++
William Durston first described isolated esophageal atresia (EA)
in 1670. He later described 40 cases of EA and tracheoesophageal
fistula (TEF) and astutely noted that the fistulas typically arose
just proximal to the carinal bifurcation. In 1697, Thomas Gibson
was first to describe EA with TEF. Gibson was summoned to care for
an infant with aspiration associated with all feeding attempts.
The infant died, and on autopsy Gibson noted the EA and TEF. It
was not until the 1900s that operative interventions were attempted.
The first survivors were reported by William Ladd and N. Logan Leven in
1939. Both used a staged approach that consisted of fistula ligation,
cervical esophagostomy, and gastrostomy tube placement. Later esophageal
continuity was established via an antethoracic esophagus.2,3 Cameron
Haight performed the first successful extrapleural primary repair
of an EA and TEF in 1941.4
+++
Clinical Features
and Classification
++
Esophageal atresia (EA) has been classified into 5 types based
on whether the esophagus is present and the location of fistula
to the trachea. Figure 392-1 illustrates
the different types of atresias with their incidence and anatomic
findings. The most common type of EA has atresia of the esophagus
with a distal tracheoesophageal fistula (TEF) (85%) with
an esophageal fistula that typically originates just proximal to the
carina in the posterior trachea. The next most common is a pure esophageal
atresia without TEF (10%). The blind-ending upper esophageal
pouch can have a variable length that determines if primary repair
is feasible.
++
++
Tracheoesophageal fistula is associated with several other congenital
anomalies.5 The most common association is described by
the acronym VACTERL and includes anomalies of the
vertebrae (similar to those of spondylocostal dysplasia), intestinal atresia,
cardiac malformations (patent ductus arteriosus, atrial septal defect,
or ventricular septal defect), tracheoesophageal fistula, renal
anomalies (urethral atresia with hydronephrosis), and limb anomalies
(hexadactyly, humeral hypoplasia, radial aplasia, and proximally
placed thumb) (see Table 415-2). Rare cases
of VACTERL association in the offspring of an affected individual
have been reported, but this is uncommon. eTable
392.1 shows the incidence of anomalies in
a large series of patients with esophageal atresia.5 Other
associated anomalies include hypospadias, undescended testis, duodenal
atresia (as in oculodigitalesophagoduodenal syndrome with gene defect
in the 2p24–p23 region), and hydrocephalus secondary to
aqueductal stenosis. Genetic syndromes, including oculoauriculovertebral dysplasia
(Goldenhar syndrome) and Opitz-G syndrome, can also be associated
with esophageal anomalies.
++
++
Polyhydramnios is present in about one third of mothers carrying
infants with esophageal fistula with distal tracheoesophageal fistula
and in virtually 100% of mothers carrying infants with
pure esophageal atresia, apparently due to the inability of the
fetus to swallow amniotic fluid. Other antenatal ultrasound findings
often include microgastria or a distended upper esophageal pouch.
++
After birth, the presence of excessive drooling or aspiration
and coughing with feeds should lead to an evaluation for esophageal
atresia. Confirmation of the diagnosis can be made by attempting
to gently pass a 10 French orogastric tube into the stomach. If
esophageal atresia is present, a radiograph can be taken while mild pressure
is placed on the tube to determine the approximate length of the
proximal esophageal pouch. Rib spaces or vertebrae can be counted
between the tip of the orogastric tube and the carina to determine
the length of the esophageal gap (Fig. 392-2).
The site of the carina on chest x-ray can serve as a surrogate for
the location of the distal esophageal segment. The chest radiograph also
diagnoses a fistula if air is seen in the intestines. This can be in
the form of a distal, isolated, or double fistula. Conversely, if
the infant has a gasless abdomen, then the diagnosis is true esophageal atresia
or a proximal tracheoesophageal fistula. Contrast studies should
be avoided due to the risk of aspiration.
++
++
In contrast to this typical presentation, patients with H-type
fistulas (intact esophagus with a congenital tracheoesophageal fistula)
typically present later in life, with repeated bouts of aspiration pneumonia.
Because their esophagus is in continuity, they are able to eat;
however, they aspirate swallowed or refluxed esophageal contents
via the fistula. The diagnosis can be difficult; routine radiographic
barium swallows often overlook
the fistula. The fistula is best identified by instilling thin barium
through the tip of a nasogastric tube, with moderate pressure to
distend the esophagus while slowly withdrawing the tube from the
stomach into the esophagus. This tends to force the fistula open
and allows visualization, as seen in eFigure 392.2A. Repeated
radiography and/or rigid esophagoscopy and bronchoscopy
may be necessary before the diagnosis is recognized (eFig.
392.2B).
++
++
Once the diagnosis of esophageal atresia is made, a search for
associated anomalies is required. Radiographs should be performed
to evaluate for possible vertebral anomalies. A renal ultrasound
is performed to identify renal anomalies. Spinal ultrasound or magnetic
resonance imaging should be performed to evaluate for a tethered
cord, and a voiding cystourethrogram should be performed at some
point prior to discharge. Prior to performing operative repair, it
is imperative that an echocardiogram be performed to rule out cyanotic
congenital heart anomalies that may require immediate repair and
to define the anatomy of the aortic arch, which can determine the
surgical approach.
+++
Esophageal Atresia
with Distal Tracheoesophageal Fistula
++
Initial management of these infants is focused on minimizing
aspiration by elevating the head (>30˚) and inserting an
orogastric catheter into the blind upper esophageal pouch. A double-lumen
sump-type orogastric catheter should be secured appropriately and
connected to low suction. The catheter prevents saliva from pooling
in the pouch, and elevation of the head facilitates drainage of
secretion into the pouch for suctioning and prevents reflux of gastric
contents into the lungs. Antibiotic therapy should be initiated and
surgical consultation obtained.
++
Timing and selection of surgical intervention in patients with
esophageal atresia and distal tracheoesophageal fistula is determined
by the degree of prematurity, patient weight, overall clinical condition,
presence of other associated anomalies, and the length of the gap
between the ends of the esophagus. In healthy near-term or full-term
infants without other severe anomalies and with minimal pneumonitis,
closure of the fistula and primary esophageal anastomosis is typically
performed within the first 24 to 72 hours after birth. Usually,
the esophageal gap is 1 to 2 cm, allowing primary anastomosis of
the 2 ends without tension. This repair can be performed with an
extrapleural or transpleural approach. The advantage of the extrapleural
approach is the avoidance of pleural contamination if an anastomotic
leak occurs. In the case of a straightforward primary esophageal
repair, a gastrostomy tube is rarely placed.
++
Continuous intensive care is crucial in the postoperative period. Intuitively,
manipulation of the trachea and esophagus must be avoided in the
postoperative period to prevent disruption of the tracheal repair
and esophageal anastomosis. These patients are typically intubated
postoperatively, and it is imperative to prevent migration of the
endotracheal tube and possible perforation of the tracheal closure. To
prevent this, the endotracheal tube should be secured adequately
to the infant and its location in relation to the mouth noted and
checked with nursing assessments and chest x-rays. Deep suctioning,
placement, or manipulation of orogastric tubes/nasogastric
tubes must be avoided. Depending on the circumstances, the endotracheal
tube may remain in place for a few hours to several days. Postoperative
nutritional support is provided either parenterally via a Silastic
transanastomotic tube placed at the time of surgery for enteral
nutrition. An esophagram is typically obtained 5 to 7 days after
surgery to check for an anastomotic leak. If no leak is detected,
then oral feeds are instituted. If a contained leak is seen, the
patient is not fed until the study is repeated in several days.
++
In infants with severe pneumonia, prematurity, or other significant medical
problems, the risk of major surgery is increased, so a gastrostomy
tube is placed to allow decompression of the intestinal tract and
medical optimization prior to esophageal repair. Head-of-bed elevation
and upper pouch suction are maintained until surgery can be accomplished. In
the extremely small infant in whom it is apparent that surgical
correction of the tracheoesophageal anomaly must be delayed, surgical
closure of the tracheoesophageal fistula can be performed in addition
to decompressive gastrostomy, allowing gastrostomy feeding. If the esophageal
reconstruction will be delayed for an extended period, a cervical
diversion esophagostomy may be performed to reduce the risk of aspiration.
++
A subset of patients with esophageal atresia and distal tracheoesophageal
fistula will develop respiratory failure. These patients are unable
to deliver adequate tidal volumes because of leakage through the
fistula and into the gastrointestinal tract. This can lead to intestinal
distension, which inhibits diaphragmatic movement, further compromising respiration.
Temporary nonoperative techniques to help improve ventilation while
one awaits operative intervention include right mainstem bronchus
intubation and high-frequency oscillatory ventilation. Intubation
may worsen respiration because positive pressure can exacerbate
intestinal distension. However, by placing the endotracheal tube
distal to the fistula in the right mainstem bronchus, leakage of
the tidal volume and increased gastric distension will be minimized.
High-frequency oscillatory ventilation produces small tidal volumes
leading to decreased leakage through the fistula. Emergent operative
approaches to improve oxygenation include gastrostomy tube placement
and fistula ligation and division. Gastrostomy decompression can
increase diaphragmatic excursion. Additionally, the gastrostomy
tube can be connected to a Pleurovac on water seal to create resistance
to air flowing through the fistula. This may result in the tidal
volume preferentially going to the lower resistance pathway (the
lungs) rather than to the increased resistance in the gastrostomy
tube. Definitive treatment of the fistula can be performed by its
ligation and division via thoracotomy, but a primary repair of the
esophagus is usually not performed at the time of ligation in these
unstable patients. A staged approach with initial fistula ligation
and division followed by stabilization and later primary esophageal
repair is preferred.
+++
Esophageal Atresia
Without Tracheoesophageal Fistula
++
Infants with esophageal atresia without a tracheoesophageal fistula
present with excessive oral secretions, drooling, and choking, similar
to esophageal atresia and tracheoesophageal fistula patients, but
they have a flat, gasless abdomen. If fed, they are unable to swallow,
and diagnostic studies confirm the presence of a blind upper esophageal pouch.
The length of the atretic segment varies, but a wide gap usually
divides the upper and lower ends of the esophagus, thus making primary
anastomosis difficult or impossible.
++
Initial management of these patients includes gastrostomy tube
placement and intraoperative determination of the gap length. Primary
repair can usually be achieved if the gap is less than 2 vertebral
spaces. For gaps spanning 3 to 6 vertebrae, delayed esophageal anastomosis
is planned in 2 to 3 months. During this period, a variety of surgical
approaches can be attempted to bridge the gap between the 2 esophageal
segments in the hopes of achieving a primary repair. These vary
from bougienage to novel methods such as placing magnets in each
segment to draw the ends closer, thus lengthening both limbs and
narrowing the gap. Esophageal lengthening is unlikely to succeed
if the gap is wider than 6 vertebrae. In such instances, gastrostomy
tube placement and cervical esophagostomy are performed with later esophageal
replacement. Conduits for esophageal replacement include gastric
tube, gastric transposition, and colonic and jejunal interposition
grafts.
+++
Isolated Tracheoesophageal
Fistula (H-Type Fistula)
++
Isolated tracheoesophageal fistulas course obliquely from the
posterior trachea to the more caudal anterior esophagus. Infants with
isolated tracheoesophageal fistula may present with intermittent
episodes of choking during feeding in the newborn period. Others
present later with persistent choking episodes, chronic cough, recurrent
pneumonias, or reactive airway disease. Presumed congenital isolated
tracheoesophageal fistulas have presented in adulthood with only
mild symptoms of cough and intermittent pneumonias since childhood. Surgical
division of the fistula can be achieved through a cervical approach.
No gastrostomy is required, and patients usually have minimal morbidity.
+++
Postoperative Management
and Complications
++
Early complications following surgical repair of esophageal atresias
include anastomotic leak, anastomotic stricture, and recurrent fistula.
Anastomotic leaks occur in 15% to 20% of patients,
with a majority being managed nonoperatively by providing parenteral
nutrition and antibiotics until spontaneous closure is documented
by a repeat esophagram. Then oral feeding is begun. Major leaks
are rare and usually present with tachypnea, sepsis, and/or
tension pneumothorax on the second or third postoperative day. Treatment
includes tube thoracostomy and possible reexploration.
++
Stricture formation at the site of the esophageal anastomosis
develops in 30% to 40% of patients. Contributing
factors to stricture formation include anastomotic tension, ischemia,
and leak. Late stricture development is attributed to gastroesophageal
reflux. Anastomotic strictures usually respond to repeated dilatation
either by radiographic placement of a balloon dilator or by progressive
bougie-type dilatation. Recalcitrant strictures may benefit from
injection of triamcinolone at the time of dilation,6 although
some require resection and secondary anastomosis.
++
Recurrent tracheoesophageal fistula is thankfully rare with an
incidence of 5%. Symptoms of recurrence include coughing, choking, cyanosis,
apnea, and recurrent pulmonary infections. Diagnosis can be difficult,
and the same techniques described for isolated fistulas are utilized:
rigid esophagoscopy/bronchoscopy and withdrawal esophagram
via a nasogastric tube. Treatment is surgical and requires division
of the fistula.
++
Gastroesophageal reflux is common after esophageal repair.7 A
recent study in postoperative esophageal atresia and tracheoesophageal
fistula patients showed the incidence of gastroesophageal reflux
doubles from age 6 months to 1 year (16–39%),
and then it continues to rise to 51% at age 5.8 Incompetent
lower esophageal sphincter, alteration of the angle of His, and
poor distal esophageal motility all contribute to gastroesophageal
reflux disease with a risk of esophagitis, peptic stricture, and
Barrett esophagus. Treatment with proton pump inhibitors is usually
adequate. Alternatively, antireflux surgery can be considered. Patients
with esophageal atresia/tracheoesophageal fistula should
be followed long-term because they are at risk of developing Barrett
esophagus and esophageal cancer.9-11
++
Children who have required an esophageal replacement procedure
are at even higher risk of gastroesophageal reflux with ulceration
of the interposed segment. In addition, these children often have
subclinical gastroesophageal reflux with silent aspiration, resulting
in the development of chronic pulmonary disease. Interposed segments,
particularly colonic or gastric interpositions, may distend with
time, further compromising pulmonary function and occasionally requiring
surgical revision.
++
Other complications include food or foreign impaction either
at the site of esophageal structuring or in the aperistaltic segment
of the distal esophagus, and dysphagia (particularly for solids)
is common because of abnormal peristalsis in the lower esophageal
segment. Occasionally, bronchial remnants are associated with tracheoesophageal
fistula.
++
The overall prognosis after surgical repair of esophageal atresia
and tracheoesophageal fistula depends on the type of anatomic abnormality
and the presence of other anomalies. In general, survival is greater
than 90%, and most patients have normal life expectancy
and productive lives.