cancer, Barrett’s esophagus and other conditions that could require further treatment later in life.
Pediatric surgeons at Riley at IU Health and the Indiana University School of Medicine are part of a Midwest consortium of high-volume pediatric health centers that study EA and TEF to find better ways of treating children. Since this condition is so rare, it is critical for doctors and researchers to combine their experience and insights and work cooperatively to improve outcomes.
Visit the websites below to find support groups, services and learn more about esophageal atresia.
This professional association for pediatric surgeons shares the latest insights about esophageal atresia for the benefit of patients and families.
This U.S. government website offers health information to help parents and caregivers learn about esophageal atresia.
This U.S. National Library of Medicine resource publishes a fact sheet about esophageal atresia.
This National Institute of Health website provides information to the public about esophageal atresia and tracheoesophageal fistula.
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