Esophageal Atresia and TEF

• ANATOMIC VARIETIES
– The five major varieties of EA and TEF .
– The most commonly seen variety is EA with distal TEF (type C),
which occurs in approximately 85% of the cases in most series.
– The next most frequent is pure EA (type A), occurring in 8 to 10% of
patients, followed by TEF without EA (type E).
– The latter occurs in 8% of cases and is also referred to as an H-type
fistula, based on the anatomic similarity to that letter.
– EA with fistula between both the proximal and distal ends of the
esophagus and trachea (type D) is seen in approximately 2% of
cases, and type B, EA with TEF between the proximal esophagus
and trachea, is seen in approximately 1% of cases

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• The five varieties of esophageal atresia (EA) and
tracheoesophageal fistula (TEF).
– A. Isolated EA. = 8-10%
– B. EA with TEF between the proximal segment of the
esophagus and the trachea. = 1%
– C. EA with TEF between the distal esophagus and the
trachea=85
– D. EA with fistula between both the proximal and distal ends of
the esophagus and the trachea. =2
– E. TEF without EA (H-type fistula)=8.
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• Other congenital anomalies commonly occur in association
with EA-TEF.
• For instance, VACTERL syndrome is associated with
– vertebral anomalies (absent vertebrae or hemivertebrae),
– anorectal anomalies (imperforate anus),
– cardiac defects,
– tracheoesophageal fistula,
– renal anomalies (renal agenesis, renal anomalies), and
– radial limb anomalies (most often radial dysplasia).
• In nearly 20% of the infants born with EA, some variant of
congenital heart disease occurs.
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 CLINICAL PRESENTATION
• The anatomic variant of EA-TEF in the infant predicts the clinical
presentation.
• When the esophagus ends either as a blind pouch or as a fistula into
the trachea (as in types A, B, C, and D), infants present with
excessive drooling, followed by choking or coughing immediately
after feeding is initiated as a result of aspiration through the fistula
tract.
• Air in the distal intestine indicates either a patent esophagus or a
fistula to the respiratory tract.
• In type C TEF, as the neonate coughs and cries, air is transmitted
through the fistula into the stomach, which results in abdominal
distention.
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• As the abdomen distends, it becomes increasingly more
difficult for the infant to breathe.
• This leads to further atelectasis, which compounds the
pulmonary dysfunction.
• In patients with the C and D varieties, the regurgitated gastric
juice passes through the fistula, collecting in the trachea and
lungs and leading to a chemical pneumonitis , which further
exacerbates the pulmonary status.
• In many instances, the diagnosis is actually made by the
nursing staff who attempt to feed the baby and notice the
accumulation of oral secretions.
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• The diagnosis of EA is confirmed by the inability to pass an
orogastric tube into the stomach (Fig. 39-10).
• The dilated upper pouch occasionally may be seen on a plain
chest radiograph.
• If a soft feeding tube is used, the tube will coil in the upper
pouch, which provides further diagnostic certainty.
• An important alternative diagnosis that must be considered
when an orogastric tube does not enter the stomach is that of
an esophageal perforation.
• This problem can occur in infants after traumatic insertion of a
nasogastric or orogastric tube.
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• Whenever there is any diagnostic uncertainty, a contrast study can
confirm the diagnosis of EA and occasionally document the TEF;
however, the obvious risks of aspiration associated with an un drained
blind pouch cannot be overstated.
• The presence of a TEF can be demonstrated clinically by the finding of air
in the GI tract.
• This can be proven at the bedside by percussion of the abdomen and
confirmed by plain abdominal radiograph.
• Occasionally, a diagnosis of EATEF can be suspected prenatally on
ultrasonographic evaluation.
– Typical features include failure to visualize the stomach and
– the presence of polyhydramnios.
• These findings reflect the absence of efficient swallowing by the fetus.

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• In a child with EA, it is important to identify whether coexisting anomalies are present.
• These include
– cardiac defects in 38%,
– skeletal defects in 19%,
– neurologic defects in 15%,
– renal defects in 15%,
– anorectal defects in 8%, and
– other abnormalities in 13%.
• Examination of the heart and great vessels with echocardiography is important to exclude
cardiac defects, because these are often the most important predictors of survival in
these infants.
• The echocardiogram also demonstrates whether the aortic arch is left sided or right sided,
which may influence the approach to surgical repair.
• Vertebral anomalies are assessed by plain radiography,and a spinal ultrasonographic
scan is obtained.

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• A patent anus should be confirmed clinically.
• The kidneys in a newborn may be assessed clinically by
palpation.
• An ultrasonographic scan of the abdomen will demonstrate
the presence of renal anomalies, which should be suspected
in the child who fails to produce urine.
• The presence of other extremity anomalies is suspected
when there are missing digits and can be confirmed by
plain radiographs of the hands, feet, forearms, and legs.
• Rib anomalies may also be present.
• These may include the presence of a thirteenth rib.
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 INITIAL MANAGEMENT
• The initial treatment of infants with EA-TEF includes
• attention to respiratory status,
• decompression of the upper pouch, and
• appropriate timing of surgery.
• Because the major determinant of poor survival is the presence of
other severe anomalies, a search for other defects, including
congenital cardiac disease, is undertaken.
• The initial strategy after the diagnosis is confirmed is to place the
neonate in an infant warmer with the head elevated at least 30
degrees.
• A sump catheter on continuous suction is placed in the upper pouch.
• Both of these strategies are designed to minimize the degree of
aspiration from the esophageal pouch.
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• Acid prophylaxis is given to reduce the chemical irritation of
any refluxed contents.
• When saliva accumulates in the upper pouch and is
aspirated into the lungs, coughing, bronchospasm, and
desaturation episodes can occur, which may be minimized by
ensuring the patency of the sump catheter.
• IV antibiotic therapy is initiated, and warmed electrolyte
solution is administered.
• When possible, the right upper extremity is avoided as a
site to start an IV line, because this location may interfere
with positioning of the patient during the surgical repair.
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• The timing of repair is influenced by the stability of the
patient's condition.
• Definitive repair of the EA-TEF is rarely a surgical
emergency.
• If the child is hemodynamically stable and is oxygenating
well, definitive repair may be performed within 1 to 2 days
after birth.
• This allows a careful examination for the presence of
coexisting anomalies and selection of an experienced
anesthetic team.
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 PRIMARY SURGICAL CORRECTION
• In an infant in stable condition, definitive repair is
achieved through performance of a primary
esophagoesophagostomy.
• There are two approaches to this operation:
– open thoracotomy and
– thoracoscopy.
• In the open approach, the infant is brought to the
operating room, intubated, and placed in the lateral
decubitus position with the right side up in preparation for
right posterolateral thoracotomy.
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• Infants with pure oesophageal atresia and no tracheo-
oesophageal fistula are usually best managed by a
temporary gastrostomy and delayed primary repair.
• Except for very-low-birth weight babies and those with
major congenital heart disease, most infants with
repaired oesophageal atresia have a good prognosis.

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 COMPLICATIONS OF SURGERY
• 1. Anastomotic leakage occurs in 10 to 15% of patients
and may be seen either in the immediately
postoperative period or after several days.
• 2. Strictures are not infrequent (10 to 20% of cases),
particularly if a leak has occurred.
• 3. Gastroesophageal reflux commonly occurs after repair
of EA-TEF
• 4. Recurrent tracheoesophageal fistula may represent a
missed upper pouch fistula or a true recurrence.
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 SPECIAL CIRCUMSTANCES
• Patients with type E TEFs (commonly referred to as H-type) present
beyond the newborn period.
• Presenting symptoms include
• recurrent chest infections,
• Bronchospasm , and
• failure to thrive.
• The diagnosis can be suspected from the results of
– barium esophagography and
– confirmed by endoscopic visualization of the fistula.
• Surgical correction is generally possible through a cervical approach after
placement of a balloon catheter across the fistula and requires
mobilization and division of the fistula.
• Outcome is usually excellent.

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• Patients with duodenal atresia and EA-TEF may require
• urgent treatment due to the presence of a closed obstruction of
• the stomach and proximal duodenum. In stable patients, treatment consists of repair of the esophageal
anomaly and correction of the duodenal atresia if the infant is stable during surgery.
• If not, a staged approach should be used consisting of ligation
• of the fistula and placement of a gastrostomy tube. Definitive
• repair can then be performed at a later point in time.
• Primary EA (type A) represents a challenging problem,
• particularly if the upper and lower ends are too far apart for
• an anastomosis to be created. Under these circumstances, treatment strategies include placement of a
gastrostomy tube and
• performing serial bougienage to increase the length of the upper
• pouch. This occasionally allows for primary anastomosis to be
• performed. Occasionally, when the two ends cannot be brought
• safely together, esophageal replacement is required using a
• gastric pull-up, reverse gastric tube, or colon interposition

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• Outcome. Various classification systems have been used to
• predict survival in patients with EA-TEF and to stratify treatment. A system devised by
Waterston in 1962 was used to stratify neonates based on birth weight, the presence of
pneumonia,
• and the identification of other congenital anomalies. In response
• to advances in neonatal care, the surgeons from the Montreal
• Children’s Hospital proposed a new classification system. In
• the Montreal experience, only two characteristics independently
• affected survival: preoperative ventilator dependence and associated major anomalies.
Pulmonary disease as defined by ventilator dependence appeared to be more accurate than
pneumonia.
• When the two systems were compared, the Montreal system
• more accurately identified children at highest risk. Spitz and
• colleagues analyzed risk factors in infants who died with EATEF. Two criteria were found to
be important predictors of outcome: birth weight less than 1500 g and the presence of major
• congenital cardiac disease. A new classification for predicting
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• Outcome in EA was therefore proposed:
– group I: birth weight ≥1500 g, without major cardiac disease, survival 97%
– group II: birth weight <1500 g, or major cardiac disease, survival 59%; and
– group III: birth weight <1500 g, and major cardiac disease, survival 22%

• In general, surgical correction of EA-TEF leads to a satisfactory outcome with nearly

normal esophageal function in most patients.
• Overall survival rates of greater than 90% have been achieved in patients classified
as stable in all the various staging systems.
• Unstable infants have an increased mortality (40% to 60% survival) because of
potentially fatal associated cardiac and chromosomal anomalies or prematurity.
However,
the use of a staged procedure also has increased survival in even these high-risk
infants

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