Esophageal atresia with or without tracheoesophageal fistula
Esophageal Atresia With or Without
Esophageal atresia refers to a congenitally interrupted esophagus. One or more fistulae
may be present between the malformed esophagus and the trachea-tracheoesophageal
History of the Procedure: The condition was first described anecdotally in the 17th
century. In 1862, the famous pediatrician from Copenhagen, Hirschsprung, described 14
cases of esophageal atresia. Later in that century, this condition was recognized to often
occur in conjunction with other gastrointestinal and renal anomalies.
With the advent of the 20th century, surgeons were theorizing about how the lesion
could be repaired. In 1939 and 1940, Ladd of Boston and Lever of Minnesota first
achieved surgical success in stages, where success meant that the affected children
survived and skin-lined pharyngogastric conduits were eventually constructed. However,
in 1941 Cameron Haight of Michigan successfully repaired esophageal atresia in a 12day-old baby by using a primary 1-stage left-sided extrapleural approach. Subsequent to
that child's survival and with advances in surgical and anesthetic techniques,
esophageal atresia is now regarded as an eminently correctable congenital lesion.
Problem: The lack of esophageal patency prevents swallowing. In addition to
preventing normal feeding, this problem may cause infants to aspirate and literally
drown in their own saliva, which quickly overflows the upper pouch of the obstructed
esophagus. If a TEF is present, fluid (either saliva from above or gastric secretions from
below) may flow directly into the tracheobronchial tree.
Frequency: In North America, the incidence of esophageal atresia is 1 case in 30004500 births. This frequency may be decreasing for as yet unknown reasons.
Internationally, the highest incidence of this disorder is in Finland, where it is 1 case in
Etiology: As yet, no human teratogens that cause esophageal atresia are known.
Reports exist of esophageal atresia occurring in families. A 2% risk of recurrence is
present when a sibling is affected. The occasional association of esophageal atresia
with trisomies 21, 13, and 18 further suggests genetic causation. Also, twinning occurs
about 6 times more frequently in patients with esophageal atresia than in those without
At present, most authorities believe that the development of esophageal atresia has a
nongenetic basis. Debate about the embryopathologic process of this condition
continues, and little about it is known. The old His theory that lateral infoldings divide the
foregut into the esophagus and trachea is attractively simple, but findings from human
embryology studies do not support this theory.
In 1984, O'Rahilly proposed that a fixed cephalad point of tracheoesophageal separation
exists, with the tracheobronchial and esophageal elements elongating in a caudal
direction from this point. This theory does not easily account for esophageal atresia, but
it explains TEF as a deficiency or breakdown of esophageal mucosa, which occurs as
the linear growth of the organ exceeds the cellular division of the esophageal epithelium.
In a 1987 report, Kluth eschews the concept that tracheoesophageal septation has a key
role in the development of esophageal atresia. Instead, he bases the embryopathologic
process on the faulty development of the early but already differentiated trachea and
esophagus in which a dorsal fold comes to lie too far ventrally; thus, the early
tracheoesophagus remains undivided. He also suggests that esophageal vascular
and/or ischemic events may be causes in cases of esophageal atresia without fistula.
Using a rat model of adriamycin-induced teratogenesis, in 2003, Spilde et al found
esophageal atresia–TEF formations in the embryos. They detected specific absences of
certain fibroblast growth factor (FGF) elements, both FGF1 and the IIIb splice variant of
the FGF2R receptor. They postulated that these specific FGF signaling absences allow
for the nonbranching development of the fistulous tract from the foregut that then
establishes continuity with the developing stomach.
In 2001, Orford et al postulated that the ectopic ventrally displaced location of the
notochord in an embryo at 21 days' gestation can lead to a disruption of the gene locus,
sonic hedgehog-signaled apoptosis in the developing foregut, and variants of
esophageal atresia. This situation may be due to a variety of early gestation teratogenic
influences such as twinning, toxin exposure, or possible abortion. More studies are
Pathophysiology: The variants of esophageal atresia have been described using many
anatomic classification systems. To avoid ambiguity, the clinician should use a narrative
description. Nevertheless, Gross of Boston described the classification system that is
most often cited. The types in this system are as follows (with the approximate incidence
in all infants born with esophageal anomalies in parentheses):
Type A - Esophageal atresia without fistula or so-called pure esophageal atresia
Type B - Esophageal atresia with proximal TEF (<1%)
Type C - Esophageal atresia with distal TEF (85%)
Type D - Esophageal atresia with proximal and distal TEFs (<1%)
Type E - TEF without esophageal atresia or so-called H-type fistula (4%)
Type F - Congenital esophageal stenosis (<1%) (These are not discussed in this
The fetus with esophageal atresia cannot effectively swallow amniotic fluid, especially
when TEF is absent. In the fetus with esophageal atresia and a distal TEF, some
amniotic fluid presumably flows through the trachea and down the fistula to the gut.
Polyhydramnios may be the result of this change in the recycling of amniotic fluid
through the fetus. Polyhydramnios, in turn, may lead to premature labor. The fetus also
appears to derive some nutritional benefit from the ingestion of amniotic fluid; thus,
fetuses with esophageal atresia may be small for their gestational age.
The neonate with esophageal atresia cannot swallow and drools copious amounts of
saliva. Aspiration of saliva or milk, if the baby is allowed to suckle, can lead to an
aspiration pneumonitis. In a baby with esophageal atresia and a distal TEF, the lungs
may be exposed to gastric secretions. Also, air from the trachea can pass down the
distal fistula when the baby cries, strains, or receives ventilation. This condition can lead
to an acute gastric perforation, which is often lethal. Prerepair esophageal manometric
studies have revealed that the distal esophagus in esophageal atresia is essentially
dysmotile, with poor or absent propagating peristaltic waves. This condition results in
variable degrees of dysphagia after the repair and contributes to gastroesophageal
The trachea is also affected by the disordered embryogenesis in esophageal atresia.
The membranous part of the trachea, the pars membranacea, often is wide and imparts
a cross-sectional D shape to the trachea, as opposed to the usual C shape. These
changes cause secondary anteroposterior structural weakening of the trachea, or
tracheomalacia. This weakening can result in a sonorous cough as the intrathoracic
trachea resonates and partially collapses with forceful expiration. Secretions can be
difficult to clear and may lead to frequent pneumonias. Also, the trachea can partially
collapse during feeding, after repair, or with episodes of gastroesophageal reflux; this
partial collapse can lead to ineffective respiration; hypoxia; and, somewhat inexplicably,
Clinical: The mother who is carrying a fetus with esophageal atresia may have
polyhydramnios, which occurs with approximately 33% of fetuses with esophageal
atresia and distal TEF and with virtually 100% of fetuses with esophageal atresia without
fistula. Characteristically, the neonate born with esophageal atresia drools and has quite
a bit of mucus, with excessive oral secretions. If suckling at the breast or bottle is
allowed, the baby appears to choke and may have difficulty maintaining an airway.
Significant respiratory distress may result. In the delivery room, the affected infant may
have the sonorous seal-bark cough that indicates concomitant tracheomalacia. If an oral
tube is placed to suction the stomach, as it is in some delivery rooms, it characteristically
becomes blocked 10-11 cm from the lips.
VACTERL are associated anomalies that should be readily apparent on physical
examination. If any of these anomalies are present, the presence of the others must be
assessed. The VACTERL syndrome occurs when 3 or more of the associated
anomalies are present. This syndrome occurs in approximately 25% of all patients with
esophageal atresia. Anomalies in this syndrome include the following:
Vertebral defects - Multiple or single hemivertebrae, scoliosis, rib deformities
Anorectal malformations - Imperforate anus of all varieties, cloacal deformities
Cardiovascular defects - Ventricular septal defect (VSD), which is most common,
tetralogy of Fallot, patent ductus arteriosus, atrial septal defects, atrioventricular
canal defects, aortic coarctation, right-sided aortic arch, single umbilical artery,
Tracheoesophageal defects - Esophageal atresia
Renal anomalies - Renal agenesis including Potter syndrome, bilateral renal
agenesis or dysplasia, horseshoe kidney, polycystic kidneys, urethral atresia,
Limb deformities - Radial dysplasia, absent radius, radial-ray deformities,
syndactyly, polydactyly, lower-limb tibial deformities
Other associated conditions include coloboma, heart defects, atresia choanae,
developmental retardation, genital hypoplasia, and ear deformities (CHARGE).
The following anomalies occur with increased frequency in esophageal atresia:
neurologic defects (eg, neural tube defects, hydrocephalus, tethered cord,
holoprosencephaly), gastrointestinal defects (eg, duodenal atresia, ileal atresia,
hypertrophic pyloric stenosis, omphalocele, malrotation, Meckel diverticulum),
pulmonary defects (eg, unilateral pulmonary agenesis, diaphragmatic hernia), and
genitalia defects (eg, undescended testicles, ambiguous genitalia, hypospadias). Also,
trisomy 13, 21, or 18 and Fanconi syndrome may be present. The overall incidence of
associated anomalies is approximately 50%. Cardiovascular anomalies occur in 35% of
cases; genitourinary anomalies occur in 20%; and associated gastrointestinal anomalies
occur in approximately 20%. A tethered cord would usually be detectable by
ultrasonography in the newborn period or later by MRI (or less desirably by CT
scanning) if findings are equivocal.
The indication and timing of surgical repair may be determined by using the Waterston,
Spitz, or Poenaru prognostic classification system.
In 1962, Waterston developed a prognostic classification system for esophageal atresia
that is still used today. Category A includes patients who weigh more than 5.5 lb (2.5 kg)
at birth and who are otherwise well; category B includes patients who weigh 4-5.5 lb
(1.8-2.5 kg) and are well or have higher birth weight and moderate pneumonia and other
congenital anomaly; and category C includes patients who weigh less than 4 lb (1.8 kg)
or have higher birth weight and severe pneumonia and severe congenital anomaly.
Management strategies are the following: category A, immediate primary repair;
category B, delayed repair; and category C, staged repair.
In 1994, after analyzing findings in 387 patients, Spitz recognized that the presence or
absence of cardiac disease is a proven major prognostic factor. Spitz suggested the
following groups, which are analogous to those in the Waterston classification system:
group I, birth weight greater than 1.5 kg and no major cardiac disease; group II, birth
weight less than 1.5 kg or major cardiac disease; and group III, birth weight less than 1.5
kg and major cardiac disease.
In 1993, Poenaru proposed a simpler 2-group classification system based on logistic
regression analysis findings in 95 patients. Note that birth weight is not a factor. The
classes are the following: class I patients who are low risk and do not meet criteria in
class II and class II patients who are high risk and ventilator dependent or those with
life-threatening anomalies regardless of pulmonary status.
Using a refinement of the Waterston classification, in 1989, Randolph et al reported a
clinically useful system of using a patient's physiologic status to determine the surgical
management, ie, immediate repair, delayed primary repair, or staged repair. Weight,
gestational age, and pulmonary condition were not considered. If the patient's
physiologic parameters were good, they were managed with immediate repair. Staged
repairs were used for severely compromised infants, especially those with severe
cardiac anomalies. In this group, the survival rate was 77% and overall it was 90%.
The above prognostic groupings can allow for the stratification of high-risk patients with
esophageal atresia in planning for delayed and/or staged repair, whereas low-risk
babies can usually undergo early (first 24-48 h) primary one-stage repair. For instance,
a 2-kg baby with esophageal atresia and distal TEF who also has the tetralogy of Fallot
is a Waterston category C, Spitz group II, or Poenaru class II; in this patient, delayed or
staged repair may be best. These classification systems help physicians to compare
results in an organized and meaningful way. Comparing the 3 prognostic classification
systems, Waterston, Poenaru, and Spitz, the Spitz classification appears to have the
most applicability in current practice. Ductal-dependent cardiac lesions still seem to
significantly affect the survival of children born with esophageal atresia.
RELEVANT ANATOMY AND CONTRAINDICATIONS
Relevant Anatomy: The treatment plan for each baby must be individualized. The
prognostic classifications can provide guidance in patients with multiple problems, but
decisions in identifying the most life-threatening anomaly must be made early.
Management plans for a delayed repair of the esophageal atresia may include the
following: A 10F Replogle double-lumen tube may be placed through the mouth or nose
well into the upper pouch to provide continuous suction of pooled secretions from the
proximal portion of the atretic esophagus. The baby may be positioned in the 45° sitting
position. Prophylactic broad-spectrum antibiotics such as ampicillin and gentamicin may
be used. General supportive care and total parenteral nutrition are needed.
With careful bedside attendance, these measures may permit a delay of days to
perhaps weeks. Some have described cases in which the baby was discharged home
with a Replogle tube in situ while waiting for staged repair of an esophageal atresia.
However, deaths have been reported in infants in whom the tube did not maintain an
empty upper pouch. A gastrostomy, distal TEF ligation, or cervical esophagostomy may
permit longer delays in the esophageal atresia repair. Each intrusion, however, carries a
A gastrostomy may be created under local anesthesia if necessary, unless no distal TEF
is present. In such cases, the stomach is small, and laparotomy is required. In all cases
of esophageal atresia in which a gastrostomy is created, care should be taken to place it
near the lesser curve to avoid damaging the greater curve, which can be used in the
formation of an esophageal substitute. When a baby is ventilated with high pressures,
the gastrostomy may offer a route of decreased resistance, causing the ventilation
gases to flow through the distal fistula and out the gastrostomy site. This condition may
complicate the use of ventilation.
In cases such as those just mentioned or in cases in which a distal fistula continues to
cause lung soiling, consider distal TEF ligation. This ligation is performed by means of a
right-sided thoracotomy ideally performed via an extrapleural approach. The fistula may
be clipped or simply ligated. If it is ligated and divided, subsequent staged repair of the
esophageal atresia may be difficult because the distal esophageal segment tends to
retract inferiorly to a substantial degree when it is detached from its tracheal mooring.
However, simple fistula ligation may allow subsequent reopening of the fistula. Division
of the fistula and attempts to anchor it at the mid chest with sutures are usually
A cervical esophagostomy or spit fistula may be constructed in the right or left side of
the neck, depending on the choice for subsequent esophageal substitution. It allows
drainage of the upper pouch and precludes aspiration from the upper pouch. Sham
feeding may be commenced in cases in which a long delay to repair is anticipated. This
feeding may prevent subsequent oral aversion, which is a real problem in babies who
have not been fed by mouth in their early weeks to months of life. However, cervical
esophagostomy usually dooms the child to some form of esophageal substitution.
Contraindications: Potter syndrome is bilateral renal agenesis and has a 100%
mortality rate; therefore, repair of esophageal atresia is contraindicated.
In babies with esophageal atresia, samples should be drawn to determine baseline
values of the following:
Complete blood cell count
Venous gas concentrations
Blood urea nitrogen and serum creatinine levels
Blood glucose level
Serum calcium level
Arterial blood gas concentrations, as necessary
Prenatal ultrasonography may reveal the size of the gastric bubble,
polyhydramnios, and VACTERL anomalies, all of which may indicate esophageal
atresia in the fetus.
The sensitivity of prenatal ultrasonography is approximately 40%.
A diagnosis prenatally of esophageal atresia may be associated with a
Chest radiography is mandatory and should be performed as soon as possible if
esophageal atresia is suspected.
The value of the chest radiography is enhanced if a Replogle tube is in place
and if 5-10 mL of air is injected to distend the upper pouch.
Great caution should be exercised if liquid contrast material is injected into
the proximal pouch. First, only about 1 mL of isotonic water-soluble contrast
should be used to prevent spillage into the airway. A catheter with an endhole should be used. Second, if an upper pouch fistula is present, the
contrast material flows directly into the airway. Usually, a contrast-enhanced
study is unnecessary.
The heart shadow and size should be assessed.
Vertebral and rib anomalies should be assessed.
The lung fields should be assessed for possible aspiration pneumonitis and
for the rarely associated diaphragmatic hernia or congenital lung lesion.
The presence or absence of gastrointestinal air below the diaphragm is an
important finding. Complete absence of gas in the gastrointestinal tract
denotes the absence of a distal TEF (although rare reports exist of distal
fistulae simply occluded by mucous plugs). In cases of esophageal atresia
without fistula, assume that the distance between the ends of the atretic
esophagus is too long for early one-stage primary repair. These infants
require a delayed repair.
Early renal ultrasonography is mandatory and is used to evaluate associated
kidney and/or ureteral anomalies.
Echocardiography is indicated early in the care of the infant with esophageal
atresia who has clinical signs of cardiovascular disease. However, a 1-day-old
neonate with significant congenital heart disease may have normal findings at
physical examination. Therefore, some argue that echocardiography should be
performed in all infants with esophageal atresia. This examination also provides the
surgeon with information regarding the side of the aortic arch. A right-sided aortic
arch is not uncommon in cases of esophageal atresia, and the surgeon should be
aware of this finding.
Limb radiography is indicated if the limbs appear abnormal. The possibility of
associated radial-ray deformities should be investigated.
Spinal ultrasonography is a simple test that takes advantage of the neonate's
relatively transparent lumber lamina in the assessment of an associated tethered
cord. This examination may be performed when the baby is younger than 1 month,
although it is not critically important in the early care of the infant.
In cases in which the distance between the 2 atretic ends of the esophagus is
suspected to be too long for a primary repair, a gap-o-gram is useful in assessing
A gastrostomy is created, and the upper pouch is intubated with a 10F
Replogle tube with radiopaque markings. A small-diameter Bakes dilator is
introduced into the gastrostomy and directed superiorly under fluoroscopic
guidance into the distal esophageal segment. With gentle but definite force
on both the Bakes dilator and the Replogle tube, the 2 ends are pushed
toward each other under fluoroscopic control.
At the point of least separation, an image is obtained, and the distance
between the 2 ends is determined in terms of vertebral bodies, which
provide an inherent reference for measurement.
Generally, a separation distance of 2 (some say 3) vertebral bodies or fewer
is usually small enough for an anastomosis. If greater distances separate the
ends, a delay of weeks to months may be required for the ends to grow
closer together, for reassessment with gap-o-grams every 4-6 weeks, or for
esophageal replacement or lengthening surgery.
Medical therapy: The preparation of a 1-day-old neonate for surgery includes the
Intravenous fluid containing an adequate glucose concentration (ie, 10% glucose)
is administered at a rate appropriate for the neonate's gestational age and weight.
Prophylactic broad-spectrum antibiotics (eg, ampicillin, gentamicin) are
The neonate is kept warm by using an incubator or overhead warmer, and he or
she is positioned supine in the Fowler position, with the head elevated by
A 10F Replogle tube is placed nasally or orally well into the upper pouch and
connected to a continuous suction device. Every 30 minutes, the tube is checked
for patency first by suctioning with an empty syringe and then by gently injecting 5
mL of air. (Never use water.) In small infants, an 8F double-lumen tube may be
The parents should be fully briefed about the nature of the congenital anomaly. A
diagram is invaluable for explaining not only the pathologic anatomy and intended
repair but also the possible complications. Their consent for treatment should be
obtained, and the discussion with them should be documented in appropriate detail
on the baby's medical record.
Surgical therapy: This section provides some details about surgical approaches for the
repair of the most common type of esophageal atresia, ie, esophageal atresia with distal
TEF, in low-risk patients. Surgical techniques vary according to surgeons' preferences and
variations in pathologic anatomy. Modifications for special anatomic challenges are briefly
discussed. In particular, infants born with esophageal atresia without fistula represent a
specific and challenging subgroup. These babies should undergo an early gastrostomy
procedure in the new born period.. A gap-o-gram should be obtained to assess the
prospects for anastomotic repair.
In infants with atresia without fistula, surgical decisions must be made regarding the
following: the length of time to wait for the ends to grow closer; performing one of a
number of esophageal lengthening procedures such as the Kimura, Livaditis, Scharli, or
Foker procedures; undertaking an esophageal substitution procedure, with or without the
formation of a cervical esophagostomy; and using a gastric tube (reversed and proximally
based or antegrade and distally based).
The use of colonic (left chest or substernal), gastric pull-up, or jejunal vascularized graft
segments is difficult and should be based on the condition of the infant, the pathologic
anatomy, associated defects (eg, gastric pull-up is usually contraindicated in significant
cardiac disease, colonic esophageal replacement is usually contraindicated with
concomitant imperforate anus), and the surgeon's experience.
As a rule, a child's own esophagus is better than any substitution. Recent favorable
reports of the Foker technique used for serial dynamic lengthening in cases of long gap
realize that advantage. It involves 2 thoracotomies. First, anchoring sutures are placed
securely at the 2 ends of the atretic esophagus and brought out diagonally to the chest
wall. Over a period of days to weeks, the 2 ends are brought closer together by a series of
daily lengthenings by traction on the exposed sutures. The closure of the gap is monitored
radiologically with radio-opaque markers at the atretic ends. A second thoracotomy is then
performed to effect a tension-free anastomosis.
Preoperative details: Bronchoscopy performed just prior to repair of the esophageal
atresia may enable the following:
Detection of a upper pouch fistula
Localization of the distal fistula, which usually lies at a level just above that of the
Detection of an aberrant right upper lobe bronchus emanating from the trachea,
which is not uncommon in cases of esophageal atresia
Early assessment of the cross-sectional shape of the trachea, which may help in
determining the risk of significant postoperative tracheomalacia
Assessment of specific vascular anomalies (eg, right-sided aortic arch, aberrant
right subclavian artery [for which one looks for the pattern of pulsation on the
Identification of a laryngotracheoesophageal cleft
The infant is endotracheally intubated without paralysis. The anesthesiologist must be
mindful of the distal fistula. With skill, the long end of the distal endotracheal tube bevel
may be positioned over the fistula to decrease the passage of gases into the stomach.
This maneuver helps prevent gastric distension, maximizes ventilation, and minimizes the
chances of a gastric perforation. As much as possible, the baby should be allowed to
breathe spontaneously until the fistula is occluded. In reality, and especially because the
chest is open and the lung retracted, the anesthesiologist manually assists with the baby's
ventilation. However, mechanical ventilation should be avoided until the fistula is
controlled. This procedure requires great skill, experience, and focus on the part of an
anesthesiologist in caring for these babies in the operating room.
Managing infant with premature lungs
In positioning the baby in full right thoracotomy position, the surgeon must ensure that the
anesthesiologist has full and easy access to the infant's nose and to the Replogle tube,
which is not taped so that it can move in or out. If a right-sided aortic arch is detected
preoperatively, controversy exists about whether a left thoracotomy provides easier
access. A left-sided approach has its merits, but in this instance, the esophagus is still a
right-sided structure, and access from the right is best.
Lastly, the baby is covered with antiseptic solution, and drapes are placed with the areas
from the nipple to mid back and from the axilla to the 10th rib exposed.
Intraoperative details: The surgeon should wear magnification loupes. The assistants
and nurses should be briefed about their duties and about special points of care regarding
the delicate nature of the procedure and the baby's tissues.
The procedure is performed as follows: A transverse right thoracotomy incision is made
from the anterior axillary line to approximately one finger's breadth posterior to the
posterior axillary line at a level 1 cm inferior to the palpable tip of the scapula. The
latissimus dorsi is divided with the coagulating current of the electrocautery device. The
fascia lying just posterior to the posterior margin of the serratus is divided with
electrocautery, and the serratus is retracted anteriorly. Usually, an incision in the serratus
is not needed. The scapula is then lifted away from the chest wall, and the ribs are
counted from the first to the fourth. Ideally, the chest is entered through the fourth
interspace. With careful use of forceps and the electrocautery device, the outer and
innermost intercostal muscles are divided in this interspace down to the parietal pleura.
By using either moist sponges or peanut gauze on the forceps, the parietal pleura is
dissected away from the chest wall, proceeding posteriorly but also dissecting somewhat
superiorly and inferiorly as well. A small mechanical Finochietto-type rib retractor is placed
in the open thoracotomy site, and the pleural dissection proceeds to a point medial to the
azygos vein. The azygos vein is ligated and divided with fine silk. This extrapleural
dissection then allows retropleural repair of the esophagus. If an anastomotic leak occurs,
it tends to be more contained compared with the empyema that results if the repair is
At this point in the dissection, the anatomy is defined first by having the anesthesiologist
push on the indwelling Replogle tube; this action usually reveals the upper pouch that
rhythmically bulges out in the apex of the right chest cavity. The distal fistula is at the level
of the carina and usually lies just beneath the divided azygos vein. It expands slightly with
each inspiration. One must take great care not to mistake the aorta for the fistula.
Mistaken ligation of the aorta is possible. If doubt exists, a 25-gauge needle can be
passed into the structure to check.
Gaining control of the fistula now relieves the anesthesiologist. A silicone rubber vessel
loop can be passed around the fistula at a convenient level near the trachea. Gentle
retraction on this occludes the fistula. Most advise dividing the fistula with suturing of its
tracheal aspect. This division can be accomplished by cutting into the fistula as it enters
the back wall of the trachea in short snips and by oversewing the tracheal aspect as it
opens in stages. Usually, about 4 interrupted sutures suffice. Most advocate the use of an
absorbable synthetic suture material such as polyglactin. This sutured fistula site may be
covered with an azygos or pleural patch for extra security.
The fistula closure should be checked by covering the closure in saline and manually
ventilating the patient for a Valsalva test. If bubbles appear, the closure is leaking and
must be resutured. Turning his or her attention to the upper pouch, the anesthesiologist
again can push on the Replogle tube to facilitate placement of a traction suture into the
distal end of the upper pouch. The upper pouch is then dissected superiorly to increase its
length. Blood supply to the upper portion is linearly arrayed from the cervical and
subclavian vessels; ischemia is not a concern. This dissection must be carefully
performed between the pouch and the trachea while the presence of an upper pouch
fistula that emanates from the side, not the end, of the pouch is determined. Also, the
back wall of the trachea may be entered inadvertently. This condition is repairable with
Avoid extensive dissection of the distal end because its blood supply is segmental from
the aorta, and it can easily become ischemic. A gap between the ends may seem to be
present. If it is very lengthy, the muscular covering of the upper pouch may be cut without
entry into the lumen to achieve an extra 1 cm or so. Distal dissection may be performed;
the risk of ischemia should be recognized. If absolutely necessary, the 2 ends may be
simply bridged using 2 stout silk sutures in the hopes that they form a fistula and that they
can be dilated to form a functional esophagus. More commonly, the 2 ends are reasonably
close, and an anastomosis is possible.
The distal portion of the upper pouch is cut off, and the proximal portion of the distal
segment is trimmed. Both the mucosal and muscularis layers of the esophagus should be
carefully sutured in a single layer to form an anastomosis with simple interrupted stitches.
Once again, most advocate the use of an absorbable synthetic suture with a caliber of
approximately 5-0 (eg, braided polyglactin). The back wall is sutured, and the upper pouch
tube is passed through the half-completed anastomosis into the stomach to help rule out a
distal stricture and to empty the stomach of accumulated gas.
This tube is left in place as the anterior wall of the anastomosis is completed. The tube is
then gently withdrawn from the body. Some advocate leaving the transanastomotic tube to
act as a stent, although this tube may be partially moved, potentially injuring the
anastomosis. A small-caliber 10F chest tube may be left in place as an extrapleural chest
drain. The ribs are closed by encircling them with two 3-0 absorbable sutures and by
restoring their normal anatomic position. The muscles and skin are closed in layers with
In some pediatric surgical centers, surgeons are gaining experience in repairing
esophageal atresia using a minimally invasive thoracoscopic approach. At present, this
approach could still be termed investigational and should be undertaken only by those
who have extensive experience in pediatric thoracoscopic surgery.
Postoperative details: The intubated patient is transported to the neonatal intensive care
unit. Antibiotics are continued until the chest drain is removed, and the endotracheal tube
is suctioned as necessary. Oral suctioning to a depth of no more than 7 cm from the lips is
performed every half hour for the first day, then every hour or more frequently as
necessary on the second day. Thereafter, it is performed as needed. Suctioning is
required to handle the sometimes copious oral secretions that can build up in the first day
or so after surgery. As the swelling of the esophagus settles, the secretions taper.
The chest draining tube is placed in 2 cm of water only to seal it; it is not connected to a
suction device, which could encourage an anastomotic leak. Morphine is infused as
necessary for the patient's comfort, and peripheral parenteral nutrition should be
commenced. The endotracheal tube should remain until weaning from ventilation is
ensured, usually after 1-2 days. Premature extubation and subsequent intubation in the
setting of a freshly closed tracheal fistula invites reopening of the fistula.
Watch for saliva exiting out the chest drain; this is a signal of anastomotic leakage. Often,
it is accompanied by visible distress. Signs of sepsis may or may not be present. A chest
radiograph should be obtained. Provided that the baby is stable, a contrast-enhanced
study of the esophagus with a water-soluble isotonic medium may be performed on day 6
or 7 to assess for leaks and to view the caliber of the repair (see Image 6). If the
esophagus is patent and reasonably sized, the baby may be fed orally; starting with
expressed breast milk is ideal. Then, the chest tube is removed. As soon as the baby is
feeding well, the intravenous line is discontinued, and the baby can be discharged. Oral
ranitidine is prescribed for 6 months because of the propensity for gastroesophageal reflux
in this group of patients and because of the risk of stricture as a secondary effect.
Follow-up care: If all is well with the patient and if the parents have been briefed on what
to look for, a reasonable follow-up regimen may include the following steps:
Make contact with the community physician who is responsible for the general
medical care of the child and ensure that he or she is briefed on the baby's history,
condition, and expected outcome.
The nurse on the surgical team should follow up by telephone in 1 week.
The surgeon should follow up in 1 month to interview the parents and generally
assess the child's condition, growth, and healing at the surgical site.
The patient should return at 3 months for a similar assessment.
At a 1-year follow-up and general assessment, swallowing function, respiratory
issues, and so on should be addressed.
Radiologic assessment of the esophagus is required only if a significant history of choking,
cyanosis, regurgitation, dysphagia, growth failure, coughing, or wheezing exists.
Subsequent endoscopic evaluation can be performed as indicated.
Follow-up care when the child is older can be performed as needed. Specific
reassessment with esophageal endoscopy and biopsy when the patient is aged
approximately 12 years has been advised by some, who also advise follow-up with
periodic endoscopy every few years until the patient is an adult. Although Barrett
esophagus, and even subsequent malignant change, has been described in this condition,
presumably because of gastroesophageal reflux, the jury is still out on the true need for
endoscopic surveillance in patients with repaired esophageal atresia.
Early complications may include an anastomotic leak, recurrent TEF, and anastomotic
An anastomotic leak tends to occur 3 or 4 days after surgery. This leak has been reported
to occur in approximately 15% of cases. Pain and distress often are evident. Signs of
sepsis may be present. The chest tube drains saliva. Treatment is supportive; appropriate
antibiotics should be used, and the child should be given nothing by mouth. Surgery is not
indicated, even with huge leaks. If the leak persists, esophagography may be performed
with water-soluble contrast material to assess its magnitude. The usual protocol is to wait
and let the leak close. If an extrapleural approach was used, the child usually is less ill
than with other approaches, and the resultant esophagocutaneous fistula closes within
days. If a transpleural approach was used, then the child is more ill and has an empyema
that may require further treatment and drainage. No absolute evidence indicates that
postoperative leaks lead to anastomotic stenoses.
Recurrent TEF may occur within days; most often, it occurs weeks later. Its incidence has
been variously reported as 3-14%. Its first manifestation may be pneumonia, although the
child may cough and have respiratory distress with feeding. The diagnosis is made by
means of an esophagography performed with water-soluble contrast material under
fluoroscopic guidance with the child prone. The contrast material is slowly injected through
a catheter in the esophagus as the tube is slowly withdrawn, and lateral views are
obtained by means of videofluoroscopy.
The recurrent fistula is observed as a wisp of contrast material that suddenly crosses over
to the trachea. This so-called pull-back esophagraphy is the most accurate method for
diagnosing a recurrent fistula. Bronchoscopy and esophagoscopy may provide
supplementary information. One endoscopic technique is to inject 0.5 mL of methylene
blue into the endotracheal tube and through the esophagoscope while watching for it to
come through the fistula. Historically, these fistulae were believed to require surgical
repair by means of repeat right-sided thoracotomy; however, the authors have been
successful in a minority of cases of fistulae by allowing them to close spontaneously while
maintaining the nothing by mouth restriction and while administering antibiotics for 1 week.
Endoscopic cautery and fibrin glue have also been reported to be successful on occasion.
Anastomotic stricture has been reported in as many as 50% of cases, but the rate partially
depends on the definition of stricture. Essentially 100% of babies have a waist at the
anastomotic site, but this may not be functionally significant. In cases in which the stricture
appears to be functionally significant on oral contrast-enhanced studies, esophageal
dilation is best and is most safely performed by means of a Grьntzig balloon technique
under fluoroscopic control, in the authors' opinion. This procedure should be performed by
an experienced radiologist who can monitor the balloon pressure, position, and inflation
diameter. In newborns, this technique of dilatation would best be deferred until the child is
at least 6 weeks old, and at least 4 weeks after anastomosis.
Other methods involve the passage of tapered dilators of various sorts (eg, Tucker and
Maloney dilators). Certainly, the methods can be effective but are performed in essentially
a blind manner unless done under fluoroscopic control, and they involve longitudinal and
radial force vectors as opposed to the pure radial force vectors of the Grьntzig technique.
Repeat dilations are often necessary. H2-receptor blockade should be started because
acid reflux can be both an aggravating and a causative factor in stricture formation.
Other factors to consider include the surgical technique, type of suture used, length of the
atretic gap, ischemia of the distal portion, and possibly, whether an anastomotic leak has
occurred. Strictures resistant to a few dilations need more aggressive treatment, which
may include an antireflux operation, stricture resection, or both; rarely, they require
esophageal replacement. Stents have been used, but they are still investigational.
Surprisingly, parents can be taught to perform regular Maloney dilations at home in
Late complications may include gastroesophageal reflux, esophageal dysmotility, and
tracheomalacia. Some of these complications may appear early.
Gastroesophageal reflux is particularly problematic in patients with esophageal atresia
because of congenital distal dysmotility of the esophagus, dysfunction of the physiologic
antireflux barrier, possible partial vagotomy during surgery, or essential vagal dysfunction
that can lead to delayed gastric emptying. Essentially all babies with esophageal atresia
have detectable gastroesophageal reflux. Patients who require treatment must be
All babies with esophageal atresia should be treated prophylactically with ranitidine until
they are aged 6 months. Failure to thrive, coughing, choking spells, wheezing and asthma,
recurrent pneumonias, vomiting, cyanosis, dying spells, excessive drooling, and apparent
dysphagia are all indications to investigate the degree of gastroesophageal reflux. Oral
contrast material should be administered, and endoscopy should be performed. Strictures
should be dilated. A pH probe study may help if the probe is placed below any present
stricture. A gastric emptying scan should be obtained. All factors should be carefully
Surgical approaches to helping the child may include an antireflux operation. A partialwrap fundoplication is usually preferred because of the dysmotility of the repaired
esophagus. Dysphagia after even a very loose wrap is not uncommon. If the stomach has
delayed emptying, balloon pyloroplasty or surgical pyloroplasty may be considered to
speed emptying. The authors have used a surgically conservative approach in children
with this condition; the authors prefer to treat the reflux medically, with H2-receptor
blockade or proton pump inhibition when possible. However, certainly some patients
require a surgical approach for later complications.
Esophageal dysmotility is an ongoing problem. It has various dysphagic manifestations.
The children eventually learn that they must masticate thoroughly and drink fluids when
eating. Food bolus obstructions, even without a significant stricture, are not uncommon in
toddlers. Parents must be mindful of this possibility and choose their child's foods
accordingly. The use of motility agents such as domperidone may help.
Tracheomalacia is the manifestation of disordered embryogenesis. In its severe form,
which occurs in approximately 10% of patients, dramatic signs include an inability to wean
the patient from a ventilator and the classic dying spells in which the patient becomes pale
and limp and, usually, apneic and cyanotic for a short time. Children with this condition
require examination and treatment. Milder cases of tracheomalacia may cause recurrent
pneumonias or asthma attacks, and in general respiratory ailments are common in these
Bronchoscopy performed while the patient is spontaneously breathing reveals a trachea
that significantly collapses, flattens, or closes on expiration. Treatment consists of
aortopexy, which suspends the aortic arch to the underside of the sternum and thus
secondarily suspends the anterior tracheal wall anteriorly, preventing its collapse. If this is
unsuccessful, stent placement may help, but this option is controversial. Tracheostomy is
the final management option. Fortunately, tracheomalacia tends to improve with time,
growth, and maturation.
OUTCOME AND PROGNOSIS
Statistics regarding mortality rates in esophageal atresia are constantly changing and
improving. One must consider the classification system used in reporting such statistics.
Montreal classification (rates reported in Poenaru, 1993) - Class I, 7.3% mortality
rate; class II, 69.2% mortality rate
Spitz grouping (rates reported in Spitz, 1993) - Group I, 3% mortality rate; group II,
41% mortality rate; group III, 78% mortality rate
Waterston categorization (rates reported in Engum, 1995) - Category A, 0%
mortality rate; category B, 4% mortality rate; category C, 11% mortality rate
Fetuses with prenatal diagnoses of esophageal atresia seem to have a worse prognosis
(Stringer, 1995). The cohort of babies in whom esophageal atresia is detected prenatally
has a 75% mortality rate, whereas the cohort of babies in whom esophageal atresia is not
detected prenatally has a 21% mortality rate. Babies who survive have varied morbidities
related to any of the associated anomalies and complications. However, most children
who undergo a successful repair of esophageal atresia are relatively healthy.
FUTURE AND CONTROVERSIES
The future is bright, with the following considerations: More accurate prenatal diagnosis
and prenatal treatment may be possible. Minimally invasive techniques for repair with
thoracoscopic surgery are now being used in some centers, with good results. A better
understanding of the pathoembryologic processes of this condition may reveal its
causative agents or genetic factors. This knowledge, in turn, may lead to specific prenatal
treatments or preventive techniques. Recently, the incidence of this disorder has
decreased, perhaps because of increased usage of prenatal folic acid supplements.
Debates continue about the best operative technique (eg, right-sided or left-sided
thoracotomy) for patients with right-sided aortic arches, suture type and technique,
esophageal lengthening strategies, and procedures for mobilizing the distal esophagus.
Other discussions concern when to use cervical esophagostomy, the choice of
esophageal replacement, and so on. The advent of esophageal atresia repairs that
combine both minimally invasive and radiologic interventional techniques may be near.
The management of gastroesophageal reflux in esophageal atresia is particularly
challenging; some advocate aggressive fundoplication, and others prefer more
conservative medical treatment. In addition, the true incidence and treatment of
tracheomalacia continues to be the subject of debate. Lastly, the proper evidence-based
guidelines for long-term follow-up are still elusive.