Please note: This information was current at the time of publication but now may be out of date. This handout provides a general overview and may not apply to everyone. 

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Am Fam Physician. 1999;59(4):919-920

See related article on esophageal atresia and tracheoesophageal fistula.

What is esophageal atresia?

In babies with esophageal atresia, the esophagus doesn't go into the stomach. It just ends in a pouch, so nothing the baby swallows gets into the stomach. (This is how you say these words: ee-sof-ah-gee-all at-tree-see-ah; tray-key-oh-ee-sof-ah-gee-all fist-you-lah.)

What is a tracheoesophageal fistula?

A fistula is a connection between two tubes. The breathing tube that connects the nose and mouth with the lungs is called the trachea. The esophagus is the swallowing tube. The breathing tube and the swallowing tube aren't supposed to be connected. But when a child has a tracheoesophageal fistula, the fistula connects the two tubes. This means that food or milk in the stomach can get into the lungs. This can cause breathing problems and even pneumonia (new-mone-yah).

What causes atresia and fistula?

We don't really know what causes these problems. When the esophagus and the trachea grow in the embryo, they start from the same bit of tissue. Sometimes the tubes don't develop right. We don't think these problems are inherited.

Are these problems common?

About one baby out of 4,000 babies has one or both of these problems. They usually occur together. But sometimes a baby has atresia with no fistula.

How does the doctor know this is what's wrong with my baby?

Most babies with this condition have feeding problems right away. They may spit up a lot or have lots of bubbly mucus in their mouth. If your baby has a fistula, breathing may be hard. If your doctor thinks your baby has one of these conditions, an x-ray can help make the diagnosis.

How is this problem fixed?

Your baby will need surgery to fix the problem. First, the swallowing tube must be connected to the stomach. Then, if a fistula is connecting the esophagus to the trachea, it must be closed. Your child's doctor will decide when to do the surgery. If the baby isn't premature and doesn't have any other problems (like pneumonia or birth defects), the surgery can usually be done when the baby is just a few days old.

How long will my baby be sick?

It depends. If your baby was premature, the recovery time is affected. Another factor is how complicated the operation is. If the surgery is harder, it takes a few days longer for your baby to recover. Your baby will stay in the hospital during this time.

In less complicated cases, your baby may be eating by one week after surgery. Meanwhile, until your baby can swallow milk or formula, your baby will be fed through a vein (this is called an “IV”) or through a stomach tube. Before regular feeding starts, an x-ray can check for holes at the place the surgeon fixed.

Does my baby have any other problems?

Some babies with esophageal atresia have heart problems, kidney problems, stomach and bowel problems, or muscle and bone problems. A physical exam by your doctor, maybe with some other x-ray or ultrasound pictures, will usually show if your baby has other problems. If your baby has other problems, the surgery to fix the swallowing tube might have to wait.

Will my baby have other problems in the future?

Babies born with esophageal atresia sometimes have long-term problems. Probably the most common problem is gastroesophageal reflux disease, or “GERD.” GERD is what doctors call heartburn. Heartburn is a burning feeling caused by acid that comes up from the stomach into the swallowing tube. GERD can usually be treated with medicine.

Another problem is scar tissue. Sometimes scar tissue grows where the esophagus connects to the stomach. This scar tissue can make swallowing hard or painful because the food can't get past the scar tissue easily. Sometimes another surgery is needed to open the scar tissue.

Your child may need more x-rays or endoscopy later. Endoscopy is a way of taking a picture. A narrow tube holding a tiny camera is put into the swallowing tube. The picture helps your doctor see that the surgery is working.

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