After a previous surgery in her home state of New York to remove a tumor resulted in her inability to speak or swallow, Nancy Falconeri came to Massachusetts General Hospital for colon interposition (complex esophageal reconstruction) as well as voice box reconstruction.
Tracheoesophageal Fistula and Esophageal Atresia
Tracheoesophageal fistula is a connection between the esophagus and the trachea. The esophagus is the tube that connects the throat to the stomach. The trachea is the tube that connects the throat to the windpipe and lungs. Normally, the esophagus and trachea are 2 tubes that are not connected. This issue is also called TE fistula or TEF. It can happen in one or more places.
The Airway and Tracheal Surgery Program in the Division of Thoracic Surgery at Massachusetts General Hospital is a national leader in specialized care for a wide range of airway and tracheal conditions.
The Pediatric Surgery service at MassGeneral Hospital for Children is an international referral center for the complete spectrum of general and thoracic pediatric and neonatal surgical services.
Tracheoesophageal Fistula and Esophageal Atresia
What are tracheoesophageal fistula and esophageal atresia?
Tracheoesophageal fistula is a connection between the esophagus and the trachea. The esophagus is the tube that connects the throat to the stomach. The trachea is the tube that connects the throat to the windpipe and lungs. Normally, the esophagus and trachea are two tubes that are not connected. This problem is also called TE fistula or TEF. It can happen in one or more places.
TE fistula is a birth defect. This means it’s a problem you are born with. It happened when your baby was forming during pregnancy.
When a baby with a TE fistula swallows, liquid can pass through the connection between the esophagus and trachea. When this happens, liquid gets into your baby's lungs. This can cause pneumonia and other problems.
TE fistula often happens with another birth defect called esophageal atresia. This means your baby’s esophagus doesn’t form well during pregnancy. It forms in two parts instead of one. One part connects to the throat. The other part connects to the stomach. But the two parts don’t connect to each other.
Since the esophagus is in two parts, liquid that your baby swallows doesn’t pass as it should through the esophagus and reach the stomach. This means your baby can't digest milk or other fluids.
What causes TE fistula and esophageal atresia?
As a baby is growing during pregnancy, organ systems are forming. The trachea and the esophagus start forming as one tube. At 4 to 8 weeks of pregnancy, a wall forms between the esophagus and trachea. This separates them into two tubes. TE fistula and esophageal atresia happen when this wall doesn’t form as it should.
Who is risk for TE fistula or esophageal atresia?
Having the following issues can raise your baby’s risk for these conditions:
Trisomy 13, 18, or 21
Other digestive tract problems, such as diaphragmatic hernia, duodenal atresia, or imperforate anus
Heart problems, such as ventricular septal defect, tetralogy of Fallot, or patent ductus arteriosus
Kidney and urinary tract problems, such as a horseshoe or polycystic kidney, absent kidney, or hypospadias
Muscular or skeletal problems
VACTERL syndrome, which involves spinal, anal, heart (cardiac), TE fistula, kidney (renal), and limb issues
Up to one half of babies with TE fistula or esophageal atresia also have another birth defect.
What are the symptoms of TE fistula or esophageal atresia?
Your child’s healthcare provider will often spot symptoms soon after your baby is born. Symptoms can happen a bit differently in each child. They can include:
Frothy, white bubbles in the mouth
Coughing or choking when feeding
Blue color of the skin, especially when the baby is feeding
Very round, full stomach
Your baby may also have other birth defects.
The symptoms of these conditions may look like symptoms of other health problems. Your child must be diagnosed with this condition by their healthcare provider.
How are a TE fistula or esophageal atresia diagnosed?
Your child’s healthcare provider will ask about your child’s health history. They will also give your child an exam.
Your child may need an X-ray of their chest and belly. For this test, the healthcare provider will put a small tube into your child's mouth or nose. It will be guided into the esophagus. With esophageal atresia, the tube often can't go very far into the esophagus. The tube's position can be seen with the X-ray. This can help your child’s healthcare provider diagnose the condition.
How are a TE fistula or esophageal atresia treated?
Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. If your child has one or both of these issues, they will need surgery.
With a TE fistula, the connection between the esophagus and trachea is closed in surgery.
Sometimes children with esophageal atresia need more than one surgery. This depends on how close the two tubes are to each other. Your baby's surgeon and healthcare team will decide when your baby should have the surgeries.
What are possible complications of a TE fistula or esophageal atresia?
When a baby with a TE fistula swallows, liquid can pass through the connection between the esophagus and trachea. This can cause liquid to get into your baby's lungs. This can lead to pneumonia and other problems.
With esophageal atresia, the esophagus is in two parts. Liquid that your baby swallows doesn’t pass normally through the esophagus and reach the stomach. Your baby can't digest milk or other fluids.
Living with a TE fistula or esophageal atresia
Children who had surgery for a TE fistula may develop scar tissue. This can make swallowing tricky. Your child may need another surgery to open the scar tissue.
Some children with esophageal atresia have long-term problems. It may be hard for them to swallow foods and liquids. This may be because of the following:
Trouble with the movement of foods and liquids down the esophagus (peristalsis).
Scarring that can happen after surgery as the wounds heal. This can partially block the passage of foods.
Some children may need a special test to widen a narrowed esophagus. Your child’s healthcare provider may do this while your child is under general anesthesia. Other children may need another surgery. Your child’s surgeon will open up the esophagus so food can pass to the stomach.
About one half of children who had surgery for esophageal atresia will develop gastrointestinal reflux disease (GERD). GERD causes acid to move up into the esophagus from the stomach. This causes a burning or painful feeling called heartburn. GERD can often be managed with medicines.
Work with your child’s healthcare team to create a care plan for your child. This will help your child grow and develop well.
When should I call my child's healthcare provider?
Before surgery: Call your child’s healthcare provider right away if your child has trouble breathing or breathes liquid into their lungs.
After surgery: Call your child’s healthcare provider if your child has trouble swallowing or persistent vomiting, or isn't gaining weight.
Key points about TE fistula and esophageal atresia
TE fistula is an abnormal connection between the esophagus and the trachea. Esophageal atresia happens when the esophagus has 2 segments. These parts don’t connect to each other.
Your child’s healthcare provider will often spot symptoms of these issues soon after your baby is born.
Both of these problems can happen together. If your child has one or both issues, they will need surgery.
Even after surgery, children with these conditions may have trouble swallowing well.
It’s important to work with your child’s healthcare team to create a care plan for your child.
Tips to help you get the most from a visit to your child’s healthcare provider:
Know the reason for the visit and what you want to happen.
Before your visit, write down questions you want answered.
At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
Ask if your child’s condition can be treated in other ways.
Know why a test or procedure is recommended and what the results could mean.
Know what to expect if your child does not take the medicine or have the test or procedure.
If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.
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