Tracheoesophageal Fistula and Esophageal Atresia
Overview
Tracheoesophageal fistula
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 problem is also called TE fistula or TEF. It can happen in 1 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.
Esophageal atresia
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 2 parts instead of 1. One part
connects to the throat. The other part connects to the stomach. But the 2 parts don’t
connect to each other.
Since the esophagus is in 2
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.
Causes
As a baby is growing during
pregnancy, organ systems are forming. The trachea and the esophagus start forming
as 1
tube. At 4 to 8 weeks of pregnancy, a wall forms between the esophagus and trachea.
This
separates them into 2 tubes. TE fistula and esophageal atresia happen when this wall
doesn’t form as it should.
Risk Factors
Having the following issues can
raise your baby’s risk for these conditions:
- Trisomy 13, 18, or 21. —These are
genetic conditions that happen when there is an extra copy of a chromosome
- 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), kidney (renal), and limb issues
Up to half of babies with TE
fistula or esophageal atresia also have another birth defect.
Symptoms
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
- Vomiting
- Blue color of the skin, especially
when the baby is feeding
- Trouble breathing
- 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.
Diagnosis
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.
Treatment
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 1 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 1 surgery. This depends on how close the 2 tubes are to each
other. Your baby's surgeon and healthcare team will decide when your baby should have
the surgeries.
Complications
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 2 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
TE fistula
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.
Esophageal atresia
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 and 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 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 to Call a Healthcare Provider
Before surgery. Call your
child’s healthcare provider right away if your child breathes liquid into their lungs.
Call
911 if your child has trouble
breathing.
After surgery. Call your
child’s healthcare provider if your child has trouble swallowing, persistent vomiting,
or isn't gaining weight.
Key Points
- 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 1 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.
Next Steps
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.