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A tracheoesophageal fistula (TEF) is an abnormal connection (fistula)

between the esophagus and the trachea. TEF is a common congenital
abnormality. In most cases, the esophagus is discontinuous (an
esophageal atresia), causing immediate feeding difficulties.

Esophageal atresia is a congenital medical condition which affects the

alimentary tract. It causes the esophagus to end in a blind-ended pouch
rather than connecting normally to the stomach.

Esophageal atresia (EA) is a variety of congenital anatomic defects

that are caused by an abnormal embryological development of the
esophagus. Anatomically characterized by a congenital obstruction of
the light of the esophagus with interrupted the continuity of the
esophageal wall. The esophagus is divided into two blind pouches, an
upper and lower, which may or may not communicate with the
tracheobronchial tree through fistulous tracts called
Tracheoesophageal Fistula (TEF).

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The trachea, or windpipe, carries air to the lungs. The esophagus
carries food to the stomach. Sometimes during development these two
tubes do not separate completely but remain connected by a short
passage. When this happens, air enters the gastrointestinal system,
causing the bowels to distend, and mucus is breathed into the lungs
causing aspiration pneumonia and breathing problems.

There are three main types of TEF. In 85 to 90 percent of

tracheoesophageal fistulas, the top part of the esophagus ends in a
blind sac, and the lower part inserts into the trachea. In the second
type, the upper part of the esophagus is connected directly to the
trachea, while the lower part ends in a pouch. In a rare type of fistula
called an H type, both the esophagus and trachea are complete, but
they are connected by a small passageway. This is the most difficult
type of tracheoesophageal fistula to diagnose, because both eating and
breathing are possible. TEFs often occur in babies with additional birth
defects.

Congenital esophageal atresia (EA) represents a failure of the

esophagus to develop as a continuous passage. Instead, it ends as a
blind pouch. Tracheoesophageal fistula (TEF) represents an abnormal
opening between the trachea and esophagus. EA and TEF can occur
separately or together. EA and TEF are diagnosed in the ICU at birth
and treated immediately.

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TEFs occur in about one of every 3,000 live births. They are slightly more common
in boys than in girls. Some studies suggest that the occurrence of TEFs increases
with the age of the mother.

 
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A "Long Gap", ´Pureµ or Esophageal Atresia without fistula. Type A Esophageal Atresia is
´Isolatedµ Esophageal characterized by the presence of a ´gapµ between the two
Atresia esophageal blind pouches.

B Esophageal Atresia Esophageal Atresia where the upper esophageal pouch connects
with proximal TEF abnormally to the trachea.This abnormal connection is called a
fistula. The lower esophageal pouch ends blindly.

C Esophageal Atresia Esophageal Atresia where the lower esophageal pouch makes an
with distal TEF abnormal connection with the trachea.This abnormal connection is
called a fistula. The upper esophageal pouch ends blindly.

D Esophageal Atresia Esophageal Atresia where the upper and lower esophageal pouches
with both proximal make and abnormal connection with the trachea in two separate,
and distal TEFs isolated places. The upper esophageal atresia also still ends in a
blind pouch.

E TEF only with no This rare form finds the esophagus fully intact and capable of its
Esophageal Atresia normal functions, however, there is an abnormal connection
and there is a blind between the esophagus and the trachea.This abnormal connection
pouch over there is called a fistula.

F Esophageal Stenosis Also known as an Esophageal Stricture.This rare form also finds
the esophagus fully intact and connected to the stomach,
however, the esophagus gradually narrows, causing food and saliva
to become ´caughtµ in the esophagus.On occasion, Type F can go
undiagnosed until adulthood.




The presence of EA is suspected in an infant with excessive salivation
(drooling) and in a newborn with drooling that is frequently
accompanied by choking, coughing and sneezing. When fed, these
infants swallow normally but begin to cough and struggle as the fluid
returns through the nose and mouth. The infant may become cyanotic
(turn bluish due to lack of oxygen) and may stop breathing as the
overflow of fluid from the blind pouch is aspirated (sucked into) the
trachea. The cyanosis is a result of laryngospasm (a protective
mechanism that the body has to prevent aspiration into the trachea).
Over time respiratory distress will develop.

 

Tracheoesophageal fistulas arise as a developmental abnormality. This

birth defect arises in the fourth fetal week, when the trachea and
esophagus should begin to separate from each other. It can be
associated with disorders of the tracheoesophageal septum

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Diagnosis that the esophagus is interrupted is confirmed by the
inability to insert a nasogastric suction tube into the stomach. The
exact type and location of the fistula can be determined using a
radiopaque catheter, which allows pictures to be taken of the
esophagus. X rays may show air in the bowels. Endoscopy often fails to
locate the fistula if it is small.

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This test is an x-ray of the throat and esophagus .This test may also
be called a barium esophagram. The person is made to brink barium.
Barium helps the esophagus and stomach show up better on x-rays.

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This test may be done to look inside theairways and lungs. Caregivers
use a bronchoscope to do this test. It is a long tube with a light and
magnifying glass on the end. The scope goes into the mouth and into
the lungs. The caregiver may give some medicine for pain or to help
relax during the bronchoscopy. Caregivers may also do a
bronchoalveolar lavage through the scope. This is when fluid and cells
are sucked up from your lungs to be sent to the lab for tests.

  


Caregivers use it to see how the trachea, esophagus, lungs, and heart
look like and are doing. This may also be used to look for signs of
infection, like pneumonia, or collapsed lungs. Chest x-rays may show
tumors, broken ribs, or fluid around the heart and lungs.



  

  

This test is also called a CT or CAT scan. A special x-ray machine uses
a computer to take pictures of the neck and chest. Before taking the
pictures, the person may be given a dye through an IV. The dye helps
the esophagus, trachea, lungs, heart, and blood vessels show up better
in the pictures. People who are allergic to shellfish (lobster, crab, or
shrimp) may be allergic to this dye.

‘


This test uses a scope to see the inside of the digestive tract. A scope
is a long, bendable tube with a light on the end of it. A camera may be
hooked to the scope to take pictures. During an endoscopy, caregivers
may find problems with how the digestive tract is working. Samples
may be taken from the digestive tract and sent to a lab for tests.
Small tumors may be removed, and bleeding may be treated during an
endoscopy.




Any attempt at feeding could cause aspiration pneumonia as the milk

collects in the blind pouch and overflows into the trachea and lungs.
Furthermore, a fistula between the lower esophagus and trachea may
allow stomach acid to flow into the lungs and cause damage. Because of
these dangers, the condition must be treated as soon as possible after
birth.

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Babies with all but H type fistulas are unlikely to survive without
surgical separation and repair of the trachea and the esophagus.
Surgery is usually done at a hospital that has special facilities for
treating seriously ill newborns. However, surgery cannot always be
performed immediately because of prematurity , the presence of
other birth defects, or complications from aspiration pneumonia.



The survival rate of infants with tracheoesophageal fistulas improved

dramatically toward the end of the twentieth century. In
uncomplicated cases, the survival rate is close to 100 percent.
However, often babies with TEFs have other birth defects that limit
their recovery.

When the esophagus is successfully separated and reattached, many

infants have difficulty swallowing, because the contractility of the
esophagus is impaired. Infants may also have problems with
gastroesophageal reflux, in which the acidic contents of the stomach
back up into the bottom of the esophagus and cause ulcers and
scarring. Long-term follow-up, however, finds that 80 to 90 percent of
children who have repaired TEFs as infants eat normally by the time
they are in elementary school. As more individuals with corrected TEFs
reach adulthood, there is some evidence that suggests they are more
susceptible to esophageal cancers.

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This case pertains to a child named____________ who is presently

aged ______ .

The child was diagnosed with TYPE B esophageal atresia wherein the
upper esophageal pouch was abnormally connected to the trachea
(TEF).The lower esophageal pouch ended blindly, i.e there was a
connection between the esophagus and trachea but no connection
between the upper and lower parts of the esophagus.

This abnormality was diagnosed within 5-7 minutes of birth . After

conformations through tests and X-rays

a decision to perform a surgery was taken within 40 minutes.

While awaiting surgery, the infant's condition is stabilized.

Preoperative care concentrates on avoiding aspiration pneumonia and
includes the following:

˜? Elevating the head to avoid reflux and aspiration of the stomach

contents
˜? Using a suction catheter to continuously remove mucus and saliva
that could be inhaled
˜? When necessary, placement of a gastrostomy tube for feeding
withholding feeding by mouth
 

An incision is made on the child's chest, usually on the right side. Then
a cut is made through the abnormal channel between the windpipe and
esophagus and sewn close. Next if the two ends of esophagus are sewn
together. In this case, the two ends of the esophagus being closely
located they could be sewn together. If the two ends of the esophagus
were too far apart to be reattached, a piece of tissue from the large
intestine would have been used to join the parts.

An opening through the wall of the baby's belly is made and a tube is
placed into the stomach. This provides a safe way to give the baby
nutrition until they can feed by mouth. It also lets doctors give the
baby medicine, if needed.

The surgeon takes out the gastrostomy after the baby has surgery to
repair the esophagus and has had time to heal. This usually takes at
least several months.
   

Post surgery all critical organs were found to be intact which solved a
major threat.

no breathing problems were found which could have reduced the

chances of survival.

Gastrostomy tube for feeding was removed after 4 days and after +ve
response to mechanical feeding mother's milk was given on the 9th day.

About one to three weeks after discharge from the hospital, the child
had to see the surgeon for follow up visit. The surgeon made sure the
incision was healing and the child was recovering, gaining weight and
growing properly. Further follow up sessions were planned accordingly.

Utmost hygiene was maintained to protect the child from any kind of
infection as it could create further complications. The posture of the
baby was maintained as instructed by the doctor to facilitate healing
and proper growth of the gastro-intestinal tract.

As they grow older, children with TEF/EA usually can eat normally.
They probably would need to eat more slowly and chew more thoroughly
than people who did not have TEF/EA.
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